Abstract
A 9-year-old girl presented with a 4-week history of right upper limb weakness. Clinical assessment and neurophysiological studies suggested an atypical brachial plexitis. She re-presented 312 years later with progressive muscle weakness involving both legs and left arm and hand. There had been no interval improvement in her right upper limb. Clinical, neurophysiological and pathological findings were consistent with chronic inflammatory demyelinating polyneuropathy. She responded to a single course of intravenous immunoglobulin—and review more than 6 years after treatment confirms that she remains functionally normal. Focal upper limb neuropathy preceding a diffuse demyelinating process by several years has not been previously described in a child. Long-term follow-up of this patient allows us to comment on the natural history of her condition and the apparent long-term efficacy of intravenous immunoglobulin in this case.
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