text:
Summary: Cytochrome B6-F complex Fe-S subunit
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Rieske protein Edit Wikipedia article
Rieske protein from cytochrome b6f complex. (PDB: 1vf5)
|1rie / SUPFAM
|OPM superfamily
|OPM protein
|Cytochrome B6-F complex Fe-S subunit, alpha helical transmembrane domain
crystal structure of cytochrome b6f complex from m.laminosus
Rieske proteins are iron–sulfur protein (ISP) components of cytochrome bc1 complexes and cytochrome b6f complexes and responsible for electron transfer in some biological systems. John S. Rieske and co-workers first discovered and isolated the proteins in 1964.[1] It is a unique [2Fe-2S] cluster in that one of the two Fe atoms is coordinated by two histidine residues rather than two cysteine residues. They have since been found in plants, animals, and bacteria with widely ranging electron reduction potentials from -150 to +400 mV.[2]
Biological function (in oxidative phosphorylation systems)
Ubiquinol-cytochrome-c reductase (also known as bc1 complex or complex III) is an enzyme complex of bacterial and mitochondrial oxidative phosphorylation systems. It catalyses the oxidation-reduction reaction of the mobile components ubiquinol and cytochrome c, contributing to an electrochemical potential difference across the mitochondrial inner or bacterial membrane, which is linked to ATP synthesis.[3][4]
The complex consists of three subunits in most bacteria, and nine in mitochondria: both bacterial and mitochondrial complexes contain cytochrome b and cytochrome c1 subunits, and an iron–sulfur 'Rieske' subunit, which contains a high potential 2Fe-2S cluster.[5] The mitochondrial form also includes six other subunits that do not possess redox centres. Plastoquinone-plastocyanin reductase (b6f complex), present in cyanobacteria and the chloroplasts of plants, catalyses the oxidoreduction of plastoquinol and cytochrome f. This complex, which is functionally similar to ubiquinol-cytochrome c reductase, comprises cytochrome b6, cytochrome f and Rieske subunits.[6]
The Rieske subunit acts by binding either a ubiquinol or plastoquinol anion, transferring an electron to the 2Fe-2S cluster, then releasing the electron to the cytochrome c or cytochrome f heme iron.[3][6] The reduction of the Rieske center increases the affinity of the subunit by several orders of magnitude, stabilizing the semiquinone radical at the Q(P) site.[7] The Rieske domain has a [2Fe-2S] center. Two conserved cysteines coordinate one Fe ion while the other Fe ion is coordinated by two conserved histidines. The 2Fe-2S cluster is bound in the highly conserved C-terminal region of the Rieske subunit.
Rieske protein family
The homologues of the Rieske proteins include ISP components of cytochrome b6f complex, aromatic-ring-hydroxylating dioxygenases (phthalate dioxygenase, benzene, naphthalene and toluene 1,2-dioxygenases) and arsenite oxidase (EC 188.8.131.52). Comparison of amino acid sequences has revealed the following consensus sequence:
- Cys-Xaa-His-(Xaa)15–17-Cys-Xaa-Xaa-His
3D structure
The crystal structures of a number of Rieske proteins are known. The overall fold, comprising two subdomains, is dominated by antiparallel β-structure and contains variable numbers of α-helices. The smaller "cluster-binding" subdomains in mitochondrial and chloroplast proteins are virtually identical, whereas the large subdomains are substantially different in spite of a common folding topology. The [Fe2S2] cluster-binding subdomains have the topology of an incomplete antiparallel β-barrel. One iron atom of the Rieske [Fe2S2] cluster in the domain is coordinated by two cysteine residues and the other is coordinated by two histidine residues through the Nδ atoms. The ligands coordinating the cluster originate from two loops; each loop contributes one Cys and one His.
- Rieske iron–sulfur protein, C-terminal InterPro: IPR005805
- Arsenite oxidase, small subunit InterPro: IPR014067
Human proteins containing this domain
- ^ Rieske JS, Maclennan DH, Coleman, R (1964). "Isolation and properties of an iron-protein from the (reduced coenzyme Q)-cytochrome C reductase complex of the respiratory chain". Biochem. Biophys. Res. Commun. 15 (4): 338–344. doi:10.1016/0006-291X(64)90171-8.CS1 maint: multiple names: authors list (link)
- ^ Brown, E.N. and Friemann, R. and Karlsson, A. and Parales, J.V. and Couture, M.M. and Eltis, L.D. and Ramaswamy, S. (2008). "Determining Rieske cluster reduction potentials". J. Biol. Inorg. Chem. 13 (8): 1301–1313. doi:10.1007/s00775-008-0413-4. PMID 18719951.CS1 maint: multiple names: authors list (link)
- ^ a b Harnisch U, Weiss H, Sebald W (May 1985). "The primary structure of the iron-sulfur subunit of ubiquinol-cytochrome c reductase from Neurospora, determined by cDNA and gene sequencing". Eur. J. Biochem. 149 (1): 95–9. doi:10.1111/j.1432-1033.1985.tb08898.x. PMID 2986972.
- ^ Gabellini N, Sebald W (February 1986). "Nucleotide sequence and transcription of the fbc operon from Rhodopseudomonas sphaeroides. Evaluation of the deduced amino acid sequences of the FeS protein, cytochrome b and cytochrome c1". Eur. J. Biochem. 154 (3): 569–79. doi:10.1111/j.1432-1033.1986.tb09437.x. PMID 3004982.
- ^ Kurowski B, Ludwig B (October 1987). "The genes of the Paracoccus denitrificans bc1 complex. Nucleotide sequence and homologies between bacterial and mitochondrial subunits". J. Biol. Chem. 262 (28): 13805–11. PMID 2820981.
- ^ a b Madueño F, Napier JA, Cejudo FJ, Gray JC (October 1992). "Import and processing of the precursor of the Rieske FeS protein of tobacco chloroplasts". Plant Mol. Biol. 20 (2): 289–99. doi:10.1007/BF00014496. PMID 1391772.
- ^ Link TA (July 1997). "The role of the 'Rieske' iron sulfur protein in the hydroquinone oxidation (Q(P)) site of the cytochrome bc1 complex. The 'proton-gated affinity change' mechanism". FEBS Lett. 412 (2): 257–64. doi:10.1016/S0014-5793(97)00772-2. PMID 9256231.
Further reading
- Iwata S, Saynovits M, Link TA, Michel H (May 1996). "Structure of a water soluble fragment of the 'Rieske' iron-sulfur protein of the bovine heart mitochondrial cytochrome bc1 complex determined by MAD phasing at 1.5 A resolution". Structure. 4 (5): 567–79. doi:10.1016/S0969-2126(96)00062-7. PMID 8736555.
- Huang JT, Struck F, Matzinger DF, Levings CS (December 1991). "Functional analysis in yeast of cDNA coding for the mitochondrial Rieske iron-sulfur protein of higher plants". Proc. Natl. Acad. Sci. U.S.A. 88 (23): 10716–20. doi:10.1073/pnas.88.23.10716. PMC 53001. PMID 1961737.
- Brandt U, Yu L, Yu CA, Trumpower BL (April 1993). "The mitochondrial targeting presequence of the Rieske iron-sulfur protein is processed in a single step after insertion into the cytochrome bc1 complex in mammals and retained as a subunit in the complex". J. Biol. Chem. 268 (12): 8387–90. PMID 8386158.
- Ferraro, D.J., Gakhar, L. and Ramaswamy, S. (2005). "Rieske business: structure-function of Rieske non-heme oxygenases". Biochem. Biophys. Res. Commun. 338 (1): 175–190. doi:10.1016/j.bbrc.2005.08.222. PMID 16168954.CS1 maint: multiple names: authors list (link)
- Mason, J.R. & Cammack, R. (1992). "The electron-transport proteins of hydroxylating bacterial dioxygenases". Annu. Rev. Microbiol. 46: 277–305. doi:10.1146/annurev.mi.46.100192.001425. PMID 1444257.
- Schmidt, C.L. (2004). "Rieske iron-sulfur proteins from extremophilic organisms". J. Bioenerg. Biomembr. 36 (1): 107–113. doi:10.1023/B:JOBB.0000019602.96578.78. PMID 15168614.
- Schneider, D. & Schmidt, C.L. (2005). "Multiple Rieske proteins in prokaryotes: where and why?". Biochim. Biophys. Acta. 1710 (1): 1–12. doi:10.1016/j.bbabio.2005.09.003. PMID 16271700.
- Brown, E.N. and Friemann, R. and Karlsson, A. and Parales, J.V. and Couture, M.M. and Eltis, L.D. and Ramaswamy, S. (2008). "Determining Rieske cluster reduction potentials". J. Biol. Inorg. Chem. 13 (8): 1301–1313. doi:10.1007/s00775-008-0413-4. PMID 18719951.CS1 maint: multiple names: authors list (link)
External links
- PDB: 1RIE - X-ray structure of Rieske protein (water-soluble fragment) of the bovine mitochondrial cytochrome bc1 complex
- PDB: 1RFS - X-ray structure of Rieske protein (water-soluble fragment) of the spinach chloroplast cytochrome b6 fcomplex
- PDB: 1FQT - X-ray structure of Rieske-type ferredoxin associated with biphenyl dioxygenase from Burkholderia cepacia
- PDB: 1G8J - X-ray structure of Rieske subunit of arsenite oxidase from Alcaligenes faecalis
- PDB: 2I7F - X-ray structure of the Sphingomonas yanoikuyae B1 Rieske ferredoxin
- PDB: 2QPZ - X-ray structure of the Pseudomonas Naphthalene 1,2-dioxygenase Rieske ferredoxin
- InterPro: IPR005806 - InterPro entry for Rieske [2Fe-2S] region
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Cytochrome B6-F complex Fe-S subunit Provide feedback
The cytochrome B6-F complex mediates electron transfer between photosystem II (PSII) and photosystem I (PSI), cyclic electron flow around PSI, and state transitions. This domain corresponds to the alpha helical transmembrane domain of the cytochrome B6-F complex iron-sulphur subunit.
Literature references
Stroebel D, Choquet Y, Popot JL, Picot D; , Nature. 2003;426:413-418.: An atypical haem in the cytochrome b(6)f complex. PUBMED:14647374 EPMC:14647374
Internal database links
|Similarity to PfamA using HHSearch:
This tab holds annotation information from the InterPro database.
InterPro entry IPR014909
The cytochrome b6-f complex mediates electron transfer between photosystem II (PSII) and photosystem I (PSI), cyclic electron flow around PSI, and state transitions. The cytochrome b6-f complex has 4 large subunits, these are: cytochrome b6, subunit IV (17 kDa polypeptide, PetD), cytochrome f and the Rieske protein, while the 4 small subunits are: PetG, PetL, PetM and PetN. The complex functions as a dimer.
This protein corresponds to the alpha helical transmembrane domain of the cytochrome b6-f complex Rieske iron-sulphur subunit.
Gene Ontology
The mapping between Pfam and Gene Ontology is provided by InterPro. If you use this data please cite InterPro.
|Cellular component
|thylakoid membrane (GO:0042651)
|Molecular function
|plastoquinol--plastocyanin reductase activity (GO:0009496)
|2 iron, 2 sulfur cluster binding (GO:0051537)
|Biological process
|oxidation-reduction process (GO:0055114)
Domain organisation
Below is a listing of the unique domain organisations or architectures in which this domain is found. More...
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Pfam Clan
This family is a member of clan TAT (CL0300), which has the following description:
This motif is found in a wide range of secreted proteins. It is named after the conserved pair of arginines that is followed by a hydrophobic stretch.
The clan contains the following 4 members:CytB6-F_Fe-S TAT_signal UCR_Fe-S_N UCR_TM
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|Sequence Ontology:
|Mistry J
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|Average length of the domain:
|36.90 aa
|Average identity of full alignment:
|42 %
|Average coverage of the sequence by the domain:
|19.55 %
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build method: hmmbuild -o /dev/null HMM SEED
search method: hmmsearch -Z 45638612 -E 1000 --cpu 4 HMM pfamseq
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Interactive tree
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There are 5 interactions for this family. More...
We determine these interactions using iPfam, which considers the interactions between residues in three-dimensional protein structures and maps those interactions back to Pfam families. You can find more information about the iPfam algorithm in the journal article that accompanies the website.
For those sequences which have a structure in the Protein DataBank, we use the mapping between UniProt, PDB and Pfam coordinate systems from the PDBe group, to allow us to map Pfam domains onto UniProt sequences and three-dimensional protein structures. The table below shows the structures on which the CytB6-F_Fe-S domain has been found. There are 15 instances of this domain found in the PDB. Note that there may be multiple copies of the domain in a single PDB structure, since many structures contain multiple copies of the same protein sequence.
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Infantile Spasms
OverviewInfantile spasms are a type of seizure seen in infancy that occur as the main feature of a seizure syndrome known as West syndrome. Though the terms West syndrome and infantile spasms are used interchangeably, infantile spasms is preferred. Infantile spasms generally consist of an acute onset of stiffening of the legs and arms while bending forward and may be asymmetrical. They are brief but tend to occur in clusters lasting several minutes or more at a time. They do not usually occur during sleep, but are often observed upon awakening in the morning or after a nap. They can be subtle and are sometimes confused with the Moro reflex or colic. Infantile spasms may appear as:
- Extension/stiffening of the trunk, arms, and legs
- Flexing at the waist, especially obvious when sitting
- Thrusting arms to side or across chest
- Repetitive head bobbing or nodding
- Drawing up of legs to the chest when lying down
Infantile spasms is diagnosed when an infant, approximately 3-6 months of age, has spasms that are present in clusters and in great numbers (up to 100 spasms in a cluster and sometimes many clusters a day), developmental delay, and a characteristic EEG pattern called hypsarrhythmia (high amplitude chaotic abnormal pattern). See EEG Example of Hypsarrhythmia (Epilepsy.com) for an example of this EEG pattern. Other seizure types are also seen in 30 to 50% of infants with this syndrome. Spasms usually stop as the infant gets older, but other seizure types often take their place (see the Prognosis section, below).
There are 2 kinds of infantile spasms, symptomatic and idiopathic, and they have dramatically different prognoses. In symptomatic infantile spasms, a specific etiology can be identified through history, physical exam, or testing. Tuberous sclerosis and perinatal asphyxia are the most common symptomatic etiologies. Other causes include cortical brain malformations, metabolic disorders (e.g., maple syrup urine disease, pyridoxine dependency, nonketotic hyperglycemia, phenylketonuria), chromosomal abnormalities, other neurocutaneous syndromes, infection, including prenatal infections (e.g., congenital CMV), and brain tumors. Of children with infantile spasms, 85-90% of children have the symptomatic type. [Pellock: 2010]
Infants with idiopathic infantile spasms have normal development and neurologic exams at the onset of spasms and a higher likelihood of "growing out" of them, particularly if the spasms respond well to medication. This suggests that early diagnosis and treatment may affect the outcome in children with idiopathic infantile spasms, although children who do and do not respond to medication may represent different populations that we are yet unable to otherwise differentiate. [You: 2009]
Some neurologists identify a third group, cryptogenic infantile spasms, in which children at the onset of the spasms are either delayed developmentally or have an abnormal neurologic exam at the onset of the spasms but no specific cause can be identified.
Other Names & Coding
Lightning spasms
West syndrome
G40.821, epileptic spasms not intractable, with status epilepticus
G40.822, epileptic spasms not intractable, without status epilepticus
G40.823, epileptic spasms intractable, with status epilepticus
G40.824, epileptic spasms intractable, without status epilepticus
Further coding details can be found by using the search feature at ICD10Data.com.
Prevalence0.15 to 0.2 per 1000 children 10 years of age or younger. [Mackay: 2004]
GeneticsMany of the causes of infantile spasms are genetic in origin; including tuberous sclerosis, metabolic disorders, and chromosomal abnormalities. The ARX and CDKL5 genes are associated with infantile spasms in some infants with cryptogenic infantile spasms. [Guerrini: 2007] [Bahi-Buisson: 2008]
The prognosis depends on the etiology of the spasms and whether or not there is developmental delay or an abnormal neurologic exam at the time of presentation. A good outcome is associated with a lack of identifiable etiology, normal neurologic exam and development at the time of presentation, older age at onset, and a relatively quick and complete response to treatment of the spasms. Most infants with infantile spasms have poor outcomes: 70-90% of children will have intellectual disability that is usually severe to profound, about 50% will develop some signs of cerebral palsy, and a smaller percentage of children will demonstrate autistic symptoms. [Partikian: 2009] [Mackay: 2004] Development may be improved with early, effective treatment, although the optimal regimen is not known. [Hamano: 2007] [Kivity: 2004] Between 40 and 60% of children with infantile spasms will develop Lennox-Gastaut syndrome, a refractory epilepsy syndrome of childhood. [Zupanc: 2009] The evolution from infantile spasms to Lennox-Gastaut syndrome may be decreased by early, effective treatment, although there is only circumstantial evidence for this. [You: 2009]
By group:
Treatment is more likely to result in complete or near-complete recovery in those with idiopathic infantile spasms rather than in those with symptomatic or cryptogenic infantile spasms. [Partikian: 2009] [Mackay: 2004]
Practice Guidelines
Go CY, Mackay MT, Weiss SK, Stephens D, Adams-Webber T, Ashwal S, Snead OC 3rd.
Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.
Neurology. 2012;78(24):1974-80. PubMed abstract / Full Text
Pellock JM, Hrachovy R, Shinnar S, Baram TZ, Bettis D, Dlugos DJ, Gaillard WD, Gibson PA, Holmes GL, Nordl DR, O'Dell C, Shields
WD, Trevathan E, Wheless JW.
Infantile spasms: a U.S. consensus report.
Epilepsia. 2010;51(10):2175-89. PubMed abstract
Hancock EC, Osborne JP, Edwards SW.
Treatment of infantile spasms.
Cochrane Database Syst Rev. 2008(4):CD001770. PubMed abstract
Roles of the Medical HomeAlthough treatment of infantile spasms will usually be initiated by a pediatric neurologist, the medical home clinician will provide ongoing care and monitoring for side effects during treatment. Concerns about developmental abilities will be addressed through the medical home; developmental monitoring and referral to outside agencies such as Early Intervention programs are crucial.
Clinical Assessment
OverviewPrimary care clinicians should consider a diagnosis of infantile spasms in an infant with unusual movements that cluster on awakening, especially if the infant already has developmental delay. If EEG confirms the diagnosis, an etiology for the spasms should be sought. Almost anything that causes brain damage can be associated with infantile spasms; an etiology can be found for about 3/4 of infants with infantile spasms. Etiology often determines prognosis for seizure cessation and for eventual developmental outcome. Specific therapies exist for a few of the underlying disorders including pyridoxine-dependent seizures and focal cortical dysplasias (treated with vitamin B6 and surgical resection, respectively). The Infantile Spasms Diagnostic Algorithm (Pediatric Neurology Division, University of Utah/Primary Children's Medical Center, Salt Lake City, UT) ( 53 KB) may guide the workup. The diagnostic evaluation should be tailored to the individual child, so not all studies are recommended for all children.
After they are diagnosed, infants with infantile spasms will undergo treatment with the goal of eliminating the spasms. Ongoing assessment will be needed to determine if the treatment is working and to monitor health, growth, and development during treatment. Assessment will vary depending on treatment. Children on high-dose steroids will need monitoring for side effects, including weight changes, blood pressure increases, etc. The frequency of follow-up appointments for infants who are undergoing treatment will vary depending on treatment choice and resources available. For instance, an infant receiving high-dose steroids should be seen at least weekly, though some of these visits may be replaced with a visit by a nurse during a home health visit.
Pearls & Alerts for AssessmentClusters on awakening
Infantile spasm clusters on awakening, whether in the morning or after a nap. If the family describes unusual movements in their infant with this pattern ,infantile spasms should be strongly considered.Infantile spasms and vaccines
Although it will sometimes seem to families that the onset of infantile spasms was caused by vaccine administration, there is no convincing evidence to support causality. [Willmore: 2009]Developmental assessments
Even a child who is developing typically when the manifestations of infantile spasms begin may show a slowing of milestone achievement or even regression. The medical home clinician should consider frequent developmental evaluation while infantile spasms continue. Developmental assessments may be available through Early Intervention programs (See Services below).
PresentationsAlthough spasms may begin as early as a month or 2 after birth or as late as 2 years of age, in most affected children they begin between 4 and 6 months and, in approximately 90%, within their first year of life. Characteristic symptoms include rapid muscular contractions of extremities in any of several patterns, including flexion, extension, and mixed patterns. These can be easily confused with other movements (e.g., Moro reflex, Sandifer syndrome). Individual spasms last only 1 to 2 seconds each and usually occur in clusters of from just a few to 100. Clusters may occur many times a day, are common when the child is awakening in the morning or after a nap, and may be accompanied by irritability and crying. The child's developmental trajectory often slows or reverses around the time of spasm onset; families may notice a decrease in developmental progress and a decrease in interaction with the environment.
Diagnostic CriteriaInfantile spasms refer to the seizures as described above and is often used somewhat interchangeably with West syndrome. To meet full criteria for West syndrome, the child must demonstrate the triad of spasms, intellectual disability/developmental delay, and hypsarrhythmia on EEG. See EEG Example of Hypsarrhythmia (Epilepsy.com).
Differential DiagnosisInfantile spasms are diagnosed by the appearance of the seizures (the seizure "semiology"), the characteristic age at which they appear, and distinct abnormalities on EEG. If an EEG obtained in a child with suspected infantile spasms is normal, the diagnosis should be reconsidered. Other entities to consider include:
The Moro reflex is a normal finding in infants up to about 3 or 4 months. When the infant is quickly moved, he/she will look startled, fling his/her arms out (palms up), and then draw the arms back to the body, and relax. See Moro reflex (MedlinePlus) and Moro reflex (picture) (MedlinePlus).
The bobble-headed doll syndrome is a movement disorder seen in children where there is episodic up and down or side-to-side movement at 2-3 Hz. This disorder is often associated with third ventricle tumors, aqueductal stenosis, communicating hydrocephalus and other structural brain disorders that can be diagnosed with brain MRI.
Spasmus nutans is a disorder seen in infants and young children. It consists of rapid, uncontrolled eye movements, head bobbing, and sometimes abnormal head positioning. Although an MRI of the brain is often performed to rule out brain abnormalities, a cause for this movement disorder is rarely found and it usually resolves without treatment after several months. [Dugdale: 2009]
Other seizure types - Although infantile spasms may appear similar to myoclonic and tonic seizures, they are distinct and will require different treatment. It may be helpful for the family to record episodes on video and to look for the initial contraction followed by a longer tonic phase that is typical for infantile spasms. Look for the combination of developmental delay and the characteristic EEG pattern.
Sandifer syndrome is body stiffening due to GE reflux, but may look similar to the tonic spasms seen in infantile spasms. Differentiating them may require additional workup, including a pH probe and an upper GI series. Sandifer's may be seen with severe reflux and lacks the EEG findings of infantile spasms.
History & Examination
Current & Past Medical HistoryEnsure newborn screening was performed and results are normal. Inquire about symptoms that might signal metabolic disease (e.g., unusual smelling urine, episodes of hypoglycemia or unresponsiveness, vomiting). Ask about any previous illnesses. Infants should be monitored for irritability, history of infections, and evidence of heart failure, including fatigue, listlessness, sweating or tiring easily while eating. Look for increased thirst and wet diapers as a sign of hydration.
Family HistoryInquire about a family history of neurocutaneous disorders, seizures, and developmental delay/intellectual disability.
Pregnancy/Perinatal HistoryInquire about problems during pregnancy that might have led to hypoxic ischemic encephalopathy such as intrauterine growth retardation, maternal illness, and toxin exposures. Ask about a history of, or symptoms of, CNS infection, possibly prenatal, such as CMV, herpes simplex, or meningitis/encephalitis. About 50% of infantile spasms cases are due to a prenatal cause. [Pellock: 2010]
Developmental & Educational ProgressIt is important to take a full developmental history. infantile spasms may interfere with developmental progress in children and may occur in a child with developmental delay. Development will need to be followed closely in the medical home.
Social & Family FunctioningAsk about finances, distance from pediatric medical care, and the presence of support systems - the care of a child with infantile spasms can put significant emotional and financial strains on a family. Coping skills and resources should be assessed periodically.
Physical Exam
GeneralCheck for dysmorphic features. Chromosomal and genetic abnormalities that have been associated with infantile spasms include trisomy 21, Miller-Dieker syndrome, CDKL5, and ARX mutations. Look for alertness, level of interaction with the environment, irritability, signs of infection
Vital SignsAs a baseline before medications, BP should be checked weekly for infants on high-dose steroids.
Growth ParametersHt | Wt | OFC Excessive weight gain may occur in infants on high-dose steroids, weight should be monitored weekly
SkinLook for unusual skin coloration abnormalities, especially the swirling pattern of hyperpigmented skin seen in Incontinentia Pigmenti (NINDS) or the hypomelanotic ash-leaf macules associated with tuberous sclerosis. These may be difficult to see in light-skinned infants, so at least one exam using a Woods lamp should be performed in all infants with infantile spasms.
Sensory TestingHearing and vision testing is recommended, especially in the setting of developmental delay.
Laboratory TestingIf no structural cause is found for infantile spasms, a battery of tests is generally ordered to look for a metabolic cause. These may include CBC with differential, plasma amino acids, biotinidase, lactate and pyruvate, anion gap, and urine for organic acids, uric acid, and others. A lumbar puncture (LP) will often be performed to test cerebrospinal fluid for cell count, protein, and glucose (with a near simultaneous blood glucose), lactate, amino acids, 5-Methyltetrahydrofolate (a form of folic acid), and pyridoxal 5-phosphate (a form of vitamin B6). These tests are aimed at identifying treatable metabolic disorders, infection, and glucose transporter deficiency. Screen for fecal occult blood and urine for glucose periodically in infants on high-dose steroids.
ImagingMRI of the brain (sedated) to look for signs of tuberous sclerosis, brain malformations, evidence of past hypoxia-ischemia, etc. Indicated if a genetic entity is thought to be responsible.
Other TestingEEG - wake and sleep. It is particularly helpful to have an EEG recording during the episodes thought to be infantile spasms. EEG demonstrates hypsarrhythmia, a very high-voltage chaotic pattern, or modified hypsarrhythmia, in a majority of cases. Multifocal spike discharges (MSD) are found in the remainder of EEGs. A follow-up EEG may be requested by the treating pediatric neurologist to assess treatment response.
Ophthalmologic assessment: If the infant is on vigabatrin, ophthalmological assessment to follow for retinal changes associated with vision loss is performed at about 3 month intervals after baseline evaluation.
Specialty Collaborations & Other Services
Pediatric Neurology (see NW providers [0])
Generally, children with infantile spasms should be evaluated and treated by a pediatric neurologist. Infants who are probably having infantile spasms may be admitted for the initial evaluation in order to expedite testing and treatment or if appropriate, infants may be seen urgently in an outpatient clinic. If you are considering infantile spasms as a diagnosis, contact pediatric neurology to arrange for evaluation as quickly as possible.
Pediatric Genetics (see NW providers [1])
A small percentage of children may have associated or causative genetic abnormalities. Genetic evaluation should be coordinated with Pediatric Neurology.
Developmental - Behavioral Pediatrics (see NW providers [1])
Referral may be helpful for developmental evaluation and to identify strategies for improving delays. Treatment of infants will generally be carried out in conjunction with pediatric neurology, and often by protocol. See ACTH Prescribing Protocol (Pediatric Neurology Division, University of Utah/Primary Children's Medical Center, Salt Lake City, UT) ( 41 KB) for an example.
Pediatric Ophthalmology (see NW providers [1])
Ophthalmology referrals may be helpful for diagnosis and monitoring of vision, and will be necessary if the infant is on vigabatrin.
Early Intervention for Children with Disabilities/Delays (see NW providers [2])
Infants should be enrolled in Early Intervention programs to follow and provide support for developmental progress.
Treatment & Management
Pearls & Alerts for Treatment & ManagementImmunizations timing and high-dose steroids
Immunizations should be postponed until at least a month after discontinuing high-dose steroids used to treat infantile spasms.Do not delay in treating infantile spasms
Complete cessation of seizures is the goal when treating infantile spasms. Developmental outcome may be better if the spasms are controlled quickly. [You: 2009]Treatments with known etiologies
A few causes of infantile spasms have specific treatments including pyridoxine-dependent epilepsy and glucose transporter deficiency.
Because of the prohibitive cost of ACTH, oral prednisolone has recently been investigated and may be equivalent to ACTH treatment with fewer side effects and lower cost. Doses greater than 3 mg/kg/day were more effective than doses 2-3 mg/kg/day, with effective doses between 40-60 mg/day. The regimen used by Kossoff et al., is 15 mg (5 ml 3 times) daily at first, increased to 4 times daily if necessary, then decreased to twice and then once daily at 2 week intervals. [Kossoff: 2009]
The UKISS study showed an infantile spasms cessation rate of approximately 70% with high-dose prednisolone compared to 76% with ACTH. [Lux: 2004] Infants treated with prednisolone had fewer adverse effects (53% compared to 80% with ACTH) and prednisolone was $200 for the course, whereas ACTH averaged around $70,000. [Kossoff: 2009] Other study groups have found similar results. [Azam: 2005]
ACTH at varying doses (ranging between 10 and 150 IU/m2 given either daily or every other day) has been the treatment of choice in the United States for many years and continues to be used despite its escalating cost. ACTH may be particularly useful in children who have infantile spasms due to hypoxic-ischemic encephalopathy. [Vigevano: 1997] Somewhere between 50 and 91% of children stop having infantile spasms with the 40 IU/m2 dose; a higher dose may be tried in infants who respond incompletely. ACTH is typically continued for 4 weeks after the spasms stop. [Mackay: 2004] If relapses occur, sometimes a second course of ACTH may stop the spasms again or another treatment might be tried.
ACTH must be given by IM injections and has many potentially serious side effects. Infants are usually started on GI prophylaxis (e.g., ranitidine) to prevent gastrointestinal bleeding and a low salt diet to prevent hypertension. Children are monitored by the medical home clinician, sometimes with the help of home health to check weight, irritability, blood pressure, fecal occult blood, and urine glucose. Possible side effects include weight gain, hypertension, metabolic abnormalities, ulcers leading to gastric hemorrhage, irritability, sepsis, osteoporosis, and heart failure caused by dilated cardiomyopathy. (Some series show up to a 5% mortality rate.) See ACTH Prescribing Protocol (Pediatric Neurology Division, University of Utah/Primary Children's Medical Center, Salt Lake City, UT) ( 41 KB) for an example of an ACTH protocol and see ACTH Manufacturer Information (ACTHAR gel). Also see Provider Letter for Child with Infantile Spasms (Primary Children's Hospital) ( 55 KB) for information for the primary care clinician when a child in his or her practice is started on ACTH.
In 2009, vigabatrin was licensed by the FDA to treat infantile spasms in infants 1 month to 2 years of age. Vigabatrin is thought to be more effective in infants with infantile spasms due to tuberous sclerosis, and in these cases, is often the first-line treatment even though there are no evidence-based recommendations for appropriate dosing. [Pellock: 2010] In a study where infants with tuberous sclerosis were excluded, after 2 weeks of treatment only 54% of infants responded to vigabatrin therapy compared to 70% and 76% receiving prednisolone or ACTH, respectively. [Lux: 2004] However, 12-14 months after initiation of treatment, percentages were similar in vigabatrin and hormone therapy treated groups. [Lux: 2005] Adverse effects were common, and excluding any effects on vision, occurred at similar rates with vigabatrin and hormonal treatments. [Lux: 2004] Because earlier control of infantile spasms may be correlated with improved developmental outcome, hormonal treatment may be preferable. [Lux: 2005]
Vigabatrin prescriptions are restricted to patients registered with the manufacturer due to a high incidence of vision loss in patients of all ages treated with this medication. Vision loss may be linked to taurine deficiency, and if this association is confirmed, may be amenable to dietary treatment in the future. [Jammoul: 2009] Current recommendations are that vigabatrin should be used at the lowest dose possible, for the shortest duration possible, and stopped if there is no observed benefit after 2-4 weeks of use. The goal of vigabatrin therapy, as with ACTH therapy, is cessation of infantile spasms. Dosing starts at 50 mg/kg/day in 2 divided doses and increases up to a maximum of 150 mg/kg/day, with a slow increase (25-50 mg/kg/day increments every 3 days) until maximized. Vigabatrin is given to families in packets of powder which they reconstitute depending on the dosage required. Vigabatrin is sent out directly to families from the pharmaceutical company program. Detailed instructions are given on the dosing information and the medical home clinician may need to ensure that families are giving the medication correctly. When stopping vigabatrin, the dosage should be tapered gradually. Vigabatrin Prescribing Information (Lundbeck SHARE Program)
All patients receiving vigabatrin need to be registered in the pharmaceutical company (Lundbeck SHARE) program due to concerns about its effects on vision. See Vigabatrin Prescribing Information (Lundbeck SHARE Program). This program mandates baseline vision screening, with retesting at 3-month intervals, or as recommended by the clinician, and after vigabatrin therapy has been discontinued, for all individuals receiving this medication. The pediatric neurologist, not the medical home provider, is responsible for arranging ophthalmology follow-up for infants receiving vigabatrin. In adults, vision loss usually manifests as permanent bilateral concentric visual field constriction, leading to loss of peripheral vision. Vision loss appears to be proportional to cumulative dose, but may continue after vigabatrin has been stopped. It is difficult to measure vision loss in infants, but the retinal defects thought to correlate with vision loss in adults are found, at the earliest, about 3 months after initiating treatment, making short term use seem relatively safe. [Willmore: 2009] Ultimately, 15-30% of infants treated with vigabatrin manifest retinal defects; this percentage is probably smaller than that observed in adults. [Gaily: 2009] [Willmore: 2009]
MRI changes have also been observed in some infants receiving vigabatrin for infantile spasms. These changes, which are of uncertain significance, involve increased T2 signal and a restricted, symmetric, diffusion pattern in the thalamus, basal ganglia, brain stem and cerebellum. There is no suggested screening for these changes at the current time. Vigabatrin Prescribing Information (Lundbeck SHARE Program) Other side effects including somnolence, fatigue, weight gain, edema, anemia, and peripheral neuropathy have been observed in adults on vigabatrin.
The ketogenic diet has been shown to be effective in studies by several different investigators. One study found it effective in fewer patients than ACTH (62% of infants became spasm-free with the ketogenic diet vs. 90% of those given ACTH), although side effects also occurred less often (31% with the ketogenic diet vs. 80% for ACTH). [Kossoff: 2008] Also see [Eun: 2006]. Since the nutritional content of infant formula is easier to control than that of a varied solid and liquid diet, the ketogenic diet is easier to manage in infants than in children. Further studies with variations of the ketogenic diet are necessary to further understand its role in treatment. [Kossoff: 2008]
High-dose topiramate, at least 6 mg/kg/day, may also be effective, with about 1/2 of infants becoming seizure-free. Adverse events occurred in approximately 39%. [Zou: 2008] Also see [Peltzer: 2009].
Newer antiepileptic medications and high-dose intravenous immunoglobulin are also being studied as possible treatments.
In conclusion, in infants without tuberous sclerosis, hormonal treatments appear to be superior to vigabatrin for achieving seizure cessation, with short-term success in about 3/4 of infants, compared to about 1/2 with vigabatrin. Excluding effects on vision, adverse effects were similar in the hormonal groups and the vigabatrin groups. Cessation of spasms may occur more quickly in infants treated with ACTH or prednisolone, which may have a beneficial effect on development.
Evidence for the use of prednisolone instead of ACTH for treatment of infantile spasms is mounting. Although they appear to be of similar efficacy and have about the same percentage of adverse effects, prednisolone is easier to administer and much less expensive. However, treatment with ACTH has been the gold standard and further studies would help clarify this issue further. Newer options such as the ketogenic diet and topiramate also need more study.
For example of a treatment algorithm, see Infantile Spasms Treatment Algorithm (Pediatric Neurology Division, University of Utah/Primary Children's Medical Center, Salt Lake City, UT) ( 67 KB).
Specialty Collaborations & Other Services
Pediatric Neurology (see NW providers [0])
Treatment will generally be initiated by pediatric neurology with collaborative monitoring for efficacy and side effects, as well as developmental progress, by the medical home.
Pediatric Ophthalmology (see NW providers [1])
Infants on vigabatrin need pre-vigabatrin treatment screening, subsequent screening, and post-treatment screening.
Development (general)
Specialty Collaborations & Other Services
Developmental - Behavioral Pediatrics (see NW providers [1])
May be helpful for developmental progress monitoring and management suggestions.
Early Intervention for Children with Disabilities/Delays (see NW providers [2])
Important for frequent monitoring of development and helping families support developmental progress.
Physical Therapy (see NW providers [0])
If Early Intervention is not adequate, may be helpful for patients who are not meeting gross motor milestones.
Occupational Therapy, Pediatric (see NW providers [0])
If Early Intervention is not adequate, may be helpful for patients who are not meeting fine motor milestones.
Speech - Language Pathologists (see NW providers [3])
If Early Intervention is not adequate, may be helpful for patients who are not developing language appropriately.
Issues Related to Infantile Spasms
Funding & Access to CareWriting Letters of Medical Necessity
Ask the Specialist
Is it safer to use vigabatrin or ACTH?
The choice of one therapy over another should be made only after a full discussion of benefits and risks with your child's physician. Although both these treatments have potentially dangerous side effects, the consequences of untreated infantile spasms are often devastating. You might consider joining a support group of parents who have made a similar choice to get a parent perspective. (See Support Groups under Resources, below).
Resources for Clinicians
On the Web
Genetics in Primary Care Institute (AAP)
Contains health supervision guidelines and other useful resources for the care of children with genetic disorders; American Academy of Pediatrics.
Helpful Articles
PubMed search for articles published in the last 2 years about Infantile Spasms
Nabbout R, Dulac O.
Epileptic syndromes in infancy and childhood.
Curr Opin Neurol. 2008;21(2):161-6. PubMed abstract
Shields WD.
Infantile Spasms: Little Seizures, BIG Consequences.
Epilepsy Curr. 2006;6(3):63-9. PubMed abstract / Full Text
Wheless JW, Gibson PA, Rosbeck KL, Hardin M, O'Dell C, Whittemore V, Pellock JM.
Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents.
BMC Pediatr. 2012;12:108. PubMed abstract / Full Text
Clinical Tools
Care Processes & Protocols
ACTH Prescribing Protocol (Pediatric Neurology Division, University of Utah/Primary Children's Medical Center, Salt Lake City, UT) ( 41 KB)
Infantile Spasms Diagnostic Algorithm (Pediatric Neurology Division, University of Utah/Primary Children's Medical Center,
Salt Lake City, UT) ( 53 KB)
A diagnostic algorithm for infantile spasms used by the Pediatric Neurology Division, University of Utah /Primary Children's Medical Center, Salt Lake City, UT. To arrive at this algorithm, recent literature was reviewed and division members came to consensus.
Patient Education & Instructions
Let's Talk About Infantile Spasms (Primary Children's Medical Center) ( 27 KB)
Infantile Spasms Information (NINDS)
Infantile Spasms (Epilepsy Foundation)
Parent focused information on infantile spasms.
Resources for Patients & Families
Information on the Web
Epilepsy Foundation
A national organization that provides information about epilepsy; programs to improve epilepsy treatment; materials to assist in helping people with epilepsy find jobs; activities in schools to educate the public; activities to educate policymakers; funds for research; links to find local and state resources; and news about conferences and other items of interest.
Infantile Spasms (NINDS)
Information from the National Institute of Neurological Disease and Stroke including diagnosis, treatment, and clinical trials in progress.
Infantile Spasms (Epilepsy.com)
Several frequently asked questions and answers about infantile spasms.
National & Local Support
Epilepsy Foundation
A national organization that provides information about epilepsy; programs to improve epilepsy treatment; materials to assist in helping people with epilepsy find jobs; activities in schools to educate the public; activities to educate policymakers; funds for research; links to find local and state resources; and news about conferences and other items of interest.
Services for Patients & Families Nationwide (NW)
|Service Categories
|# of providers* in:
|Partner states (6) (show)
|Developmental - Behavioral Pediatrics
|Early Intervention for Children with Disabilities/Delays
|Occupational Therapy, Pediatric
|Pediatric Genetics
|Pediatric Immunology
|Pediatric Neurology
|Pediatric Ophthalmology
|Physical Therapy
|Speech - Language Pathologists
For services not listed above, browse our Services categories or search our database.
* number of provider listings may vary by how states categorize services, whether providers are listed by organization or individual, how services are organized in the state, and other factors; Nationwide (NW) providers are generally limited to web-based services, provider locator services, and organizations that serve children from across the nation.
Azam M, Bhatti N, Krishin J.
Use of ACTH and prednisolone in infantile spasms: experience from a developing country.
Seizure. 2005;14(8):552-6. PubMed abstract
Bahi-Buisson N, Nectoux J, Rosas-Vargas H, Milh M, Boddaert N, Girard B, Cances C, Ville D, Afenjar A, Rio M, Héron D, N'guyen
Morel MA, Arzimanoglou A, Philippe C, Jonveaux P, Chelly J, Bienvenu T.
Key clinical features to identify girls with CDKL5 mutations.
Brain. 2008;131(Pt 10):2647-61. PubMed abstract
Dugdale, DC and Hoch, DB.
Spasmus nutans.
Medline Plus (NLM.NIH.GOV); (2009) http://www.nlm.nih.gov/medlineplus/ency/article/001409.htm.
Eun SH, Kang HC, Kim DW, Kim HD.
Ketogenic diet for treatment of infantile spasms.
Brain Dev. 2006;28(9):566-71. PubMed abstract
Fattal-Valevski A, Bloch-Mimouni A, Kivity S, Heyman E, Brezner A, Strausberg R, Inbar D, Kramer U, Goldberg-Stern H.
Epilepsy in children with infantile thiamine deficiency.
Neurology. 2009;73(11):828-33. PubMed abstract
Gaily E, Jonsson H, Lappi M.
Visual fields at school-age in children treated with vigabatrin in infancy.
Epilepsia. 2009;50(2):206-16. PubMed abstract
Go CY, Mackay MT, Weiss SK, Stephens D, Adams-Webber T, Ashwal S, Snead OC 3rd.
Evidence-based guideline update: medical treatment of infantile spasms. Report of the Guideline Development Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.
Neurology. 2012;78(24):1974-80. PubMed abstract / Full Text
Guerrini R, Moro F, Kato M, Barkovich AJ, Shiihara T, McShane MA, Hurst J, Loi M, Tohyama J, Norci V, Hayasaka K, Kang UJ,
Das S, Dobyns WB.
Expansion of the first PolyA tract of ARX causes infantile spasms and status dystonicus.
Neurology. 2007;69(5):427-33. PubMed abstract
Hamano S, Yoshinari S, Higurashi N, Tanaka M, Minamitani M, Eto Y.
Developmental outcomes of cryptogenic West syndrome.
J Pediatr. 2007;150(3):295-9. PubMed abstract
Hancock EC, Osborne JP, Edwards SW.
Treatment of infantile spasms.
Cochrane Database Syst Rev. 2008(4):CD001770. PubMed abstract
Jammoul F, Wang Q, Nabbout R, Coriat C, Duboc A, Simonutti M, Dubus E, Craft CM, Ye W, Collins SD, Dulac O, Chiron C, Sahel
JA, Picaud S.
Taurine deficiency is a cause of vigabatrin-induced retinal phototoxicity.
Ann Neurol. 2009;65(1):98-107. PubMed abstract / Full Text
Kivity S, Lerman P, Ariel R, Danziger Y, Mimouni M, Shinnar S.
Long-term cognitive outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone.
Epilepsia. 2004;45(3):255-62. PubMed abstract
Kossoff EH, Hartman AL, Rubenstein JE, Vining EP.
High-dose oral prednisolone for infantile spasms: an effective and less expensive alternative to ACTH.
Epilepsy Behav. 2009;14(4):674-6. PubMed abstract
Kossoff EH, Hedderick EF, Turner Z, Freeman JM.
A case-control evaluation of the ketogenic diet versus ACTH for new-onset infantile spasms.
Epilepsia. 2008;49(9):1504-9. PubMed abstract
Lux AL, Edwards SW, Hancock E, Johnson AL, Kennedy CR, Newton RW, O'Callaghan FJ, Verity CM, Osborne JP.
The United Kingdom Infantile Spasms Study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomised controlled trial.
Lancet. 2004;364(9447):1773-8. PubMed abstract
Lux AL, Edwards SW, Hancock E, Johnson AL, Kennedy CR, Newton RW, O'Callaghan FJ, Verity CM, Osborne JP.
The United Kingdom Infantile Spasms Study (UKISS) comparing hormone treatment with vigabatrin on developmental and epilepsy outcomes to age 14 months: a multicentre randomised trial.
Lancet Neurol. 2005;4(11):712-7. PubMed abstract
Mackay MT, Weiss SK, Adams-Webber T, Ashwal S, Stephens D, Ballaban-Gill K, Baram TZ, Duchowny M, Hirtz D, Pellock JM, Shields
WD, Shinnar S, Wyllie E, Snead OC 3rd.
Practice parameter: medical treatment of infantile spasms: report of the American Academy of Neurology and the Child Neurology Society.
Neurology. 2004;62(10):1668-81. PubMed abstract / Full Text
Nabbout R, Dulac O.
Epileptic syndromes in infancy and childhood.
Curr Opin Neurol. 2008;21(2):161-6. PubMed abstract
Partikian A, Mitchell WG.
Neurodevelopmental and Epilepsy Outcomes in a North American Cohort of Patients With Infantile Spasms.
J Child Neurol. 2009. PubMed abstract
Pellock JM, Hrachovy R, Shinnar S, Baram TZ, Bettis D, Dlugos DJ, Gaillard WD, Gibson PA, Holmes GL, Nordl DR, O'Dell C, Shields
WD, Trevathan E, Wheless JW.
Infantile spasms: a U.S. consensus report.
Epilepsia. 2010;51(10):2175-89. PubMed abstract
Although evidence-based guidelines were published in 2004, many questions remained about the diagnosis, evaluation, and management of infantile spasms. This article develops consensus guidelines regarding some of those questions.
Peltzer B, Alonso WD, Porter BE.
Topiramate and adrenocorticotropic hormone (ACTH) as initial treatment for infantile spasms.
J Child Neurol. 2009;24(4):400-5. PubMed abstract / Full Text
Shields WD.
Infantile Spasms: Little Seizures, BIG Consequences.
Epilepsy Curr. 2006;6(3):63-9. PubMed abstract / Full Text
Vigevano F, Cilio MR.
Vigabatrin versus ACTH as first-line treatment for infantile spasms: a randomized, prospective study.
Epilepsia. 1997;38(12):1270-4. PubMed abstract
Wheless JW, Gibson PA, Rosbeck KL, Hardin M, O'Dell C, Whittemore V, Pellock JM.
Infantile spasms (West syndrome): update and resources for pediatricians and providers to share with parents.
BMC Pediatr. 2012;12:108. PubMed abstract / Full Text
Willmore LJ, Abelson MB, Ben-Menachem E, Pellock JM, Shields WD.
Vigabatrin: 2008 update.
Epilepsia. 2009;50(2):163-73. PubMed abstract
You SJ, Kim HD, Kang HC.
Factors influencing the evolution of West syndrome to Lennox-Gastaut syndrome.
Pediatr Neurol. 2009;41(2):111-3. PubMed abstract
Zou LP, Lin Q, Qin J, Cai FC, Liu ZS, Mix E.
Evaluation of open-label topiramate as primary or adjunctive therapy in infantile spasms.
Clin Neuropharmacol. 2008;31(2):86-92. PubMed abstract
Zupanc ML.
Clinical evaluation and diagnosis of severe epilepsy syndromes of early childhood.
J Child Neurol. 2009;24(8 Suppl):6S-14S. PubMed abstract


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RICHMOND, Va. – Earlier this week CBS Sports released a chart that showed that the VCU men's basketball team had the pleasure of being the team that traveled the farthest for its game in the 2012 NCAA Tournament Round of 64, somewhere in the nature of 2,700 miles.
On Thursday night, the Rams decided to make the trip worth it with a thrilling 62-59 upset of fifth-seeded Wichita State in the second round of the NCAA Tournament. With the victory, VCU set a single-season school record for wins with 29.
"It was a great game. I thought coming into the game that Wichita State had one of the best teams in the country, and I still feel that way," Head Coach Shaka Smart said. "They played really, really well. But our guys hung in there and we certainly didn't play perfect basketball in the second half, but they really showed a high level of resolve."
Down 59-57 with two minutes left, senior Bradford Burgess drilled a three-pointer from the corner and junior Darius Theus made yet another big time play with a runner in the lane to cap off yet another NCAA Tournament upset for the Black & Gold.
"We talked about how we really needed to play with a lot of resolve and battle all night, even after Wichita State took the lead, our guys didn't put our heads down, they just kept fighting," Smart said. "And then these guys on this stage with me [Darius, Bradford and Troy Daniels] made some huge plays down the stretch, which turned out to be the difference in the game."
Burgess, who was making his 145th consecutive start, led all scorers with 16 points on the night. In addition, he grabbed five rebounds, four assists and picked up two steals. Theus and Daniels each finished with 10 points apiece, with Daniels tying a single-season school record with 92 treys on the season.
"It really feels great and it's a testament to the summer and offseason that I had with my teammates and coaches. They really pushed me to get better," Daniels said. "We don't stop working here, we're never satisfied."
Both defenses were on display early with the teams really scrapping for points and the Rams leading 8-7 with 14:09 showing on the first half clock. VCU quickly turned up the heat, forcing back-to-back turnovers and converting with layups from Theus and freshman Treveon Graham to take a five-point lead.
Wichita State showed why they were the 18th-ranked team in the country with an 11-0 run that gave the Shockers a 24-21 lead with 5:38 left in the first stanza.
VCU's resolve allowed them to bounce right back led by Burgess who scored nine of the Rams in a 13-1 run that closed out the first half and sent the Black & Gold into the break with a 34-25 cushion.
"We knew that Wichita State was going to make some runs, we just had to stick with our plan and battle through adversity," Burgess said.
The Shockers would slowly chip away at the lead during the second half and got it down to one, 54-53, with 5:41 to play, but Daniels bombed home his 92nd trey of the year to push the lead back to four. That's when Joe Ragland and Toure Murry answered with three-pointers of their own to give Wichita State a 59-57 lead and set up the final heroics by Burgess and Theus.
"Our coaches do an unbelievable job of keeping us focused on what we need to do to be successful, not just before the game, but during the game," Theus said. "I love this group of guys."
VCU will now move on to face the winner of fourth-seeded Indiana and 13th-seeded New Mexico State, which was being played right after the Rams' win over Wichita State. Game time on Saturday has yet to be determined.


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Faculty and Staff
Originally a teachers college, Southern places special emphasis on the quality of its faculty. The Southern graduate faculty represents the cream of worldwide academia, with far-ranging interests and scholarly achievements. About 70 percent of our faculty members hold doctoral degrees from the finest institutions in the United States and abroad. Their research and scholarship produces a steady stream of textbooks, professional journal articles, and other scholarly publications which you can find in universities and libraries around the world.


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Payday Cash Loan Expert: Personal Interview With Ryan Phillips of Relief, LLC
Payday loans have become a $40 billion industry where over 23,000 lenders trying to foster the idea that they are helping out cash-strapped borrowers by offering a payday cash loan when in fact the help really comes from people like Ryan Phillips of Relief, LLC, debt solution and loans consolidation specialists who know the ins and outs of settlement laws and negotiations and work with lenders on behalf of borrowers to stop harassing phone calls and get a fair and equitable settlement.
I caught up with Ryan at his offices in Sherman Oaks, a suburb of Los Angeles, and here's what he had to say about payday cash loans and Relief, LLC.
DH: What is a payday cash loan? RP: Payday Cash Loans are basically short-term, high interest loans.
They are usually obtained from either a walk-in storefront or from a company on the internet.
Most people get these high interest loans when they need help tiding them over to their next payday.
DH: Aren't they a good thing in today's economy? RP: This is a difficult question.
I believe there is a legitimate use for payday cash loans if used responsibly.
For example, if someone needs money to temporarily hold them over until their next payday and they are 100 percent sure they will pay the loan in full at that time, using this service would be less expensive than bouncing a check or an automated payment.
However, too often when the paycheck comes, people would rather pay only the interest and keep the rest of the money.
Before they know it, weeks, even months have gone by and all they've paid is interest payment after interest payment.
The problem is that payday lenders bank on the fact that most people won't pay the loan off quickly.
I think this plus the lack of regulation in the industry, especially with internet lenders, means that people are often given loans they should not be given.
We see clients that have 15 or 20 payday cash loans totaling over $10,000.
We see people who spend almost their entire paycheck just on interest fees to payday lenders.
Obviously this can only go on for so long before the person needs to declare bankruptcy.
So without some type of regulation to ensure that people do not get in over their heads then this type of behavior by payday lenders will likely continue to contribute to the current crisis of giving out too much credit.
DH: What would you recommend instead? RP: I would say try borrowing money from family or friends, if possible.
I know this can be emotionally difficult, but friends and family won't get you further in the hole.
For those who don't have friends or family they feel they can ask, maybe their employer can give them an advance.
It is in the employer's best interest because financial worries can cause stress both at home and on the job.
The most important thing is to be really honest with yourself about how much you owe and when you can pay the loan back -- then weigh your options.
DH: What can borrowers do if they get caught in the payday cash loan cycle of debt? RP: That is exactly why Relief, LLC was created.
We wanted to help educate people and to be an advocate on their behalf when they find themselves caught in this trap of getting one payday cash loan after another in an effort to pay each previous loan off.
At Relief, LLC we get into serious negotiations with payday lenders to give the borrower a little breathing room and to give them some time to get their loans paid off.
When and where possible we also get them a settlement on the loan to save them money.
This is the essence of our loans consolidation program.
DH: Can't borrowers just end the cycle on their own? RP: Of course, but it is very stressful to try and deal with professional collectors, especially the payday lenders who have structured their companies to be tough with people who don't pay.
And think about it.
The borrower is already stressed about not having the money to pay and now they have to deal with the frustration and muster the energy to haggle and negotiate with collectors; collectors who are trained to find any angle to bully, push and work the borrower until they feel they would be better off finding a way to pay just so they don't have to talk to the collector again.
Now consider that the borrower probably has five or ten of these loans.
It makes it very difficult.
DH: So how are the services of Relief, LLC, different from other payday loans consolidation companies? RP: Exceptional customer service.
We truly care about our clients and want to help them out of their financial stress.
If our clients are being harassed by a payday lender we respond immediately to get collection tactics stopped.
Plus we have an excellent track record of getting payday lenders paid off quickly.
A lot of other companies don't even pay the lenders until the end of a client's program which can be 6, 9 or 12 months long.
This can make things very stressful for the client in the meantime.
And we've been told we offer some of the lowest rates compared to our competitors.
For all these reasons our reputation as honest, caring and professional experts in getting payday cash loans resolved continues to grow.
com By Deborah S.


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Which led me to this post, how do you do it? Well for June's TSQL2sday I wrote a post all about using Policy Based Management (PMB) to try and enforce some sort of sensible naming convention for stored procedures. And we will follow a similar process here.
An important point to note here is the execution mode of each policy is determined by the characteristics of the facet that is used by the condition in the policy. The Autoclose option is available in several facets including Database, Database Option, Database Performance. I was hoping to implement a policy that prevents AUTCLOSE being turned on altogether but alas I don't think PMB is capable of preventing 'auto close' being turned on at the time the database option is changed, as the facets that include 'Autoclose' only have 'On demand', 'On Schedule', or 'On Change - Log Only' execution mode's available to them. Instead we will have to make to make alternative arrangements to be alerted when the option is set so we can correctly fix it before it becomes an issue. As you can see from my last AutoClose post, in a small number of circumstance some people have found a use for it but generally speaking it is better for 'auto close' to be turned off.
Create the PolicyI have talked about the steps involved in setting up Policies in a previous post, you can find that here. (http://www.gethynellis.com/2010/06/t-sql-tuesday-007-policy-based.html) so I will simply walk through creating the policy.
We have two options when it comes to creating the policy, we can use the management studio GUI or we can use TSQL. We will use the GUI for this demonstration but I will include the TSQL scripts at the end of the post for completeness, the scripts will be generated from Management Studio.
1. Connect to the instance in question in Management Studio
2. Expand the <Management> Folder, you will see 3 sub folders called <Policies>, <Conditions> and <Facets>.
3. We will start with the last folder, the <Facets> folder, expand the <Facets> folder and you will see a big long list of facets.
4. We will use the <Database Options> Facet. Right click on the <Database Option> and select <New Condition>
5. Give your condition a name, mine is called AutoCloseCheck
6. Ensure that the 'Database Options' Facet is selected.
7. In the expressions section click to add a clause.
8. In the Field Column, from the drop down list, pick @AutoClose
9. Ensure that operator is still '='
10. and change the value field to 'False' - thus ensuring that our condition checks for autoclose being set to false.
11. You can add a description in the description tab if you wish. When you're done, click <OK> and we have created the necessary condition to test.
Next we need to create a policy that tests for that condition:
12. Expand the <polices> folder.
13. Right-Click the policies folder and select <New Policy...>
14. Give you policy a name, I have picked the same name as my condition, mainly to avoid confusion so I have called it "AutoCloseCheck"
15. From the <Check Condition> drop-down list pick the condition we created above. in the <Against targets> box Every Database is selected. I'm going to leave that as the default setting as I want the policy to check on each database.
16.In the <Evaluation Mode:> drop-down list I picked the 'On-change: log only' I left the <Server Restriction> set to 'None'
17. In the description tab I simply enter a <description> and some <text to display>: 'Autoclose should be off '
We now have a policy created, so how will this policy help? Let's test the policy by changing the AUTO_CLOSE database option of one of many test databases to true. We can do this with the following script:
USE [master] GO ALTER DATABASE [Staging] SET AUTO_CLOSE ON WITH NO_WAIT GO
What do you expect to happen? That's right because our Facet Database Option does not support prevention we are only able to use the Execution Mode: On Change - Log Only which writes a useful error to the SQL Server log:
So when you come in the next morning and perform your daily SQL Server checks, or if you are very diligent as you should be and perform afternoon checks as well as morning checks on your database servers you will see this error, become very upset with your colleague, or more likely a DEV who has the necessary permissions on a DEV box or a third party supplier who setup the database and you can go and switch off the AUTO_CLOSE option and wrap the offending person on the knuckles...Hey its better than before, you know when it would be left like that until it becomes a problem.
There is something else that you can do though to make sure that you get alerted when the policy fires and writes out the log: You can use Alerts in the SQL Agent and get sent a nice warning email as soon as the database option is changes which means that you can fix the issue before it becomes a problem for you.
We can see from the screen shot above that my On Change - Log Only error was raised with a message number of 34053. The execution mode message number can be found in the following table:
Execution Mode Message Number
|Execution Mode
Message Number
|On Change - Prevent (automatic)
|On Change - Prevent (on demand)
|On Schedule
|On Change - Log Only
We need to setup an alert to look for message number 34053 and get the alert to notify us when it fires, this is how we do it:
- In SSMS expand the <SQL Server Agent>
- Expand the <Alerts> Folder
- Right-Click the <Alerts> folder and select <New Alert>
- Give your Alert a Name: AutoCloseCheck
- In the event alert definition leave <All databases> selected for <Database name>
- Make sure that Alerts will be raised based on <error number> is selected and change the error number to 34053
- On the response tab select notify operators and select who you want to alert when the error is raised, I use email, you can use whatever best suits your needs.
That's about it, we have looked at Policy Based Management and configuring it to log any changes to the database option AUTO_CLOSE and we have configured alerts to notify us of when that policy is violated so we can take action. The TSQL scripts (generated from SSMS) that can be used to recreate this can be found below. I'd be really interested to hear your thoughts on this, in particular if you used PBM and if you would use a different technique.
TSQL Scripts
These are the scripts generared by SSMS:
--Create the neccessary condition --script generated using SSMS Declare @condition_id int EXEC msdb.dbo.sp_syspolicy_add_condition @name=N'AutoCloseCheck', @description=N'', @facet=N'IDatabaseOptions', @expression=N'<Operator> <TypeClass>Bool</TypeClass> <OpType>EQ</OpType> <Count>2</Count> <Attribute> <TypeClass>Bool</TypeClass> <Name>AutoClose</Name> </Attribute> <Function> <TypeClass>Bool</TypeClass> <FunctionType>False</FunctionType> <ReturnType>Bool</ReturnType> <Count>0</Count> </Function> </Operator>', @is_name_condition=0, @obj_name=N'', @condition_id=@condition_id OUTPUT Select @condition_id GO --CREATE A POLICY using the auto close condition created -- Declare @object_set_id int EXEC msdb.dbo.sp_syspolicy_add_object_set @object_set_name = N'AutoCloseCheck_ObjectSet', @facet = N'IDatabaseOptions', @object_set_id = @object_set_id OUTPUT Select @object_set_id Declare @target_set_id int EXEC msdb.dbo.sp_syspolicy_add_target_set @object_set_name = N'AutoCloseCheck_ObjectSet', @type_skeleton = N'Server/Database', @type = N'DATABASE', @enabled = True, @target_set_id = @target_set_id OUTPUT Select @target_set_id EXEC msdb.dbo.sp_syspolicy_add_target_set_level @target_set_id = @target_set_id, @type_skeleton = N'Server/Database', @level_name = N'Database', @condition_name = N'', @target_set_level_id = 0 GO Declare @policy_id int EXEC msdb.dbo.sp_syspolicy_add_policy @name = N'AutoCloseCheck', @condition_name = N'AutoCloseCheck', @policy_category = N'', @description = N'Autoclose should be off ', @help_text = N'''Autoclose should be off ', @execution_mode = 2, @is_enabled = True, @policy_id = @policy_id OUTPUT, @object_set = N'AutoCloseCheck_ObjectSet' Select @policy_id GO --Turn on autoclsoe for a database. USE [master] GO ALTER DATABASE [Staging] SET AUTO_CLOSE ON WITH NO_WAIT GO --Create Alert to notify if autoclose is enabled USE [msdb] GO /****** Object: Alert [AutoCloseCheck] Script Date: 09/10/2010 09:35:43 ******/ EXEC msdb.dbo.sp_add_alert @name=N'AutoCloseCheck', @message_id=34053, @severity=0, @enabled=1, @delay_between_responses=0, @include_event_description_in=1, @category_name=N'[Uncategorized]', @job_id=N'00000000-0000-0000-0000-000000000000' GO


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Michigan Approves Union Curbs
The Republican-led Michigan legislature approved a pair of right-to-work bills, sending them on for the governor's expected signature, as thousands of union activists continued protesting outside the state capitol. Matthew Dolan has details on The News Hub from Lansing, Mich. Photo: Getty Images.
This transcript has been automatically generated and may not be 100% accurate.
... the ... thousands of protesters take to the streets in Michigan's capital of objections to Newquay is on duty and ... while a line of report from the state capital back and mall right here right now on the New Saab good afternoon and welcome I'm Simon Constable ... winning a straight to Lansing Wild Wall Street Journal's Matt Dolan is standing by on maps ... what's been going on ... what ... the inherent to capital land thing they they have represented after ... a pair of gold ... that are now on the taco grew in the right right to work which means that ... union which once were able to require that all the work or ... in a well presented ... workplace had to pay dues ... no longer have to do though in both the public and private sector here in Michigan ... for what deflation film in the signature of Republican Governor Rick Snyder ... the Fed last week ... for the first time that he would support of what Bush ... when she looking at video that you show us all of them ... that the protests today ... aam and this rather dank now aam ... in some states some firefighters and police have been excluded from ... from this legislation is not the same thing in Michigan ... I get what that what happened in magic in it and that the police and firefighters work clarity from the hard work what deflation McGovern argued gotten up ... yet last week that ... it would have occurred because of the need to combat medic labor strife affecting public safety worker ... what her political observers that the that that that was more and more of the GOP domestic am ... I when to come up ... with the boy in the effort to curb collector bargaining rights for public sector workers and others paid ... and police and fire were included in that it though to adapt their life ... or curtailed ... I've that would equate outcry ... people ... get killed ... off any produce worker spent ... the backlash was going to be or ... to make it to the wire with actually was ... the reason why ... I'm effort to ... curtail what the party right that day when them ... mountain out of ECB said protesters want this ... legislation not to be signed and handed it to be turned around what chance is now happening ... well they're couple of things that a Republican in the pit legislature have gone to try to prevent ... an immediate repeal of all one thing they did with that of spending provisions ... according to Michigan State law according to the two people but ... to ... that would mean that would be extremely difficult to have a voter referendum to ... repeal the legislation ... that would put unions and their Democratic allies and applying ... they'll have to walk onto other measures right now that what that would deflect are controlled by the GOP ... a lot of talk about turning twenty fourteen when Governor recover would be up for re-election if you live in them ... I'm numb ... the Michigan is long known as ... a sense of full organized labor aam did does does this change reflects a change in the mood of the whole state t think ... I'll be away from unions necessarily being a I'm you know ... a good thing ... but the really important question poured it because there's going to be a lot of different things happening in Michigan it did you can walk the walk and ... one of the pretty handily ... ah but then you look at the makeup of the bid offered to ... the state legislature the governorship ... and the Supreme Court all controlled by the GOP ... that the church have a lot of people think happened this year with an earlier proposition to try to untried collective bargaining rights of ... the state constitution ... opposition to what court by unions but was badly defeated in the November elections despite a victory for Democrats in the number of key congressional did well in the present ... an interesting interesting times in which we live thank you Brian much of a man selling in Lansing ...


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Egyptian cartoonist Ramsis Zakari was pronounced dead after a long battle with illness on Tuesday, 12 February evening, Egypt's state-run Middle East News Agency (MENA) reports.
He was 69 years old.
Zakari is considered one of the pioneers of the art of caricature in Egypt and the Arab world. His 1990s television show Ya Telefizyoun Ya (O Tv) is recognised as one of the most famous programmes of Egyptian television history.
Zakari's show was annually featured in Ramadan during the 1990s.
MENA confirms that Zakari's funeral is to take place Wednesday,13 February at Qasr Al-Dobara church located in Cairo's Tahrir Square, followed by funeral services and condolenses on Thursday, 14 February at the Evangelical Church in Cairo suburb of Nasr City.
Via SyndiGate.info
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At Applaws, making pet food matters, that's why we pride ourselves on creating cat and dog foods that are 100% natural. Applaws only sources its ingredients from natural and sustainable resources.
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Hanson developing into ideal middle infielder for Bucs
Shortstop prospect rounding into complete offensive threat in Minor Leagues
Pittsburgh Pirates shortstop prospect Alen Hanson has long been admired within the organization for having an outstanding attitude and work ethic.
Hanson is 5-foot-11 and only 152 pounds. He packs more punch and power than his wiry frame would seem to indicate.
Hanson, a native of La Romana in the Dominican Republic, has been playing in the Pirates' organization since he was 17. Now 20, the switch-hitter has a bright future with a club that is not very deep in shortstop prospects.
Hanson's teammate, South African Gift Ngoepe, similar in physical stature to Hanson, is playing with Hanson on the Arizona Fall League Scottsdale Scorpions team. Both play middle infield, but Hanson is a bit further advanced than Ngoepe.
Hanson, No. 3 on the Pirates Top 20 Prospects list, has had mixed results in parts of four seasons in the Minors.
He has played in 374 games and has been to the plate 1,630 times, not counting his Fall League experience. He has a career Minor League batting average of .292, with his best results coming in 2012, when he hit .309 at Class A West Virginia, and at the beginning of this past season.
Hanson hit 16 home runs in 2012. He also had 33 doubles and 13 triples among his 151 hits. He stole a career-high 35 bases, serving notice that he is a complete offensive threat with power and speed.
Hanson began 2013 at Class A Advanced Bradenton, where he hit .281 in 92 games. He hit 23 doubles, eight triples and seven home runs. He showed power, generated by quick hands and a good hitting approach, and he stole 23 bases.
At Double-A Altoona in the Eastern League, Hanson scuffled a bit. His batting average dropped to .255 against better quality pitching. He only hit one home run, giving him a total of eight for the season.
Hanson's statistics have always been far more favorable hitting as a left-handed batter against right-handed pitching. This past season, he hit .305 against righties and .224 against left-handed offerings. He had 112 more at-bats against right-handed pitching.
With his speed and ability to hit the gaps, Hanson can become a force at the top of the batting order. His hands are so good at the plate that he can get through the ball quickly enough to use the entire field and take pitches where they are thrown. Clearly however, he can do the most damage from his pull side batting left-handed.
In the Arizona Fall League, Hanson looks tired at the plate -- almost worn out. He struck out eight times in his first 38 at-bats. Pitchers are using every pitch in their arsenal, changing speeds and locations on him and fooling Hanson badly.
Defensively, Hanson has a history of being error-prone at shortstop. He doesn't have the strongest arm, and his initial step isn't the quickest, leaving him vulnerable at balls hit in the hole. He is fine on the average play and on coming in on ground balls, but he isn't as smooth and polished as he needs to become.
Showing solid upside as an offense-first middle infielder, given his combination of power and speed, I believe Hanson could be an ideal second baseman.
I think his offense would improve if he didn't have to be concerned with all the ground he has to cover at shortstop. His natural ability and good baseball instincts could play very well at second base.


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Yonkers is the 4th most populous city in New York state— behind New York City, Buffalo, and Rochester— and the most populous city in beautiful Westchester County. Yonkers borders the borough of the Bronx and is just two miles north of Manhattan. Fun fact: its name derives from the Dutch word for Esquire ("Jonkheer").
Has the time come for you to get out of the dorm and move into an off campus CSN apartment? Perhaps the options available from the Cochran School of Nursing housing office no longer meet your needs and you would like to find a cheap apartment close to the CSN campus. If so, you can search for off campus Cochran School of Nursing apartments with MyNewPlace's college housing search.
Although Cochran School of Nursing student housing may be very conveniently close to campus, moving to an off campus apartment can be a great way to save money, put some distance in between yourself and an RA, get accustomed to living in your own apartment and building up rental history.
CSN apartments for rent are available close to campus and in other neighborhoods around Yonkers, New York. Using our map based university apartment finder, you can find off campus housing near campus to ensure you can still get to all the CSN 0 games and make it to class on time.
We can help you find off campus CSN apartments that will best suit your collegiate lifestyle; search Cochran School of Nursing apartments by the number of bedrooms, price, neighborhood or a range of other amenities.
Have more questions? Check out our articles with renter tips in our apartment guide and search through http://www.cochranschoolofnursing.us/.