Case history A 14-year-old girl was referred for inability to walk unaided forabout 5-6 weeks. Her symptoms had deteriorated over thecourse of a year. She was unable to hold objects, her hand-writing had become illegible and she was experiencing falls,clumsiness and poor academic performance. Over the last 5-6years there were multiple (5-6) episodes of weakness thatresolved within a week or two of onset. These were thoughtby her parents to be behavioural. There had been contactswith primary care during these episodes and blood testswere normal. She was referred for further investigationwhen her symptoms were worse and had lasted for morethan 2 weeks, unlike before. Examination revealed impaired depth perception, a broad-based ataxic gait, impaired coordination and a positiveRomberg test. There was no nystagmus and examination ofthe cranial nerves was normal. She had expressive aphasia.Motor system examination showed randomly distributedupper and lower motor neurone signs. Muscle tone wasreduced in the lower limbs with clasp knife rigidity and exag-gerated bilateral tendon knee reflexes. Ankle clonus was pre-sent. She had reduced proprioception in the lower limbs withintention tremor. Examination of extraocular movementsrevealed esotropia, impaired optokinetic response, left-sidedintemuclear ophthalmoplegia and diplopia on left-sided gazeat all points on the H-test.
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