A 32-year-old male presented in 1981 with severe pancytopenia and a macular haemorrhage. He was noted to have splenomegaly and a bone marrow infiltration consistent with a lymphoproliferative disorder. A splenectomy was performed in October 1981 with subsequent resolution of his pancytopenia. The splenic sample confirmed the diagnosis of HCL. In April 1982, he presented with back pain with initial investigations suggesting osteoporosis with vertebral collapse. A bone biopsy confirmed involvement of the dorsal spine with HCL. At this time his peripheral counts were normal. He commenced and completed six cycles of chlorambucil 20 mg daily orally for three days a month in May 1982 with the back pain responding well. He remained in remission until an increasing leucopenia in October 2009 suggested a further relapse, confirmed by bone marrow aspirate and biopsy. In the bone marrow, kappa-restricted hairy cells represented 13% of B lymphocytes (), were tartrate resistant acid phosphatase-positive (), and possessed the classical HCL immunophenotype (CD5−, CD10−, CD11c+, CD19+, CD20+, CD22+, CD23−, CD25+, CD79b−, CD103+, CD200+, FMC7+). Allele-specific PCR did not detect the BRAF V600E mutation []. He commenced on a five-day course of subcutaneous cladribine in November 2009 with both clinical and immunophenotypic remissions achieved. Neutropenia signalled another relapse in June 2012. The bone marrow hairy cells (39% of B lymphocytes) had the same immunophenotype as the previous relapse, several of which were multinucleate, possibly a treatment related effect (). The presence of the BRAF V600E mutation was again not detected by allele-specific PCR and a further two commercial assays (Competitive Allele-Specific Taqman PCR, Applied Biosystems, Paisley, UK, and Cobas, Roche, Burgess Hill, UK). The B-cell population was further enriched by CD20 magnetic bead selection: treatment of these B-cells with the BRAF inhibitor PLX4720 showed no decrease in ERK phosphorylation () while Sanger sequencing of BRAF exons 11 and 15 revealed wild-type sequence (). He recommenced cladribine subcutaneously for five days with eight weekly doses of rituximab and remained clinically well at last followup.


A 79-year-old woman presented to the emergency department with complaints of weakness and numbness in the left upper and lower extremities. She had been diagnosed with gastric cancer (adenocarcinoma of the gastric cardia, T2N3 M0) 5 years previously, and chemo- and radiotherapy were performed at that time. However, her cancer was progressive and she had developed stenosis of the cardiac portion of the esophagus.
Neurological examination revealed left-sided hypoesthesia, and left-sided hemiparesis with a National Institutes of Health Stroke Scale (NIHSS) score of 7. Although the patient did not fulfill the diagnostic criteria of disseminated intravascular coagulation syndrome, she had abnormal coagulation with a fibrinogen level of 191 mg/dL; fibrin/fibrinogen degeneration products, 47.9 μg/mL; and D dimer, 29.2 μg/mL. The other laboratory parameters, including protein C, S, antinuclear antibody, antiphospholipid antibody, and homocysteine, were within normal limits.
On the initial T2WI, there was a diffuse high-intensity lesion in the right thalamus, extending into the posterior limb of the internal capsule and midbrain (). The central part of the right thalamic lesion showed high intensity on the diffusion-weighted image (DWI) (), and a decreased apparent diffusion coefficient (). Both transverse sinuses also showed high intensity on the DWI (). The T2*WI showed diminished signal and enlargement of the right basilar vein () and the vein of Galen (). On the basis of the MRI findings, she was diagnosed with CVT, and anticoagulation therapy with heparin was administered. Two days later, MRV showed an absence of flow in the inferior sagittal sinus, vein of Galen, and straight sinus (), and a reduction of flow in the transverse sinus ().


A previously well 29-year-old lady presented to neurology services in 2003 complaining of daily “jerking” of one or more limbs. This was associated with intermittent tremulous episodes and 18-month history of twice-weekly migraine with visual aura. She described a right-sided headache, vice-like in nature, which was progressive and worse on waking. Exercise appeared to precipitate presyncopal symptoms and a feeling of detachment from her surroundings. Admission was triggered by a generalized tonic-clonic seizure. There was no reported change in memory, cognition, or coordination. She took the oral contraceptive pill and family history included an uncle with multiple sclerosis. She was an only child and had no children of her own. She was completely independent with activities of daily living.
Examination demonstrated visual acuity of 6/9 in the right eye and 6/18 in the left eye. Pupils reacted equally to light and accommodation and there was no relative afferent pupillary defect or deficiency on Ishihara testing. Ophthalmoscopy revealed healthy retinae bilaterally with slightly hypopigmented choroid felt to be in keeping with the patient's complexion. No opthalmoplegia was present and there was no deficiency in facial sensation or movement. No conductive or sensorineural hearing loss was found and she demonstrated normal tongue and palatal movements.
There was no evidence of tremor or myoclonus on examination of the upper and lower limbs, with no evidence of muscle wasting. She had normal muscle tone and power in all muscle groups. Reflexes were normal in the upper limbs, yet she appeared globally hyperreflexive throughout the lower limbs with a crossed adductor reflex. Plantar responses were flexor. Sensory testing was normal in all modalities with no gait or truncal ataxia. There was minimal left upper limb ataxia with some slowness when doing up buttons. Speech was normal and there were no other signs of cerebellar dysfunction.
Examination of other systems was unremarkable.
Initial neuropsychological assessment in 2003 revealed no significant cognitive dysfunction scoring in the average range on testing verbal comprehension, perceptual organisation, working memory, and processing speed.


A 26-year-old man of Bangladeshi origin presented with a two-day history of generalised abdominal pain, loss of appetite, and nausea. His past medical history includes hypertension, type 1 diabetes mellitus, and autosomal dominant polycystic kidney disease. He is the product of a consanguineous marriage and suffered from intrauterine growth retardation and failure to thrive with persistent short stature and microcephaly. Previous investigations for chromosomal disorders at Great Ormond Street Hospital were negative, and the karyotype was confirmed as 46XY.
On examination, he was afebrile and physiologically stable with tenderness in the lower abdomen and a BMI of 14 (height 135.5 cm and weight 25.56 kg). Inflammatory markers were mildly raised (white cell count 13.2, neutrophils 8.4, and C-reactive protein 87). He was admitted for observation, intravenous fluids and antibiotics. A computed tomography scan of the abdomen and pelvis revealed mild thickening of the descending colon but no focal inflammatory changes, free air, or free fluid.
The patient deteriorated clinically and underwent a diagnostic laparoscopy, which identified a thickened inflamed loop bowel adherent to the anterior abdominal wall. A laparotomy was performed. There was extensive diverticular disease and an associated inflammation of the entire colon with an inflammatory mass around the transverse colon adherent to the anterior abdominal wall. The diverticula were large and broad based, resembling jejunal diverticulosis macroscopically. Malignancy could not be excluded, and the tissues were extremely friable, so a subtotal colectomy and end ileostomy with abdominal washout were performed. Our patient's postoperative course was complicated by an episode of aspiration pneumonia from which he made a full recovery.
Histopathological examination supported the diagnosis of true pancolonic diverticulosis. The entire specimen showed numerous large diverticula and associated inflammation/abscess formation. The majority of the diverticula had a colonic type muscularis propria and can therefore be considered as true diverticula resulting from congenital malformation (). Lymph nodes showed reactive changes only. Subsequent elastin immunohistochemical staining was within normal limits.
He was discharged 13 days postoperatively, with a view to restoration of intestinal continuity in 6 months.


A 44-year-old man was clinically diagnosed with nasopharyngeal carcinoma stage T2N3M0 and IVa as well as pathologically confirmed as having poorly differentiated squamous cell carcinoma. The patient was initially treated with 1 cycle of chemotherapy with paclitaxel 210 mg and cisplatin 120 mg from April 5 to 7, 2010. From April 14 to June 9, 2010, intensity-modulated radiotherapy (IMRT) was delivered with the following treatment plan and target volumes: 70 Gy in 33 fractions (fx) for 57 days for the primary nasopharyngeal lesion; 70 Gy in 33 fx for 57 days for the neck metastatic nodes; and 58 Gy to 61 Gy in 33 fx for 57 days for the other neck areas.
One year after radiotherapy, from June to September 2011, the patient suffered from lower back pain. The entire skeleton bone scintigraphy and lumbar MRI revealed multi-site bone metastases with the most severe bone erosion at lumbar 1. Palliative radiotherapy was delivered for the posterior field ranging from thoracic 12 to lumbar 2 at a dose of 40 Gy in 20 fx for 31 days. Subsequently, chemotherapy with docetaxel 160 mg (95 mg/m2) and cisplatin 120 mg (70 mg/m2) was administered on October 10, 2011. Five days later, erythema, edema, pururitis, and papules followed by light scaling appeared on both sides of the neck skin. In addition, swallowing pain due to pharyngitis and a symptom of dysphagia manifested during the same period. All of these symptoms occurred in the previously irradiated area (). Docetaxel-induced radiation-recall dermatitis and pharyngitis were logically inferred. A daily dosage of 10 mg of dexamethasone was intravenously administered. Seven days after chemotherapy, febrile leukopenia with WBC count of 0.4×109 cells/L developed. Cefazolin and granulocyte colony-stimulating factors were prescribed. The WBC normalized 3 days later. The neck skin normalized 8 days after the inception of radiation recall reaction.


In April 2006, a 19-year-old man was initially diagnosed with nasal non-Hodgkin lymphoma and was pathologically confirmed as peripheral T-cell type. Two cycles of CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy were administered, followed by conventional radiotherapy with the bilateral nasal cavitie tumor dose of 33.4 Gy in 17 fractions. Thereafter, another two cycles of consolidative CHOP chemotherapy were performed.
In November 2011, nasal cavity recurrence was diagnosed and pathologically classified as nasal-type NK/T cell lymphoma. Conventional radiation was delivered, including the areas of nasal and nasopharyngeal cavities, maxillary, and ethmoid sinus, with a dosage of 17.5 Gy in 9 fractions. IMRT with a dosage of 35.2 Gy in 16 fractions was prescribed to the clinical target volume. The total radiation dose calculated for the nasal cavity was 54.8 Gy in 25 fractions. Upon completion of the treatment, CT scan results indicated unconfirmed complete remission. Grade 2 dermatitis, grade 3 nasal mucositis, and grade 2 stomatitis (CTCAE v4.0) were documented during radiotherapy and resolved within one month after the end of radiation.
After 5 weeks, CHOP chemotherapy was initiated as a consolidative means on March 19, 2012. Three days later, erythema and edema appeared on the skin of the head that previously received the composite isodose distribution of radiation therapy (). Radiation recall dermatitis was reasonably considered, and the causative link was generally attributed to the agents of CHOP regimen. No special measures were delivered to treat the erythema and edema, which turned into mildly dry desquamation 5 days later and gradually disappeared. Another course of CHOP regimen was administered at the same dosage. Mild erythema and edema reappeared but were similarly resolved without any special treatment.


In October 2005, a 28-year-old man was referred to our hospital because of leukocytosis, splenomegaly, and weight loss. Peripheral blood count showed hemoglobin: 9.4 g/dL; platelets: 253×109/L; white blood cells (WBC): 292×109/L with 32% neutrophils, 2% lymphocytes, 3% eosinophils, 3% basophils, 58% immature granulocytes, and 2% blasts. Myeloid hyperplasia of the bone marrow (BM) with high proportion of cells at different stages of maturation, moderate eosinophilia without an excess of blasts, and 3% blasts was found. Cytogenetic analyses were performed on BM cells. Metaphases were obtained after short-term unstimulated cultures (24 and 48 h). The chromosomes were identified by heating R-bands through the Giemsa (RHG) technique. The chromosomes were classified following the Guidelines for the International System for Human Nomenclature. FISH was performed by using whole-chromosome paint probes for chromosomes 3 (spectrum green)/12 (spectrum orange) (Vysis, Inc., Downers Grove, IL, USA). All of the details concerning the pretreatment of slides and hybridization were carried out according to the instructions accompanying the probes. The chromosomes were counterstained with 4’,6-diamidino-2-phenylindole and viewed with an Axioskop fluorescence microscope (Zeiss, Germany) by using triple excitation/emission bandpass filters. At least 10 metaphase cells were analyzed.
Chromosomal analysis revealed 46,XY, t(9;22)(q34;q11). The patient was diagnosed as having CML in the chronic phase. Treatment was initiated with hydroxyurea (1,500 mg daily dose). Eight months after the treatment, the WBC of the patient decreased to 4.7×109/L (with 43% neutrophils), platelets: 132×109/L; hemoglobin: 14 g/dL. The patient was then treated with imatinib mesylate (400 mg daily dose). After 6 months, cytogenetic analysis revealed a major cytogenetic response with 10% Ph+ cells. Twelve months after imatinib treatment initiation, a repeat R-banding karyotype showed a complete cytogenetic response (CCyR). After 24 months, the patient’s blood count showed WBC: 5.5×109/L (65% neutrophils, 33% lymphocytes, 1% monocytes, and 1% basophils); hemoglobin: 13.4 g/dL; platelets: 177×109/L. Karyotype analysis showed additional chromosomal abnormality (trisomy 8): 47,XY,+8,t(9;22)(q34;q11)/46,XY. Subsequently, the dose of imatinib was raised to 600 mg daily. After 6 months, CCyR was reached again.
After 24 months, the patient’s blood count showed WBC: 5.3×109/L (58% neutrophils, 36% lymphocytes, 5% monocytes, and 0.5% eosinophils); hemoglobin: 11.5 g/dL; platelets: 162×109/L. Cytogenetic analysis revealed two cell clones with additional chromosomal abnormalities: 47, XY, t(9;22)(q34;q11),+der(22)t(9;22)/48, idem, +8/46, XY.
Treatment with nilotinib (200 mg daily) was initiated, and 2 months later, the blood count showed WBC: 6.7×109/L (66% neutrophils, 26% lymphocytes, 5% monocytes, and 0.5% eosinophils); hemoglobin: 14.2 g/dL; platelets: 219×109/L.
CCyR and complete molecular response were reached throughout 3 months until the patient discontinued nilotinib treatment. Hematologic parameters showed that hemoglobin was 13.8 g/dL, platelets were 273×109/L, and WBC was 60.3×109/L (61% neutrophils, 7% lymphocytes, 4% monocytes, 1% erythroblasts, 20% immature granulocytes, and 7% blastic cells) ().
Bone marrow aspiration concluded to an accelerated phase with 14% blastic cells (17% promyelocytes, 34% neutrophils, 20% eosinophils, and 6% erythroblasts) as well as an average bone marrow cellularity with rare megakaryocytes, platelets, and 21% myelocytes and metamyelocytes. Conventional karyotype analysis revealed additional recurrent translocation with t(9;22):46,XY,t(3;12)(q21;p13), t(9;22)(q34;q11) (), which was confirmed with FISH by using whole-chromosome paint probes for chromosomes 3 and 12 ().


A 59-year-old man was admitted to the vascular surgery department as a result of acute ischemia of the lower limb in December 2010. The patient also complained of weakness, lack of appetite, and weight loss that had developed in the preceding several months (20 kg in the past 4 months). He had a several-week history of abdominal pain with radiation to the lumbar region, which had recently become exacerbated. The patient did not exhibit voiding dysfunction (urine or stool). Palpation revealed abdominal tenderness in the periumbilical region, with no peritoneal signs. During hospitalization, laboratory test results indicated the presence of an inflammatory process: mild leukocytosis (white blood cells 8,300–10,100/μl), greatly elevated C-reactive protein (CRP)—up to 46 mg/l—and extremely elevated D-dimer levels (6,370–5,920 mg/ml). Ultrasound examination was performed, which revealed an inflammatory aortic aneurysm (diameter >90 mm) and embolism of the left femoral and left popliteal arteries. Computed tomographic (CT) examination (64 × 0.625 mm GE Lightspeed VCT; GE Healthcare, Waukesha, WI) revealed retroperitoneal inflammatory infiltration surrounding the aorta, an abdominal aortic aneurysm 95 mm in diameter situated below the level of the renal arteries, and embolism of the left femoral artery (Fig. ). A decision was made to perform surgical embolectomy and endovascular intervention (EVAR). The patient provided written informed consent.
During the first stage of treatment, a left-sided embolectomy was performed with a Fogarty catheter, and emboli from the femoral and popliteal arteries were removed. In the 24 h after this procedure, a further embolism of right femoral artery developed, which was removed during a second embolectomy. The next day, an EVAR procedure was carried out in the angiography suite under fluoroscopic control (Axiom-Artis; Siemens, Erlangen, Germany). It was performed with the patient in the supine position and under general anesthesia. Arterial access was achieved via an open femoral arteriotomy. Next, after open exposure and puncture of the right femoral artery, a Lunderquist Extra Stiff Wire Guide, 0.035 inches, 260 cm (Cook, Bloomington, IN), and a stiff guide wire, 0.035 inches, 260 cm (Terumo, Somerset, NJ), were inserted and passed into the aorta. A 5F pigtail was also passed into the aorta via left femoral access and was used to perform angiographies. A stent graft was passed into the aorta via the right femoral artery and placed across the neck of the aneurysm, covering it entirely while preserving flow in the common iliac arteries. The stent grafts used were Zenith: proximal body 28 × 111 mm, and distal branches 18 × 56 and 16 × 73 mm (Cook, Bloomington, IN). A check angiogram revealed no leaking into the aneurysm and maintenance of flow in the iliac arteries. At the start of the procedure, a bolus of 2500 U of heparin was administered, and a similar dose was provided in a continuous saline flush. The patient received prophylactic antibiotic therapy to prevent infection of the stent graft (spiramycin 3 million IU, two tablets per day; meloxicam 15 mg, two tablets per day, for a total of 20 days).
The patient was discharged in good general condition, with persisting mild pain in the lumbar area, which gradually increased with time. A follow-up angio-CT performed after the procedure in March 2011 confirmed the patency of the implant and confirmed that the aneurysmal sac had been successfully excluded. However, the hematoma and inflammatory infiltration that had been present previously remained visible in the surrounding tissues, with significant progression; it now encompassed the iliopsoas muscle and L4 vertebra with destruction of its body (Fig. ). Laboratory tests revealed persistent mild leukocytosis and elevated CRP levels (white blood cells 7.500–8.000/μl, CRP 103 mg/l, D-dimer 1,720 mg/ml). Additional hematoma and/or inflammatory infiltration was suspected, and the patient was readmitted to the hospital. An ultrasound-guided biopsy of the described lesion in the iliopsoas muscle was performed (Vivid 7; GE, Waukesha, WI). The presence of pus was not confirmed, and there was no bacterial growth from the aspirated material. Cytological examination revealed the presence of inflammatory granulation tissue (macrophages, lymphocytes, numerous stimulated mesothelial cells). Blood culture and tuberculosis test were both negative. Antibiotic and anti-inflammatory treatment were administered (clindamycin 3 × 300 mg, metronidazole 2 × 250 mg, omeprazole 2 × 1 tablet).
Follow-up ultrasound and CT in April 2011 revealed a persistent area of inflammation in the iliopsoas muscle and destruction of neighboring vertebral bodies (L3, L4) (Fig. ); therefore, the patient was rehospitalized. A repeat ultrasound-guided biopsy of the lesion within the iliopsoas muscle was performed. This revealed the presence of inflammatory tissue with no signs of infection (bacterial and mycological tests were once again negative). Blood culture and tuberculosis tests were also negative. White blood cell level was 5.600/μl, CRP 51 mg/l, and D-dimer 2,018–1,732 mg/ml. On the basis of these findings, a final diagnosis of inflammatory aneurysm was established. The spine was stabilized with a Jewette corset, and the patient received steroid treatment (dexamethasone 1 mg/day). During the next few days, the patient’s condition greatly improved, and pain symptoms subsided. Angio-CT was performed 5 months later in September 2011, which revealed patency of the stent graft with no signs of leaking, and the aneurismal sac had become markedly reduced in size. The extent of the retroperitoneal inflammation had diminished, and the affected vertebrae exhibited signs of remodeling and new bone formation (Fig. ).


We report an unusual pattern of 3 major components of the cardiovascular system in a 42-year-old Caucasian man who attended our hospital to undertake invasive coronary evaluation due to symptoms of stable angina. He had a history of arterial hypertension, dyslipidemia and a family history of coronary artery disease. Prior to admission, he presented in outpatient clinic with stable angina over the last two months, without ischaemic changes in rest electrocardiogram. Without receiving medication, he underwent a treadmill exercise test which was suspicious for ischaemic changes. Specifically the patient described chest pain of mild intensity at peak exercise with mild ST depression in the anterolateral leads.
A two-dimensional transthoracic echocardiogram (TTE) was performed. On short parasternal axis view at the level of great vessels the LCA seemed to share a common ostium with the RCA, originating from the right sinus of Valsava. No wall motion abnormalities were revealed, systolic function was normal and color-Doppler yielded no-pathologic findings [Figure ]. The pericardium was free of fluid. The patient then underwent a coronary arteriogram which revealed no apparent coronary obstructive lesions. What was noteworthy was the anomalous origin of the left coronary artery from the right coronary sinus. The RCA shared a common ostium with the anomalous left coronary artery. In the right anterior oblique (RAO) projection, LCA -prior to its bifurcation- follows a cranial and leftward direction [Figures and ].
In order to better deliniate the origin and the course of the coronary arteries our patient was referred for magnetic resonance angiography (MRA) and coronary computed tomography angiography (CCTA). CCTA findings [Figures , , , ] were compatible with that of a congenital anomaly in the origin of the left coronary artery which originates from right Valsava sinus with a common ostium with the right coronary artery. Left main coronary artery is 2 cm in length (longer than average) follows an interarterial course, passing between aortic root and conus arteriosus at the level of the pulmonary valve. Immediately after its exit (between the aortic root and the right ventricular infundibulum), it bifurcates to LAD and LCx. The right coronary artery originates from the RSV, coursing between the root of the aorta and the right ventricular outflow tract. LAD in its mid portion presents myocardial bridging of non significant length and depth. Coronary vessels were without any critical stenosis and coronary artery calcium score was negative. MRA clearly depicted anomalous origin of the left common carotid artery from the proximal part of the brachiocephalic trunk (innominate artery) [Figure ] and an aneurysm of the descending thoracic aorta with a transverse diameter of 3,1 cm [Figure ] thus bringing the number of incidentally found concurrent abnormal arterial patterns to three.
Finally in order to rule out ischemia as a cause of angina patient was scheduled for SPECT. SPECT was negative. Angina was attributed neither to the coronary artery ectopy nor to the myocardial bridging.


A 50-year-old otherwise healthy female arrived at our Plastic Surgery Department, with a one year history of a painless slow growing tumor on the volar aspect of the third finger of the left hand (Figure ) that progressed to a limited interphalangeal joint movement prompting medical assistance.
The presenting lesion was a 50 × 20 mm subcutaneous mass in the proximal and middle phalanx, with a firm consistency but mobile over the underlying structures; she had discrete erythema and bluish skin color without palpable pulse, thrill or bruit. She presented limited movement in flexion of the interphalangeal joint and fingertip paresthesia. There was no local hyperthermia or manifestations of systemic disease.
The Roentgenogram showed a soft tissue swelling of low density over the middle phalanx, with no bone alteration.
An ultrasound scan showed a well circumscribed isoechoic well defined ovoid mass, with a thin capsule and scarce septations, without alteration of the surrounding tissues (Figure ).
It was decided to perform an excisional biopsy with a zealous and careful dissection, with clear identification of the neurovascular structures and tendons, the tumor was nourished by two pedicles that emerged from ulnar and radial digital bundle branches (Figure ).
Pathology result reported as a lipoma that measured 53×25 mm with no neural component or malignant transformation (Figure ).
She had an uneventful postoperative course, followed by complete movement and function of the finger. The reported fingertip paresthesia disappeared.
There was no evidence of recurrence of the tumor during the 12 months of postoperative follow-up with excellent range of motion and sensivity (Figure ).


A 19-year-old male with no previous history of disease, in particular of migraine, was admitted at Valence Stroke Unit, France, following two episodes of transitory aphasia combined with acute headache and vomiting. This right-hander patient awoke on the day of hospitalization with an unusual frontal, moderate, and nonthrobbing headache combined with expressive aphasia. Clinical examination upon admission one hour later confirmed the moderate headache and one episode of vomiting. In contrast, the aphasic state regressed 20 minutes after its onset. Examination of the throat, spleen, and lymph nodes did not reveal any abnormalities. There was no evidence for an infectious episode, in particular of viral origin, and no sign of recent cranial traumatism. Emergency cerebral MR imaging (including T2*, diffusion-weight, and fluid-attenuated inversion recovery flight images) with intracranial and cervical MR angiography did not reveal any vascular or parenchymal lesions. Upon treatment with intravenous paracetamol, headache diminished in intensity but persisted and the patient kept vomiting. Five hours after onset of the symptoms, the expressive aphasia resumed. Two hours after-relapse of the aphasia, headache and nonfluent speech with reduced vocabulary persisted. There were no other obvious neurological symptoms and the patient was apyretic. An electroencephalogram revealed the presence of nonepileptiform slowing on the left hemisphere () that was consistent with the recorded clinical symptoms. Examination of the cerebrospinal fluid (CSF) found 250 lymphocytes/mL, normal glucose levels, and a minimal increase in protein concentration (0, 54 g/L). A diagnosis of encephalitis caused by herpes simplex 1 (HSV 1) was suspected and the patient was given acyclovir intravenously (10 mg/kg 3 times a day). However, a PCR performed on CSF with primers specific to HSV and EBV delivered negative results. In contrast, EBV serological test (EIA VIDAS, bioMérieux) detected isolated IgM VCA: IgM VCA index = 0, 65, IgG VCA = 0, and IgG EBNA = 0. Serological testing for a recent infection with CMV, VZV, HSV, hepatitis A, B, or C viruses, Lyme borreliosis, rickettsioses, syphilis, or HIV proved to be negative. Peripheral blood counts, C reactive protein, and liver function tests were within normal range. Clinical symptoms disappeared within 24 hours and acyclovir was discontinued upon reception of the negative PCR results, 24 hours later. The patient was discharged after 6 days of hospitalization and was reinvestigated six months later. No neurological signs could be noted with the exception of mild headache. Compilation of the clinical and laboratory features led us to pose a diagnosis of HaNDL. Serological controls at 4 weeks showed an unchanged profile with an IgM VCA index = 0, 31 and negative IgG VCA and IgG EBNA indexes.


A 20-year-old female patient presented with a request to discuss the options for prosthetic replacement of mandibular left first molar which was extracted 6 months back. Clinical examination revealed a mesioangular impacted tooth third molar which was advised to be extracted. After two months, the patient reported back again for the prosthetic replacement of the molar (Figures and ). There was no significant past or present medical history and oral hygiene was acceptable. Prior to the surgical procedure, the patient was instructed to rinse with 0.2% chlorhexidine gluconate (Hexidine, ICPA Health Products Ltd., India) for one minute. The surgical protocols emphasized complete asepsis and infection control. The type of implant used was an acid-etched, tapered self threaded implant (HI-Tec) of diameter 4.5 × 13 mm. Briefly, after induction of local anesthesia, a crystal incision was made along the crest of the ridge using Bard-Parker number 12 surgical blade, bisecting the existing keratinized mucosa (). A full thickness mucoperiosteal flap was raised buccally and lingually to the level of mucogingival junction, exposing the underlined ridge of the implant site, and a ridge alveoplasty was performed with the help of 0.2 mm round bur to achieve a flat bone surface of sufficient width (). A surgical drill guide was used for the precise placement of the pilot drill. After pilot drill application, the implant site was prepared with the corresponding size of parallel drill (). The implants were placed in the recipient site by means of an insertion device, and a torque driver set at 35 N cm which was used to evaluate primary stability of implant (). It was the level of torque that the manufacturer recommended to be applied to the implant. To detect and prevent unfavorable rotation of implant, the implant was secured through a mounting sleeve that allowed manual detection of rotation. The appropriate position of the implant neck in both the vertical and horizontal dimensions was decisive. The implant neck was positioned at the crystal bone level or slightly submerged (). Immediately after implant placement, the flap was replaced in its original position and sutured with nonresorbable suture (4-0) using a combination of inverted mattress and interrupted sutures. The inverted mattress sutures kept the bleeding edges of the flap close together, while the interrupted sutures sealed the edges. Intraoral periapical radiograph was taken immediately after surgery ().


A 37-year-old female patient presented with lumbar pack pain, and severe fever. She had a past medical history of recurrent renal-ureteric colics accompanied by fever, pelvic pain and meteoric bowels and multiple drugs allergy (ciprofloxacin, third-generation cephalosporin, and nonsteroid anti-inflammatory drugs). She reported the first menstruation when she was 10 years old, and following regular menses. Moreover she underwent cesarean section for fetal indication (fetal distress) during her only pregnancy. The urological examination found monolateral left positive sign of Giordano, left kidney area, and costovertebral angle tender to palpation. The ureteral trigger points on the left side were positive to deep palpation and the abdomen was painful but tractable.
Blood test showed neutrophilia and urine analysis showed >1.000.000 of colony forming units of E. coli.
Antibiotic therapy with amoxicillin + clavulanic acid 1 gr × 2/die e.v. and corticosteroid 4 mg/die i.m. was administrated.
Urologic ultrasound () showed left first degree hydronephrosis and the Uro-CT () scan confirmed the first degree hydronephrosis of the left kidney and showed a 15 mm long ureteral stenosis at the transition between the iliac and pelvic tracts.
Further investigation was done by Uro-MRI () which showed a ureteral hyperintense solid bulk of 12 mm below the bifurcation of the left common iliac artery.
Suspecting a rare form of endometriosis and according to gynaecology consultant, we performed dosage of tumour markers and hormonal levels, which showed the values reported in , with a detected increase only in 17 β estradiol value.
We addressed the patient to laparoscopic surgery, debulking the endometriotic-like tissue. A contemporary ureteroscopy and ureteral stenting was performed.
Histopathological findings suggested a diagnosis of endometriosis.
Macroscopically, the resected specimen was 1,5 cm in size. It was formalin fixed, paraffin embedded, and cut into 4 μm sections for the histological examination with haematoxylin and eosin stain. Microscopically, variably sized endometrial-type glands lined by a columnar epithelium embedded in an endometrial-like stroma were evident within muscular tissue (Figures and ). Immunohistochemistry demonstrated nuclear staining for estrogen and progesterone receptors (ER and PR) in the glands as well as in the endometrial stroma (). Also, CD10 stain was diffusely found in the endometrial-like stroma ().
The patient was discharged from hospital in 4 days postoperatively. Ultrasonography and blood examinations 15 days postoperatively were all within normal range. Stent removal was performed 3 months after surgery. At ultrasound control hydronephrosis had regressed completely.


An 18-year-old female with no known disease history was admitted to another center with complaints of coughing with sputum and weight loss. Chest x-ray and microscopic examination of the sputum enabled a diagnosis of smear-positive pulmonary tuberculosis. A four-drug regimen (INH, 300 mg/day; RIF, 450 mg/day; ethambutol [EMB], 1,250 mg/day; and pyrazinamide [PZA], 1,500 mg/day) was started according to her weight of 50 kg. While normal at the start of therapy (300,000/mm3), her thrombocyte count began to decrease during the second day of treatment (18,000/mm3). The therapy was stopped, and after transfusions of fresh frozen plasma and a thrombocyte suspension, her thrombocyte count became normal. Rifampicin was assumed to be the causative factor of thrombocytopenia, and therapy was restarted with INH, PZA, and EMB. However, thrombocytopenia relapsed during this regimen. The treatment was stopped and the patient was referred to our hospital.
When the patient was admitted to our hospital, her main complaint was coughing. No important previous history was present. Physical examination findings were normal with the exception of bilateral ecchymoses on her lower extremities. Chest X-ray showed a cavity with peripheral infiltration in the left upper zone. Laboratory findings revealed; sedimentation rate: 46 mm/hr, CRP: 24 mg/lt, WBC: 9,910/mm3, hemoglobin: 10 gr/dl, hematocrit: 31%, and thrombocytes: 135,000/mm3. Other parameters were noncontributory.
Because of the recurrent thrombocytopenia, we planned to start a treatment with a single agent and add the others one by one. The treatment was initiated in our hospital with INH at 300 mg/day, but this resulted in thrombocytopenia (30,000/mm3) on the third day, and the therapy was stopped. As soon as the thrombocyte count became normal, we began a three-drug regimen according to the patient’s weight: PZA, 1,500 mg/day; streptomycin (SM), 750 mg/day; and EMB, 1,250 mg/day. Because the thrombocyte count was normal (225,000/mm3), INH was again added to the regimen, but on the second day of INH initiation, the thrombocyte count decreased to 10,000/mm3. The INH was stopped and the patient underwent a consultation in the Hematology Department. Then a thrombocyte suspension and methylprednisolone (1 mg/kg/day) were given. During the follow up, the thrombocyte count became normal and the methylprednisolone therapy was stopped. After re-initiation of the three-drug regimen (EMB, PZA, and SM), RIF at 450 mg/day was added to the therapy as the fourth drug. On the third day of RIF therapy, the thrombocyte count decreased to 34,000/mm3 and RIF was ceased.
Because of thrombocytopenia caused by both INH and RIF, moxifloxacin at 400 mg/day was added to the treatment regimen. Without INH and RIF, patient had no further thrombocytopenia development during the period of therapy. After the first month of the therapy, her sputum smear was negative. There were no clinical or laboratory abnormalities during follow up examinations. After cessation of SM in the second month, the antituberculosis treatment regimen was continued and completed with three drugs (EMB, 1250 mg/day; PZA, 1500 mg/day; and moxifloxacin, 400 mg/day) without any further complications.


A 59-year-old woman presented to emergency department with dyspnea, which has gradually increased in three days. She did not complain of having any fever, cough, or sputum, but she had right-sided pleuritic chest pain. She has been taking warfarin for aortic and mitral valve replacement for 18 years and her INR levels were checked regularly. Additionally, she used 100 mg aspirin and an angiotensin receptor blocker daily. In her medical history, she did not have any systemic illnesses other than hypertension and she did not remember any kind of trauma during last month including any minor trauma.
Her body temperature was 36.8°C, blood pressure was 100/70 mmHg, oxygen saturation was 88%, and heart rate was 96 beats per minute; it was irregular with atrial fibrillation. On physical examination, her lung sounds were globally decreased at right hemithorax and she was tachypneic. Physical examination of other systems was unremarkable, except for metallic valve sounds which were heard on auscultation from aortic and mitral valves.
In her laboratory findings, her complete blood count, liver and renal function tests, and electrolyte levels were normal; serum β-human chorionic gonadotrophin was negative. Her INR level was 12.9 (0.8–1.2). The chest X-ray of the patient revealed a large pleural effusion in right hemithorax, which was not evident in her previous X-rays. The computed tomography of her thorax revealed massive right-sided hemothorax with mediastinal shift to the left side (Figures and ). Vascular structures were normal. No other mass lesions or infectious lesions were detected. Her medical history was asked again with particular attention to any trauma, and she denied having any trauma including minor trauma in the previous month.
A tube thoracostomy was performed for reexpansion of right lung and the patient was given 18 mg/kg fresh frozen plasma infusion simultaneously. 2000 cc blood was drained in 5 minutes by insertion of chest tube. Bloody drainage was confirmed via hematologic assay. She was supported with 1000 mL intravenous saline and no hypotension was observed except for a brief time interval during tube thoracostomy.
The patient was admitted to intensive care unit and decrease in bloody drainage was observed in following days. Samples of the pleural fluid were sent for cultures and Cytologic examination. The cultures of the pleural fluid and sputum revealed no infectious disease including tuberculosis. The Cytological examination was negative for malignant causes. Chest tube was removed after 5 days; there were no complications. She was discharged home, after appropriate dose adjustments for warfarin have been made. In her followup after one month, she had therapeutic INR levels and no effusion was seen in her chest X-ray.


Case 1. The first case involved a 70-year-old nonsmoking woman with a history of severe persistent asthma for 10 years including diabetes mellitus, hypertension, dyslipidemia, and ischemic heart disease. In 2011, she was referred to our allergy clinic with severe asthma. In the past 4 months, her asthma symptoms could not be controlled and asthmatic attacks always happened at night and worsened while she was sleeping. She was treated with fluticasone/salmeterol accuhaler (250/50 mcg) 2 puffs, twice daily; montelukast (10 mg); sustained release theophylline (200 mg) 1 tablet, twice daily; procaterol (25 mcg), 1 tablet, twice daily; salbutamol evohaler, 2-3 times daily for dyspnea. On physical examination, she was obese and presented expiratory wheezing and stridor in the supine position.
Chest radiography showed mild cardiomegaly, and parasinus radiography did not show sinus infection. Pulmonary function test (PFT) showed moderate restrictive lung disease and forced expiratory volume in the first second (FEV1) 59% predicted. To rule out cardiac problem, an echocardiogram was performed showing good left ventricular ejection fraction (LVEF) of 70% and marked concentric left ventricular hypertrophy. Additionally, chest dynamic multislice CT and CT virtual bronchoscopy were performed. Trachea showed collapse at the posterior wall (), with a crescent type without any intraluminal or extraluminal mass. Tracheomalacia was diagnosed and she was treated with continuous positive airway pressure (CPAP) 5 cm H2O. Her asthma symptoms had improved without nocturnal dyspnea. Three months later, she could use only the fluticasone/salmeterol accuhaler for controlling her asthma symptoms.


Case 2. In 2010, a 72-year-old nonsmoking woman with a history of hypertension and severe persistent asthma for 15 years was referred to our allergy clinic for further treatment with omalizumab (anti-IgE). Skin prick test showed positive results against house dust and cat. Total IgE was 523 U/mL (35–70) after treatment with omalizumab 300 mg taken every two weeks for four months. Her asthma symptoms remained partly controlled (asthma control test (ACT) score 20 and peak expiratory flow rate (PEFR) 180 L/min). She was treated with fluticasone/salmeterol accuhaler (250/50 mcg) 2 puffs twice daily; montelukast (10 mg); sustained release using theophylline (200 mg); tiotropium (18 mcg) once daily; salbutamal evohaler. In the first 3 months of 2012, her asthma symptoms were uncontrolled (ACT score 7 and PEFR 100 L/min). She developed acute asthma exacerbation and was treated with oral prednisolone (20–30 mg) and increased fluticasone/salmeterol accuhaler (500/50 mcg) 2 puffs twice daily and showed no improvement. On physical examination, she was observed to be obese and presented tachypnea and expiratory wheezing of both lungs.
Chest radiography showed mild cardiomegaly. PFT showed FEV1 35% predicted and no response to the bronchodilator. Echocardiogram showed LVEF 53% with no abnormal wall movement. We consulted an otolaryngologist to evaluate vocal cord movement and other upper respiratory tract obstruction. No vocal cord dysfunction or other obstructions were observed. Chest CT was normal while bronchoscopy showed that the right and left bronchus of lower lungs were narrow. Bronchomalacia was diagnosed as shown in . She denied with invasive treatment. She was treated with breath training exercise and given CPAP, 5 cm H2O at bedtime.


An 18-year-old woman was brought in by ambulance to the Emergency Department (ED) one hour after intentional ingestion of 60 VPA tablets (500 mg VPA, >600 mg/kg). Her medical history included partial symptomatic epilepsy and she was medicated with 500 mg per day VPA since two years.
On ED arrival, the patient was confused and somnolent. Her Glasgow Coma Scale (GCS) was 8. Vital signs included a temperature of 98.7°F, pulse of 129 beats/minute, respiration of 32 breaths/minute, blood pressure of 80/57 mmHg, and puls oximetry (SpO2) of %80 on room air. Pupils were equal at 2 mm and reactive to light. The cardiovascular examination revealed a regular rhythm with notable tachycardia without murmurs. The lungs were clear. The abdomen had positive bowel sounds and was soft, nontender, and with no hepatosplenomegaly. The extremities revealed no clubbing, cyanosis, or edema.
In the ED, she was treated with gastrointestinal decontamination with a single dose of activated charcoal and intravenous fluids resuscitation. Her blood VPA level at admission was 844 μg/mL (therapeutic serum concentrations range from 50 to 125 μg/mL) and ammonia level 325 μg/dL. Other laboratory values upon admission were within normal limits. Electrocardiogram revealed sinus tachycardia with no ischemic changes. Her initial chest X-ray and head CT were unremarkable. During hospitalization she was still confused and lethargic but required neither airway protection nor respiratory support.
She was taken to the intensive care unit for continuous monitoring and due to elevated VPA level (928 μg/mL). Her level of glucose was 64 mg/dL, so 25% dextrose 100 mL was administered. Levocarnitine treatment was started (50 mg/kg per day). Nephrology was consulted for evaluation for hemodialysis. Nephrology's opinion was that she could benefit from dialysis. Urgent high-flux hemodialysis (HD) with FX80 dialyzer (helixone, 1.8 m2, Fresenius Medical Care), blood flow rate 350 mL/min, and ultrafiltration rate 450 mL/hr for duration of 4 hours was performed, using right femoral venous access. After dialysis, her VPA levels dropped to 292 μg/mL. The serial VPA levels and laboratory studies were followed. Her VPA levels and other investigations are shown in .
The patient's mental status gradually improved back to baseline over 48 hours, accompanied by decreasing VPA levels (151 μg/mL). Her oral feeding was started on the 3rd day. VPA 500 mg per day was restarted on day 3 of hospitalization. After 3 days of levocarnitine, her phosphorous level fell to 2.2 mg/dL from 3.7 mg/dL before levocarnitine which was discontinued. Her phosphorous increased to 3.4 mg/dL (). By day 4, the patient's platelets count decreased to 79 × 109/L from 188 × 109/L and was normalized after 2 days without complications (). She was observed closely for any deterioration in consciousness along with VPA levels and laboratory values. By day 6, the patient's laboratory values had completely normalized, and she was transferred to an inpatient psychiatric facility for continuing therapy.


A 38-year-old male amateur, midfielder, soccer player was referred to the orthopaedic department of our institution due to persistent, aggravating right buttock pain during the previous month. He reported an insidious onset of discomfort as he had increased intensity and frequency of training, to participate in the upcoming season of local soccer league. Each practice session was based on plyometrics, strength, balance, jumping, plant and cut, bounding, and running exercises performed on a second-generation artificial turf field. Pain was exacerbated by activity and relieved with rest, worsening over time. No paresthesia or weakness to the leg was experienced. His past medical history was unremarkable, with no report of any recent or older injury.
On physical examination, a point of maximal tenderness was revealed above the midportion of his right buttock, in the area of the ipsilateral sacroiliac joint. Tenderness was also found in the right pubic ramus with extension to the pubic symphysis. Manual motor tests of both lower extremities demonstrated 5+/5 strength, with full range of joint motion. Sensation to light touch and pinprick were intact. An anteroposterior radiograph of the pelvis was performed; the findings of which were interpreted as normal. Subsequent MRI of the pelvis revealed the presence of a stress fracture in the right sacral ala ().
A diagnosis of a sacral fatigue fracture was established on the basis of both clinical and imaging data. According to our suggestions, the patient temporarily withdrew from sports activities, received analgesics, and underwent a six-week physiotherapy program focused on stretching of the posterior leg muscles, abdominal muscle strengthening, and core stabilization maneuvers. Running and soccer-specific training were then commenced with a gradual increase in the amount of practice in a weekly schedule. The patient resumed playing soccer 12 weeks later, with no residual or recurrent clinical complaints.


A 59-year-old woman presented in the ED with productive cough, malaise, and progressive weakness started a few days before. She had a history of breast cancer treated with mastectomy, chemotherapy, and radiotherapy about 6 years ago. She reported regular followup until 2010. Moreover, her medical history included blood hypertension and multinodular goiter with normal thyroid function. On initial presentation the patient showed normal blood pressure, tachycardia (160 beats/min) and tachypnea (respiratory rate: 38/min), oxygen saturation 91% on room air, and body temperature 37.6°C. Chest auscultation revealed reduced breath sounds with lower lobe crackles bilaterally. Jugular venous distention was detected at physical examination. The 12-lead EKG showed atrial fibrillation with elevated mean ventricular rate (160 b/min). Arterial blood gas analysis showed hypoxia (PaO2 62 mmHg) and elevated serum lactate (3.6 mmol/L). Laboratory data was significant for neutrophilic leukocytosis (WBC 19.8 × 109/L with 90% neutrophils), mild renal failure (creatinine 1.48 mg/dL), elevated D dimer (1314 ug/L), and procalcitonin (1.23 ng/mL). Chest radiography revealed bilateral nodular lesions, presumably secondary, above all in lower lung fields (). For suspected pulmonary embolism a venous duplex US scan was performed and deep venous thrombosis in lower limbs was excluded. During her staying in emergency room, the patient was treated with fluid therapy and broad-spectrum antibiotic therapy for sepsis. In clinical revaluation, the patient showed systolic hypotension unresponsive to fluid resuscitation, so she was treated with IV noradrenaline. Because of suspected pulmonary embolism an echocardiography was performed revealing normal left ventricular dimensions, normal systolic function, dilatation of left atrium, normal dimensions for both right atrium and right ventricle with presence of a hypoechoic mass involving tricuspid annulus, and right ventricle outflow tract obstructing the opening of anterior tricuspid flap (). Moreover, inferior vena cava appeared dilated and not collapsible. Echocardiography findings revealed suspicions of obstructive shock. Subsequently, chest ultrasonography was performed, detecting a consolidation in the right lung and confirming multiple rounded lesions involving the pleura bilaterally, compatible with metastasis (), and absence of interstitial pattern. A chest high-resolution computed tomography pulmonary angiography was performed, revealing absence of pulmonary embolism, presence of numerous nodular lesions with multiple cervical lymphadenopathies and an area of consolidation with air inside a cavitary lesion (lung abscess probably) () in the right upper lung lobe; moreover, a mass was detected pertaining to the pericardium with intracardiac extension at level of anterior right ventricle wall causing occlusion of tricuspid valve (). According to the serious medical conditions, we informed relatives about the poor prognosis and the patient was transferred to a medical division, where she died the day after.


A 45 year old male presented with acute onset rapidly progressive low backache, fatigue and weakness for past one month. On examination he had no organomegaly, lymphadenopathy, petechiae or purpura. There was no significant past and family history. There was no history of any cytotoxic drug intake. Peripheral blood examination revealed pancytopenia. Total leukocyte count was 3000/mm3 with a platelet count of 40000/mm3 and hemoglobin level of 7 gm/dl. Red blood cells were normocytic normochromic with minimal anisopoikilocytosis. Eight nucleated red blood cells were seen per hundred white blood cells. Occasional fully chromic tear drop cells were also noted. Differential leukocyte count comprised of blast 10%, polymorphonuclear leukocyte 65%, lymphocyte 23%, eosinophil 02%, basophil 0%. Blasts morphologically were myeloblast with an occasional auer rod. However cytochemical MPO stain on peripheral blood smear was negative.
Flow cytometric analysis was done on peripheral blood using Beckman Coulter FC 500 flowcytometer (Beckman Coulter, Miami, FL, USA). Gating strategy was SSC vs CD45. An antigen was considered positive if greater than 20% of blasts gated showed positivity for a specific monoclonal antibody. Antibodies used were CD19, CD10, CD34, anti-MPO, HLA DR, CD11c, CD14, CD4, CD8, CD3, cCD79a, cCD3, CD117, CD15, CD13, CD33 and CD7. Antibodies against CD41, CD61, glycophorin A could not be used as they were not available at that time. 4.1% blasts were gated which were CD45 positive (moderate) and had low side scatter. 46% of gated blasts were positive for CD34 (). Flow cytometry result is shown in .
Bone marrow aspirate was unsuccessful but bone marrow biopsy () revealed hypercellular bone marrow with panmyeloid proliferation. Dyspoietic megakaryocytes, immature myeloid precursors and erythroid precursors were seen. Numerous small megakaryocytes were noted with hypolobated and nonlobated nuclei and dispersed chromatin. Blasts were seen dispersed as well as in clusters. However no atypical localization of immature precursors was seen. Reticulin stain revealed a characteristic WHO grade III myelofibrosis ().
Immunohistochemistry was done on paraffin embedded sections using automated immunostainer (DAKO, CA, USA) based on standard streptavidin biotin-peroxidase complex technique. Antibodies used were antiCD61, anti glycophorin A, antiCD34 and anti MPO. Blasts were positive for CD34 () and negative for CD61, anti MPO and glycophorin A.
JAK2 mutation analysis was carried out and revealed a wild type mutant. Conventional cytogenetic analysis was carried out to rule out any abnormal cytogenetics. However no abnormality was detected.
The patient was treated as a case of acute myeloid leukemia with a 3+7 induction regimen of idarubicin (12 mg/m2) and cytarabine (100 mg/m2). This was followed by three consolidation cycles of HIDAC (High dose intermittent ARA-C; each cycle comprising of 4.5 g BD for three days) which led to marked improvement in symptoms. Bone marrow biopsy done for remission status after 45 days of starting HIDAC regimen revealed a dramatic reduction in fibrosis to grade I. Blasts were reduced to less than 5%. Patient is being planned for autologous hematopoietic stem cell transplant due to non-availability of HLA-matched donor.


Case 1-Epidural Hematoma (See and for the details)
A 48-year-old male patient experienced traumatic brain injury due to fall from a height of 3 m. Initial loss of consciousness was noted, but the patient remained conscious and alert after the insult. He went home and was found drowsy by a family member. He was then brought to our emergency department (GCS of 14), where head CT scan revealed a bilateral parietal EDH (40 mL in volume).
The 3D reconstruction enabled a minimally invasive approach to this case. A linear incision was made at the site where the center of the hematoma is located, and a 2.5-cm diameter craniotomy was created. Another 1.5 cm incision and 1 cm burr-hole was performed in the contralateral side, leaving the hematoma beneath the superior sagittal sinus untouched. After surgery the patient had an excellent recovery with no neurological deficits. He was discharged three days after admission to the hospital.


Case 2-Subdural Hematoma (See and for the details)
A male patient of 44 years old was brought to the emergency department by relatives after falling from a height of 2 m. As the patient was well after the fall, he did not seek medical attention immediately. When he was admitted to the emergency room he presented GCS of 14 with left hemiparesis with muscle strength grade IV. The CT scan showed a subacute subdural hematoma extending over the entire convexity of the right brain. Therefore, he was promptly sent to the operating room. Upon completion of the 3D analysis it was opted for an endoscopic treatment of hematoma by a burr-hole positioned adjacent to the hematoma. The surgery was performed with no complications, and total resection was achieved. The patient had an excellent recovery, reversing the preoperative deficits. He was discharged four days after hospital admission.


Case 3-Contusional Hematomas (See and for the details)
A 61-year-old alcoholic man was a patient who was hit by a motorcycle. Initially, he was alert and fully oriented. In the emergency department, the patient became disoriented; the examination revealed no signs of injury, and coagulation studies were normal. Within 30 min after the initial evaluation, his neurologic status deteriorated (GCS of 8). The CT scan revealed an acute right-sided post-traumatic intracranial parietal contusion containing an intraparenchymal hematoma, and acute subarachnoid hemorrhage in the right sylvian fissure. The patient underwent intubation and referred promptly to the operating room. Through the interpretation of the images 3D, we identify the best entry point for an endoscopic approach through a burr-hole. The patient had adequate recovery and was discharged seven days after surgery.


A 64 years old male with NF1 had multiple hospital admissions with abdominal and back pain between 2003 and 2004. He had lost significant amount of weight. As a part of work up he had MRI scan of the abdomen and pelvis. His scans showed a large pre-sacral mass in left retroperitoneal region with possible infiltrative characteristics. This mass extended up to left lesser trochanter and also had some calcification in it ().
A diagnosis of plexiform neurofibroma was made. In 2008 he had acute abdominal pain following trauma. There was possibility of a haematoma in the retroperitoneal mass on imaging. He had surgery with drainage of haematoma and debulking of retroperitoneal mass. He bled heavily during the surgery and required blood transfusion. The mass removed surgically showed diffuse involvement of nerve segment and its branches with tortuous expansion. Histology revealed spindle cell tumour infiltrating adjacent fat and entrapping nerve bundles ( and ). Tumour cells were positive for S100 protein immunostains. These features were consistent with malignant peripheral nerve sheath tumours. He was reviewed in genetic endocrine clinic. He appeared depressed and was overweight with a BMI of 29kg/m2. He had extensive bruising of skin on his arms and legs and his blood pressure was 160/100mmHg. He had no abdominal striae or proximal muscle weakness. His 24 hours urinary metanephrines and 5 HIAA were normal. His serum potassium, renal function and renal MRI scan was normal. He had a 2 cm enhancing mass in the wall of the second part of the duodenum representing neurofibroma. His gut peptides, chromgranin A and B were normal. His octreotide scan was negative.
He had overnight dexamethasone suppression test and his cortisol failed to suppress. He had low dose dexamethasone suppression test (LDDST) combined with CRH stimulation. At baselne he had measurable ACTH of 29ng/L with cortsiol of 689nmol/L. His cortisol was 427nmol/L on day three of low dose dexamethasone and his ACTH was 10ng/L consistent with ACTH dependent Cushing’s. After 100mcg of CRH there was no increase in cortisol or ACTH level. His ACTH precursors were high at 126 pmol/L (). A CT scan of his adrenals and MRI scan of the pituitary gland was normal. Patient did not have inferior petrosal sinus sampling in view of normal pituitary MRI scan, multiple neurofibromas and previous history of bleeding on surgery.
A diagnosis of Cushing’s syndrome due to ectopic ACTH was made. The source of ectopic ACTH was most likely his large retroperitoneal plexiform neurofibroma with MPNST. This patient was a poor surgical risk for further debulking of the plexifrom neurofibroma. He was treated with Imatinib mesylate at a daily dose of 75 mg/kg for 4 weeks.
He had repeat LDDST with CRH stimulation 4 months after the treatment. His cortisol suppressed to 27 nmol/L and ACTH was < 5ng/L on day 3. There was no response of cortisol or ACTH to CRH (). His MRI scan of the abdomen one year after treatment with imitinib showed no increase in the size of plexiform neurofibroma but there is a definite decrease in the density of plexiform mass. Immunochemical staining of the tumour did not show positive staining for ACTH.


A 12-year-old Japanese girl presented with normal birth and developmental history. There was no notable family or medical past history. At 2 years old, she experienced swelling of both knee joints without any cause. There was no rest pain in either knee; however, pain on motion in the right knee and joint effusion in both knees were noted at the first examination. Full extension was observed for the right knee but flexion was limited to 130°. The left knee had a normal range of movement. Aspiration of clear yellow joint fluid showed a negative culture result. Laboratory findings showed a white blood cell (WBC) count of 10,700/μL, C-reactive protein (CRP) of 0.45mg/dL, and erythrocyte sedimentation reaction (ESR) of 21mm/hour. Magnetic resonance imaging (MRI) revealed small masses in her knee joints (Figure ). Her symptoms were not improved after several joint aspirations. Arthroscopic synovectomy was eventually performed for both knees. During the operation, rice bodies and thickening of the synovial membrane were observed (Figure ). The pathological findings for the rice bodies showed acidophilic tissues with lymphoid infiltration (Figure ); however, no definite diagnosis was made. After the operation, the pain and swelling in the knees were improved. A 10-year asymptomatic period after arthroscopic synovectomy precluded the need for annual medical examinations.
When she was 12 years old, her left knee again showed swelling without any cause. Several joint aspirations and intra-articular injection of steroids failed to improve her symptoms. She did not experience any pain in her left knee. Full extension was observed for her knee but flexion was limited to 130°. Laboratory findings revealed a WBC count of 4,300/μL, CRP of 0.10mg/dL, ESR of 12mm/hour, rheumatoid factor (RF) of < 5IU/mL, matrix metalloproteinase-3 of 95.1ng/mL, and an antinuclear antibody (ANA) test was 1:80 positive. Contrast-enhanced MRI showed joint effusion and thickening of the synovial membrane (Figure ). An arthroscopic synovectomy was performed for her left knee (Figure ), and pathological findings revealed the presence of a villiform structure, increased blood vessels, chronic inflammatory cells, and lymphocyte infiltration (Figure ). She had no uveitis, but was diagnosed with JIA and received methotrexate (MTX) medication. Since that time there has been no recurrent knee arthritis.


A 71-year-old Caucasian man was referred to our facility due to sudden visual deterioration in his only functional eye (left). His vision was 0.2 in the affected eye and hand movement only with the right eye, which had been amblyopic since his childhood.
Anterior segment biomicroscopy results revealed a left conjunctival lesion in the superotemporal quadrant. On fundoscopy we noted a choroidal infiltration with exudative retinal detachment (Figure A,B,D,F).
Ocular and orbital imaging results revealed a choroidal mass (thickening of chorioretinal layer extending for 2.5mm) and two retrobulbar lesions around the optic nerve (6.6×4.4mm and 5.8×3.3mm) (Figure C,D).
Incisional conjunctival biopsy results revealed a B-cell lymphoma (CD20+, CD43+, bcl2+, CD3-). Additional examinations (blood films, computed tomography (CT) scans of the abdomen and thorax, and bone marrow biopsy) excluded other systemic locations of disease.
Due to the clinical presentation in the absence of any systemic extension of the lymphoma, we made a final diagnosis of a primary uveal lymphoma, which is generally an indolent tumor with a good life prognosis.
Our patient was referred for hemato-oncologic counseling and was initially treated with a systemic chemotherapy regime (CHOP, for ‘cyclophosphamide, hydroxydaunorubicin, Oncovin® (vincristine), prednisone’) with no effect; oral chlorambucil was then administered, with a good response (Figure ).
At 10 months after the onset of chlorambucil therapy, the best-corrected visual acuity in his left eye had improved to 0.4, the choroidal mass had practically disappeared and the retrobulbar lesions had regressed. At 12-month follow-up no systemic extension was noted.


A 49-year-old male presented with a two-day history of sudden, severe, right sided loin pain which was associated with vomiting. There was no history of any prior lower urinary tract symptoms. Past medical history included squamous cell carcinoma of the tongue for which the patient underwent partial excision and adjuvant radiotherapy and had no evidence of recurrence. Haematological investigations revealed deranged renal function with significantly raised serum creatinine. C-reactive protein was also elevated.
(Computed tomography) CT and (magnetic resonance) MR imaging demonstrated a solid lesion involving the lower two thirds of the right kidney (Figures and ). The left kidney was atrophic. The patient was initially managed with right JJ stenting, which resulted in normalisation of his creatinine. However, renal function began further deterioration, thus underwent right percutaneous nephrostomy tube insertion. Renal function normalised following this procedure.
As imaging demonstrated that this lesion was atypical, a renal biopsy was performed. Results were highly suggestive of a primary extramedullary plasmacytoma of the kidney (). Plasma electrophoresis for paraproteins was normal. In addition, both urinary Bence-Jones proteins and bone marrow biopsy returned normal.
Following multidisciplinary team discussion, it was decided that initial management was surgical, with an attempt to perform a partial nephrectomy. The procedure was challenging due to gross involvement of the surrounding tissues and tumour adhesion to the psoas muscle, which resulted in proceeding to right radical nephrectomy. Haemodialysis was required following surgery.
There was a smooth postoperative recovery. Histology demonstrated anaplastic extramedullary plasmacytoma of the kidney.


The bone marrow transplant recipient was a 13-year-old HCMV seropositive girl. The diagnosis of refractory cytopenia in July 2010 without genetic and molecular abnormalities was followed by bone marrow transplantation (BMT).
Before transplantation in March 2011 supportive treatment with transfusions and antibiotics was given. The non-myeloablative regimen with fludarabine (4x40 mg/m2), thiotepa (3x5 mg/kg KG) and anti-thymocyte globulin (3x20 mg/kg KG) was followed by transfusion of the marrow transplant from an unrelated 10/10 compatible HCMV seronegative (D-) donor. Neutrophil engraftment occurred very late at day +43.
After detection of an active HCMV infection at week six post transplantation, foscarnet was administered because of lower hematotoxic side effects (see Figure ). However, after one week this therapy had to be discontinued because of ulcera in the genital and in the oral mucosa. Due to the ongoing viral replication, at week 10, a therapy with foscarnet (180 mg/kg KG) and ganciclovir (10 mg/kg KG) was initiated for 3 weeks, resulting in a considerable decrease of the viral load in the blood. However, during this period the girl developed lung infiltrates consistent with the diagnosis of viral induced pneumonia. At this time, UL97 and UL54 genotyping revealed wild-type sequences. The virus carried the UL97 polymorphism D605E as a genetic marker, which is not associated with ganciclovir resistance [].
Foscarnet therapy was again discontinued at week 13 due to adverse side effects. After onset of an acute graft-versus-host-disease (GvHD), followed by an intensified immunosuppressive therapy, at week 25 a stem cell boost with selected CD34-positive peripheral blood stem cells became necessary, because of a secondary graft failure. During this period a substantial increase of the HCMV viral load up to more than 106 genome equivalents per ml (ge/ml) in EDTA blood occurred. UL97 genotyping revealed the emergence of HCMV variants with mutations, M460V and L595S, conferring GCV resistance. Additionally, due to an active adenovirus (species A) infection in August 2011 cidofovir and ribavirin were administered.
Since one cycle of cidofovir (5 mg/kg KG) did not result in a significant reduction of the HCMV viral load, a salvage therapy was induced which consisted of leflunomide (80 mg p.o. initially for the first two days, followed by 20 mg once per day) and maribavir (starting dose of MBV was 400 mg p.o. twice per day). The duration of treatment over five weeks resulted in an improvement and the patient experienced a temporary decline of the HCMV viral load to approximately 104 ge/ml. The virus load remained at that level.
A transfusion of adenovirus-specific donor-derived T-cells was performed in October (week 29). One week after termination of the salvage therapy, UL97 genotyping revealed that the M460V variant was no longer detectable. However, the L595S mutation was still present. Since the UL54 genotyping exhibited no resistance-associated mutation, foscarnet therapy was again initiated at week 32. At week 35 an increase in the virus load was observed associated with a relapse of pneumonitis. Therefore, a new adjustment of therapy with MBV (400 mg p.o. twice per day) was initiated. Two weeks later genotyping revealed a compilation of different UL97 and UL54 mutations. The UL54 sequences showed a mixture of T700A and A809V conferring potential low resistance to GCV and resistance to foscarnet, respectively []. In addition to the already known mutation L595S, a new mutation was detected in the UL97 region, namely the mutation H411Y which has been described to confer resistance to maribavir []. However, the combination therapy with foscarnet and MBV was continued due the low proportion of MBV resistant virus in the patient and the lack of alternative compounds.
At week 40 foscarnet was withdrawn. After two further weeks of MBV administration, UL97 genotyping exhibited an increase of the virus population carrying the mutation H411Y and further more, the emergence of a second mutation T409M also conferring MBV resistance [].
In order to solve the question whether the detected mixture of mutations consisted of different single, double or multiple HCMV mutants, UL97 amplicons were cloned into E.coli and individual clones were further analyzed. Sequencing revealed that all clones (n=33) carried the UL97 marker polymorphism D605E. Most of the clones carried wild type UL97 sequences (67%). However, certain clones carried one of the mutations L592S, H411Y, or T409M. As already reported by Strasfeld et al. [] in a solid organ recipient, none of the clones contained more than one single resistance mutation which indicates that the mutations were located on separate viral genomes. Surprisingly, one clone exhibited the mutation H411N also conferring MBV resistance which had not been detected by the direct sequencing of UL97 from the patient’s specimens. Regrettable, despite several attempts no virus isolates could be obtained.
Fortunately, the patient recovered from the HCMV and adenovirus infection. Until today she did not experience further serious virus associated events and can attend school.


An 18-year-old male was referred to our nephrology clinic for evaluation of azotemia and proteinuria in March 2011. The patient had previously undergone an operation to repair an anal fistula in December 2008 and an appendectomy to treat acute appendicitis in January 2009 at a local clinic. He was admitted to our hospital in June 2009 due to right upper quadrant pain and bloody diarrhea. Urinalysis revealed an absence of proteinuria and microscopic hematuria. There was no deterioration of renal function as determined by blood urea nitrogen (BUN, 5.9 mg/dL) and serum creatinine (0.9 mg/dL) analysis. Colonoscopic examination revealed linear, geographic ulcers with non-caseating granulomas in the distal ileum. The patient was diagnosed with Crohn’s disease and treated with 3 g/day of 5-aminosalicylic acid (5-ASA, mesalazine) and 9 mg/day of budesonide.
In July 2009, the patient visited the emergency room with right lower quadrant pain, loose stool, and fever. Simple abdominal X-ray showed free air below the diaphragm. He was diagnosed with an ascending colon perforation and underwent an emergency right hemicolectomy. Budesonide administration was discontinued, and the 5-ASA dosage was reduced to 2 g/day. The patient was additionally treated with ciprofloxacin (1 g/day) and metronidazole (1.5 g/day), and was kept under observation for 2 weeks. After this time, he had intermittent loose stools (0–2 times/day) without abdominal pain. Next, the patient was given 5-ASA (2 g/day) as maintenance therapy and his intestinal symptoms improved.
In December 2010, the patient experienced exacerbation of his symptoms, with non-bloody diarrhea three to four times daily. Colonoscopy revealed geographic ulcers consistent with Crohn’s disease. His blood pressure was 127/76 mmHg. Laboratory test results showed normal BUN (10.1 mg/dL) and serum creatinine (0.76 mg/dL) levels. Although urinalysis revealed mild proteinuria (1+ according to a dipstick test) and microscopic hematuria, the patient did not have a nephrology consultation. The patient was treated with 5-ASA (1 g/day) and budesonide (6 mg/day). After treatment, his abdominal pain ceased and no more loose stools were experienced until March 2011.
In March 2011, the patient experienced dark loose stools three times daily, and developed pale conjunctiva. He complained of facial puffiness and edema in his lower extremities. His blood pressure was 137/96 mmHg. Proteinuria (2+ by a dipstick test) and microscopic hematuria were detected by urinalysis. His renal function had deteriorated, with increased BUN (30.3 mg/dL) and creatinine (3.42 mg/dL) levels. He was referred to a nephrology clinic and admitted for evaluation.
His height was 170 cm and his body weight was 43 kg. A 24-h urine study revealed the presence of proteinuria (3,967 mg/day). Further serologic testing revealed a mildly increased IgA level of 492 mg/dL (reference, 70–400 mg/dL) along with normal IgG (670 mg/dL; reference, 700–1,600 mg/dL), IgM (55 mg/dL; reference, 40–230 mg/dL), C3 (126.7 mg/dL; reference, 90–180 mg/dL), and C4 (40.5 mg/dL; reference, 10–40 mg/dL) levels. Test results for anti-nuclear antibodies (ANA), anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane (anti-GBM) antibodies, anti-streptolysin O (ASO) antibodies, rheumatoid factor (RF), and cryoglobulin were all negative. Serologic tests for hepatitis B, hepatitis C, and human immunodeficiency viruses were also negative. Renal ultrasonographic findings showed that the kidneys were normal-sized, with increased cortical echogenicity.
Renal biopsy findings revealed nine crescent formations and eight scleroses in 17 glomeruli. Endocapillary hypercellularity was also present. Inflammatory cells had infiltrated into the interstitium. Mild tubular atrophy with interstitial fibrosis was observed by light microscopy. In the mesangium, immunofluorescence assays revealed 2+ diffuse fine granular staining for IgA and 1+ staining for IgM and C3. Electron microscopy revealed dense deposits in the mesangial area (Figure A, B). The patient was subsequently diagnosed with rapidly progressive IgAN. Two days after the renal biopsy was performed, serum creatinine levels increased from 3.9 mg/dL to 4.8 mg/dL. The patient was treated with prednisolone pulse therapy (250 mg twice daily) for 3 consecutive days; the dose was tapered to 1 mg/kg/day (Figure ). Planned intravenous cyclophosphamide therapy was canceled because the patient developed abdominal symptoms on the fifth day of steroid treatment. He had watery diarrhea 10 times daily, and laboratory data revealed that he suffered from metabolic acidosis caused by severe diarrhea. Blood gas analysis showed the following parameters: pH 7.24, PaCO2 22.4, PaO2 82.6, and HCO3 9.7. His serum chloride level was 121 mmol/L; BUN and serum creatinine levels were 89 and 3.88 mg/dL, respectively. The patient was treated with 5-ASA (2 g/day) and loperamide (6 mg/day). In addition, intravenous fluid therapy was administered to restore volume loss. The watery diarrhea subsided in 2 weeks, and the patient was discharged. He was further treated at the outpatient department with oral doses of deflazacort (48 mg/day) and cyclophosphamide (100 mg/day). In July 2011 (3 months after treatment), these doses were tapered to 24 mg/day and 50 mg/day, respectively. 5-ASA dosage was maintained at 2.0 g/day. The patient’s BUN level was 61.2 mg/dL, and his serum creatinine concentration was 2.5 mg/dL. Proteinuria was 2+ by dipstick test, and the spot urine protein-creatinine ratio was 1.8 mg/g.
In September 2011, cyclophosphamide was replaced with azathioprine (100 mg/day). One month later, the patient was switched from azathioprine to mycophenolate sodium (360 mg/day) because of bone marrow suppression. His intestinal symptoms were not aggravated, and renal functions were also maintained (33.8 mg/dL BUN and 2.32 mg/dL serum creatinine) while taking mycophenolate sodium (360 mg/day) and deflazacort (12 mg/day) during December 2011.
To evaluate pathophysiological characteristics common for the exacerbation of Crohn’s disease and IgAN, as well as possible mediators, we measured interleukin (IL)-17 expression in renal and colon tissues from the patient. We also compared renal IL-17 expression to that in tissues from cases of primary IgAN without Crohn’s disease. Immunostaining for IL-17 was positive in both renal tissue and exacerbated colonic ulcers from the patient (Figure C, D), in comparison, this staining was negative in renal tissues from a patient with primary IgAN (control; Figure E).


A 75-year old male, heavy smoker (70 p/y), was referred to our department for evaluation of persistent hoarseness and associated non-productive cough. He reported the onset of these symptoms two months before his admission. His past medical history included arterial hypertension, hypercholesterolaemia and a resected basal cell skin carcinoma of the neck. He was receiving daily atorvastatin (20 mg), ezetimibe (10 mg) and valsartan plus hydrochlorothiazide (80+12.5 mg). Additionally, he did not mention any immunosuppressive condition or any cytotoxic drug intake.
Chest examination revealed diffuse expiratory wheezing. The rest of physical examination was unremarkable. Initial laboratory testing was normal except for a slightly increased erythrocyte sedimentation rate (35 mm/h) and C-reactive protein (2.5 mg/dl). Standard serum biochemistry tests were within normal range. Spirometry showed decreased FEV1, FVC, FEF25–75% (57, 63 and 28% of predicted values), increased FEV1/FVC ratio (93% of predicted value) and normal FIVI thus representing a moderate restriction with associated small airways obstruction. Arterial blood gases while breathing room air showed mild hypoxemia (PaO2: 70 mmHg, PaCO2: 37 mmHg, pH: 7.44 and bicarbonate: 27.5 mmol/l). Chest computed tomography showed mid and distal trachea lumen stenosis. No lesion was noted in the lung parenchyma.
Fiberoptic bronchoscopy demonstrated prominent mucosal thickening at the left false and true vocal cord and the anterior commissure of glottis (Figure A), as well as irregular and mucosal surfaces protruding in mid and lower third of the trachea resulting in lumen stenosis (Figure B). Biopsies showed tracheal mucosa fragments that were lined by thickened squamous epithelium with mild hyperplasia and atypia and slight increase of mitotic activity in its basal half. There was no evidence of malignancy. Focally, the epithelial cells demonstrated intranuclear inclusions typical of herpes virus (Figure ). Similar findings were found in biopsies from lesions of vocal cords. Molecular testing using polymerase chain reaction (PCR) identified HSV type 1 in tissue specimens. Based on histopathology results, the patient received intravenous acyclovir (5 mg/kg every 8 hours) for 7 days followed by oral drug administration for the next two weeks. Three months after the completion of treatment, a repeat fiberoptic bronchoscopy showed substantial regression of the laryngeal and tracheal lesions. No evidence of HSV infection was noted in multiple biopsy specimens. The patient’s voice improved and annual bronchoscopic examination was recommended.


Mr. B. O’ D. is a 63 year old patient who was admitted to the Veterans Administration Boston surgical intensive care unit (ICU) in May 2011 following a hepatic resection for metastatic colon carcinoma. His comorbidity included hypertension, hyperlipidemia and post-traumatic stress disorder (PTSD). His habitual medications consisted of anti-hypertensives, a statin, benzodiazepines, a serotonin-reuptake inhibitor, and a phenothiazine antipsychotic. His six-hour intraoperative course was complicated by a blood loss estimated at 2–3 liters, associated with several episodes of hypotension. He was admitted to the ICU late in the day, planning to extubate his trachea in the morning when fully stabilized. However, every attempt to wean sedative medications resulted in uncontrollable agitation: he was severely delirious. A number of pharmacologic interventions were unsuccessfully attempted, including intravenous haloperidol and a continuous infusion of dexmedetomidine. It was subsequently learned that the patient had been consuming for some time substantial doses of unprescribed benzodiazepines. Subsequently, the combination of higher doses of lorazepam and an oral anti-psychotic allowed discontinuing continuous intravenous sedation. After two failed tracheal extubations due to a combination of respiratory failure and delirium, a tracheostomy was performed. The patient was eventually transferred to a rehabilitation facility after 19 days of ICU and 5 additional days on the general ward, with a tracheostomy tube in place. Mr. O’ D.’s case raises a number of relevant issues, including:
Delirium prolonged his ICU course, and resulted in the need for a tracheostomy. Overall, his hospital course was much longer than expected, and costs for his care were much higher than would have been otherwise for him, his family and society. Despite the violence of his delirium, he did not injure himself. The least recommended class of drugs for treating delirium, benzodiazepines, was instrumental for his clinical turn-around, although it did not resolve delirium per se. The newest and most expensive drug, dexmedetomidine, was inconsequential. A number of factors may have contributed to the onset of delirium, including hypotension, tracheal intubation, and benzodiazepine withdrawal. Although not reported in the literature to our knowledge, one wonders about the role of PTSD as a promoter of delirium.


A 71-year-old man was admitted to our observation with a five months history of a gradually enlarging pulsanting painful neck mass, localized in the left laterocervical side. He reported dysphagia and breathing difficulty since about four weeks. He did not report symptoms associated with cranial nerve deficit.
Intraoral inspection demonstrated a swelling in the tonsillar region without erosion of the mucosa.
Computed tomography (CT) scan, performed with a 64-row scanner (LightSpeed VCT, General Electric Medical System, Milwaukee, WI, USA), showed a well-defined isodense expansive lesion in the left PPS, which determined bulging of the oropharyngeal wall.
After the injection of contrast medium organo-iodized (Iomeron 350 mg/mL, Bracco Imaging, Milan, Italy), the mass showed inhomogeneous contrast enhancement () because of the presence of low-density foci corresponding to necrosis, suggestive of malignant lesion. The lesion displaced the ipsilateral internal carotid artery and the left internal jugular vein without evidence of invasion; the latter presented an expansive thrombosis extending distally up to the confluence with anonymous trunk ().
Different diagnostic hypotheses were formulated: pleomorphic adenoma, neurogenic tumour, and lymphnode pathology. A subsequent dynamic contrast-enhanced MRI with a 1.5 T unit (Gyroscan Intera, Philips Medical Systems, Best, The Netherlands) was performed with the head-neck coil to better characterize the mass and to assess the topographical relationship of the tumour with vessels and with neighbouring structures. MRI protocol included axial and coronal T2 (T2WI), axial and coronal fat-saturated T2, axial e coronal T1 (T1WI), and axial T1W fat-saturated postcontrast agent (gadopentetic acid and dimeglumine salt, Magnevist; Schering, Berlin, Germany).
MRI confirmed the presence of a well-defined rounded lesion localized in the left PPS. The lesion compressed the pharynx, and it had a cranial extension almost up to the Eustachian tube.
The mass presented a signal hyperintensity in T2WI and in T2WI fat-saturated sequences and a signal hypointensity in T1WI sequences.
Post-Gadolinium T1WI fat-saturated sequences showed an early inhomogeneous contrast enhancement of the lesion (). The multiplanar capability of MRI demonstrated ipsilateral vascular structures dislocation and a low signal intensity after contrast medium administration localized in the left internal jugular vein, suggestive for an extensive thrombus. It also revealed the relationship between the tumour and the ipsilateral sternocleidomastoid muscle, which appeared compressed but not infiltrated.
The fat tissue thickness appeared reduced. Multiple increased in size lymph nodes were detected behind the left mandibular angle and in the bilateral laterocervical spaces.
The patient underwent an incisional biopsy with multiple Tru-Cut needle biopsy which revealed a neoplasm composed of fusiform cells with small necrotic foci. Immunohistochemistry demonstrated strongly positive staining for neurone specific enolase (NSE), slightly positive staining for a smooth, actine, and positive staining for S-100 protein, consistent with malignant neurosarcoma or MPNST ().
CT total body examination showed no evidence of metastases.
The patient underwent a transcervical surgical excision of the tumour without any subsequent complication, followed by radiation therapy.


A 30-year-old male suffered complete body burns in a boiler explosion. He was admitted to the hospital eight days later and the diagnosis on admission was burns to 95% of the TBSA (3% superficial second-degree burns, 77% deep second-degree burns and 15% third-degree burns) complicated with whole-body burn wound sepsis. The patient had renal failure, anuria, an infection in both lungs, heart failure and severe hypoxemia. The patient received treatment with a ventilator, blood filtration, antibiotics, nutritional support and dressing changes. On day 22 following hospital admission, wound bleeding increased and airway and gastrointestinal bleeding was observed. The platelet levels of the patient were reduced to 41 ×109/l on day 24, giving an activated partial thromboplastin time of 54.3 sec and a plasma prothrombin time of 14.7 sec. The patient was in a light coma with pupillary asymmetry (left pupil 2 mm, right 4 mm). CT revealed multiple patchy areas of high density, particularly in the right temporal lobe. The bleeding lesion was irregular and 2.5 ml in size. There were no abnormalities in the ventricle and cistern. Following symptomatic treatment and fiber bronchoscopy for airway congestion, platelet injection, cryoprecipitation and packed erythrocyte infusion, the patient began to recover on day 29, with a platelet level of 112 ×109/l. On day 43, CT reexamination revealed hematoma absorption (). On day 50, the patient exhibited a marked improvement and was transferred to a general ward. The patient received multiple skin grafts and was discharged 3 months later.


A 54-year-old male, was admitted to hospital for a cough and throat pain 23 days after receiving alkali burns to the entire body. The diagnosis on admission was burns to 80% of the TBSA (20% superficial second-degree burns, 30% deep second-degree burns and 30% third-degree burns) complicated by burn wound sepsis and lung infection. Hypoproteinemia, anemia and a severely low platelet count were observed, the patient received wound dressings, escharectomy, antibiotic treatment, packed erythrocyte infusion, platelet injection and nutritional support. On day 12 following admission, the patient complained of a severe headache and dizziness, and his platelet level was found to be 38 × 109/l. The CT revealed cerebral hemorrhaging in the left occipital lobe, subarachnoid hemorrhaging and slight ventricular hemorrhaging. On day 14, a place of bleeding in the right lower limb appeared and CT revealed a new lesion in the right parietal lobe, however, a lumbar puncture revealed that the cerebral spinal fluid was normal. On day 16, the place of bleeding in the right lower limb was aggravated and CT revealed a new lesion in the left parietal lobe with an improvement to the subarachnoid and ventricular hemorrhages. On day 18, the patient fell unconsciousness with intermittent, paroxysmal, generalized tetanic spasms. CT revealed no changes in the left parietal lobe hemorrhage, but reductions in the right occipital lobe, subarachnoid and ventricular hemorrhages were observed. The platelet level was 108 ×109/l. On day 20 the patient regained consciousness, presenting with first-degree muscle strength in the right lower limb. On day 47, CT revealed a significant improvement to the hemorrhage of the left occipital lobe (). Further symptomatic treatments, including wound dressing, antibiotics, decompression and nutritional support, led to the patient being discharged three months later.


A 60-year-old woman with history of bilateral sensory-neural hearing loss for a few years, presented to our clinic for a second opinion regarding her musical hallucinations. Her musical hallucinations developed suddenly, one evening while trying to go to sleep, and approximately 10 months prior to presentation in our clinic. Her hallucinations were described as music playing from a radio at the back of her head, without further lateralization. She heard parts of popular songs, consisting of musical excerpts with well-formed vocals and accompanying instrumental music. There were songs she was able to recognize, but there were also songs she could not. However, she was able to sing parts of the songs or hum the tunes she experienced during her hallucinations, while her husband was often able to recognize those as popular songs. All of the songs she heard at some point in her life. Initially, she perceived the music only at night time while attempting to fall asleep, but within 4 months after onset, the musical hallucinations were present all the time. At times, one song would play repeatedly for 3 weeks before being substituted by another song. There was no variation in loudness. The patient was able to hear and follow conversations in her environment. Concurrent exposure to different tunes would not influence her musical hallucinations. She did not experience non-musical auditory hallucinations, visual, tactile, or olfactory hallucinations. She also had some dull frontal headaches and insomnia that at times interfered with daily activities. Eight years prior to presentation, she recalled onset of a rather monotonous bilateral tinnitus, and 3 years prior to presentation, she was diagnosed with mild to moderate sensory-neural hearing loss. She was advised to have hearing aids which she did not obtain due to apparent financial constraints. There was no history of head trauma changes in mood, energy, appetite, or cognitive functioning. Prior trials of clonazepam, quetiapine, and zonisamide did not improve her symptoms, and were subsequently stopped due to side effects. Neurologic examination was unremarkable except for bilateral, symmetric sensory-neural hearing impairment.
Complete blood count, complete metabolic panel, gamma-glutamyl transpeptidase, C-reactive protein, erythrocyte sedimentation rate, rheumatoid arthritis screen, serum vitamin B12, serum folate, iron, plasma homocysteine, and vitamin D levels were normal. Anti-nuclear antibody was positive at 1:80 dilution. Magnetic resonance imaging (MRI) of the brain with and without contrast was normal. Positron emission tomography (PET) scan was unremarkable. A 24-h video electroencephalogram (EEG) was unremarkable. Audiogram demonstrated moderate sensory-neural hearing loss. Video nystagmography showed paroxysmal up and left beating nystagmus both with right and left ear undermost with Dix–Hallpike testing, suggestive of central lesion. The patient was started on carbamazepine with some improvement of her symptoms after 6 months of follow-up.


A 22-year-old female presented with back pain and her primary care physician palpated a right-sided abdominal mass. Family history was positive for renal cell carcinoma in her grandmother diagnosed at the age of 63 years old but was negative for tuberous sclerosis. Computed tomography (CT) scan of the chest/abdomen/pelvis revealed a 8-9 cm enhancing right upper pole renal mass with suspicion for tumor thrombus into the renal vein and IVC (). There was no lymphadenopathy or pulmonary involvement. Magnetic resonance imaging (MRI) confirmed a Zincke Level II venous tumor thrombus into the inferior vena cava to approximately 3 cm below the hepatic venous confluence ().
Open right radical nephrectomy with IVC thrombectomy was performed with removal of right kidney with mass and tumor thrombus en bloc (). Transplant surgery was present and assisted with mobilization of the liver.
Her postoperative course was complicated by bilateral pulmonary emboli. On postoperative day number two the patient had tachycardia and tachypnea. CT PE protocol revealed diffuse pulmonary emboli bilaterally. Systemic anticoagulation with a heparin drip was initiated and localized infusion of tissue plasminogen activator (tPA) was performed. The patient remained in the hospital until stable from a cardiac and respiratory standpoint and was discharged home on oral anticoagulation with warfarin.
Pathology revealed malignant epithelioid angiomyolipoma (PEComa), stage pT3bNx, Fuhrman grade IV. There was 1–50% tumor necrosis and extensive lymphovascular invasion, with involvement of the right renal vein and inferior vena cava as noted above, as well as renal sinus and perinephric fat invasion. Surgical margins were negative. The patient was referred to medical oncology to discuss close surveillance versus potential adjuvant chemotherapy. Based on her pathology, she is at intermediate risk for disease progression and ultimately chosen surveillance.


A 41-year-old Hispanic female with a history of type 2 diabetes mellitus presented with four-to-five days of progressively worsening fever, headache, right eye pain, blurred vision, nausea, and vomiting. She also complained of shortness of breath and pleuritic chest pain over the last twenty four hours. On examination, a hypopyon was visualized in the anterior chamber of right eye, and bilateral crackles were heard on lung auscultation. No significant weakness or numbness was found on neurological exam. Complete blood count showed leukocytosis with neutrophilic predominance. Urine analysis was consistent with urinary tract infection. Her condition deteriorated at this point, and she became confused and disoriented. Cultures were obtained, and she was started on empiric intravenous antibiotics (Vancomycin and Zosyn). Ophthalmology service was consulted, and she was treated with intravitreal injection of vancomycin and ceftazidime. MRIs of her brain and orbits were obtained which showed inflammatory changes surrounding the right ocular globe, consistent with endopthalmitis (). There were also multifocal regions of increased FLAIR signal within the cortex consistent with septic embolic disease. CT of her chest revealed multiple cavitary lesions, likely secondary to embolic phenomenon (). Given the embolic involvement of lung, brain, and eyes, a workup to assess the possible source of infection was conducted. The patient underwent trans-thoracic and transesophageal echocardiographies which were both negative for endocarditis. She had no signs or symptoms consistent with thrombophlebitis (Lemierre's syndrome) as a source of embolic disease.
On the third day of admission, patient's urine culture grew Klebsiella pneumoniae. CT scan of the abdomen showed a left renal abscess, with the largest component in subcapsular location (). The patient underwent CT guided drainage of the renal abscess and culture of the collected specimen grew Klebsiella pneumoniae (serotype K1) which was resistant to most antibiotics except the carbapenems. She was started on intravenous meropenem (2 grams every 8 hours) for a duration of 8 weeks. After a 10-week complicated hospital course, which included ICU care, the patient recovered and was able to be discharged home. Follow-up imaging of her abdomen showed resolution of her previously seen renal abscess ().