Progressive Weakness, Cognitive Dysfunction and Seizures.

Answer: B A 45-year-old man developed recurrent partial seizures with secondary generalisation. Four years earlier, he had fi rst presented with diplopia from left abducens palsy and right hemiparesthesia, followed by left hemiparesis 1 month later. His magnetic resonance imaging (MRI) brain scan done then is shown in Figure 1. His MRI cervical spine with contrast was unremarkable for any cord lesion. The erythrocyte sedimentation rate, anti-nuclear antibody, antidouble-stranded deoxyribonucleic acid, anti-extractable nuclear antigens, anti-neutrophil cytoplasmic antibodies, anti-cardiolipin antibodies, lupus anticoagulant test, and cerebrospinal fl uid (CSF) analysis were unremarkable. He was treated with pulsed steroids and an immunomodulatory agent, but was lost to follow-up. On re-evaluation during the current admission for seizures, his neurological function had been deteriorating progressively over the past 4 years. He had severe cognitive defi cits (Mini-Mental State Examination 2/28), cerebellar dysarthria, and generalised pyramidal weakness with spasticity. Repeat MRI brain scan is shown in Figure 2. Serum calcium, vitamin B12, lactate, pyruvate, caeruloplasmin, rheumatoid factor, anti-thyroid peroxidase antibodies, antiglutamic acid decarboxylase antibodies, anti-gliadin IgA and IgG antibodies, human immunodefi ciency virus and syphilis serologies, and very long chain fatty acid levels were unremarkable. Ophthalmological examination excluded retinal vasculitic lesions or infl ammation; pathergy test was negative. Repeat CSF analysis was positive for oligoclonal bands. Computed tomography thorax showed no evidence of pulmonary sarcoidosis. He was treated with but did not respond to further courses of pulsed steroids.