Journal of the neurological sciences · 2011
Multiple endocrine neoplasia type 1 presenting as refractory epilepsy and polyneuropathy--a case report.
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de Paiva Anderson Rodrigues Brandão, Castro Luiz Henrique Martins, Rodrigues Waldyr, Passarelli Valmir, Jorge Carmen Lisa, Brotto Mario Wilson I, Hirata Maria Teresa Alves, Marchiori Paulo Eurípedes
Аннотация
Hypoglycemia is a well recognized cause of acute symptomatic seizures. The fact that hypoglycemia can cause peripheral neuropathy is less appreciated. We describe a case of insulinoma associated peripheral neuropathy. A 17 year-old previously healthy man was referred for investigation of refractory epilepsy. A history of recurrent seizures, slowly progressive weakness of his feet and hands, and weight gain was obtained. Physical examination showed signs of a chronic sensory-motor polyneuropathy. He was diagnosed with insulinoma and primary hyperparathyroidism, characterizing multiple endocrine neoplasia, type 1 syndrome. Cases of insulinoma associated peripheral neuropathy are very rare. The more characteristic clinical picture appears to be distal weakness, worse in the intrinsic hand and feet muscles, and no or mild sensory signs. Peripheral nervous system symptoms may not completely resolve, despite removal of the cause of hyperinsulinism/hypoglycemia and full reversion of central nervous system symptoms. Mechanisms underlying hypoglycemic neuropathy are still poorly understood.