Reversible extrapontine and central pontine myelinolysis presenting with extrapyramidal features.

摘要

Central pontine myelinolysis (CPM) is a potentially devastating complication of rapid correction of hyponatremia. Although spastic quadriparesis and dysarthria resulting from involvement of the basis pontis constitute the classic phenotype, extrapontine presentations are well documented. There is no specific treatment, and the prognosis is usually poor. We present a patient with behavioral changes and a movement disorder due to extrapontine evolving into pontine myelinolysis. He experienced a near complete recovery with supportive care.A 35-year-old man with initially unrecognized daily alcohol intake presented with unexplained jaundice, diminished verbalization, and delayed terse responses. His serum sodium (123 mEq/L) was corrected to normal in 48 hours with dextrose and 1/2 normal saline at a 60 cc/hr rate. Elevated transaminases, alkaline phosphatase, ammonia, and jaundice resolved within a week without specific intervention. Liver evaluation, including biopsy, provided no explanation for his illness.His neurological condition deteriorated over 3 weeks. Abulia progressed to mutism. His cranial nerve examination was normal. Motor examination revealed a symmetric akinetic rigid syndrome with intermittent resting and coarse action tremor, more prominent on the left. Occasional myoclonic jerks were observed. Extensor plantar responses were his only unequivocal pyramidal signs. He was unable to ambulate.