Multiple endocrine neoplasia type 1 presenting as refractory epilepsy and polyneuropathy ?A case report

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• 作者：Anderson Rodrigues Brandã ; o de Paiva^a ; ^{arbrandao@oi.com.br} ; Luiz Henrique Martins Castro^b ; Waldyr Rodrigues Jr.^c ; Valmir Passarelli^b ; Carmen Lisa Jorge^b ; Mario Wilson I. Brotto^c ; Maria Teresa Alves Hirata^c ; Paulo Eurí ; pedes Marchiori^a
• 关键词：Refractory epilepsy ; Peripheral neuropathy ; Insulinoma ; Multiple endocrine neoplasia type 1
• 刊名：Journal of the Neurological Sciences
• 出版年：2012
• 出版时间：15 April, 2012
• 年：2012
• 卷：315
• 期：1-2
• 页码：172-175
• 全文大小：612 K

文摘

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.