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Hypertension with recurrent focal deficits

© Author(s) (or their employer(s)) 2021. No commercial reuse. See rights and permissions. Published by BMJ. CLINICAL SCENARIO A 42yearold businessman had a 27year history of multiple complaints. He had been born of a nonconsanguineous marriage, but his siblings had youngonset hypertension. When aged 15 years, he developed an ulcerated skin lesion on the medial right thigh with right foot drop. This was presumptively treated as tuberculosis with corticosteroids and antituberculous therapy; the skin lesion resolved in 1–2 weeks and the foot drop recovered within 6–8 weeks. He stopped antituberculous therapy after 1 month. When aged 24 years, he developed a left foot drop that improved with oral corticosteroids in 3–4 weeks. However, on corticosteroid withdrawal he developed fever, left foot ulceration and abdominal pain. Biopsy of the skin ulcer was reported as ‘systemic lupus erythematosuslike’. His symptoms resolved with tapering corticosteroids over 4–6 months. He was also diagnosed and treated for hypertension at this time. When aged 30 years, he developed similar complaints. A rheumatologist prescribed monthly cyclophosphamide for 6 months with symptom resolution. He was then lost to followup for 6 years. When aged 36 years he presented with fever, abdominal and testicular pain, with skin ulcerations in the lower limbs. His viral markers for HIV, hepatitis B, hepatitis C were negative and a CT aortogram was normal. His condition responded well to pulsed corticosteroids and mycophenolate mofetil. He had further recurrent relapses every 1–2 years and tried a combination of multiple immunosuppressants: mycophenolate, azathioprine and cyclophosphamide. When aged 40 years, he had another relapse (fever, abdominal pain, left foot drop and weight loss) with significant (22 kg) unintentional weight loss over 2 months. CT scan of abdomen showed a left perirenal haematoma, with splenic and bilateral renal cortical infarcts. CT aortic angiogram showed multiple aneurysms in mediumsized abdominal aortic visceral branches. Chest Xray and twodimensional echocardiography were unremarkable. Upper gastrointestinal endoscopy identified oesophageal candidiasis, whereas colonoscopy showed multiple linear ulcers throughout the colon, the biopsy of which suggested cytomegalovirus colitis. He was managed conservatively with intravenous immunoglobulin along with antiviral and antifungal therapy and later received rituximab infusions (375 mg/m for 4 weeks). His foot drop persisted and nerve conduction studies showed an axonal pattern of sensorimotor involvement in the lower limbs (left >right). When aged 42 years he developed a 2month history of worsening subacute onset moderateintensity holocranial headache, with sudden onset of left haemiparesis and leftsided focal seizures. On examination, he had alopecia, multiple healed ulcerations below the knee, livedo reticularis in the distal foot without any organomegaly, a left haemiparesis with left appendicular cerebellar signs. Q1: What are the most likely differential diagnosis? Q2: What further investigations would you perform?