Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

Distigmine bromide improves chronic intestinal pseudo-obstruction in a case of MELAS

Yoshiharu Nakae, M.D., Hitaru Kishida, M.D., Yasuhito Hakii, M.D., Shigeru Koyano, M.D., Yume Suzuki, M.D. and Yoshiyuki Kuroiwa, M.D.

Department of Neurology, Yokohama City University, School of Medicine

A 34-year-old man with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) showed chronic intestinal pseudo-obstruction (CIPO), which was improved by the administration of distigmine bromide. He exhibited generalized tonic clonic seizures at the age of 21, and mitochondrial DNA analysis showed the MELAS mutation. At the age of 34, he became akinetic mutism after nonconvulsive status epilepticus and needed enteral nutrition through a nasogasrtic tube. However, he developed abdominal distention and vomiting, and was diagnosed as CIPO, therefore tube feeding was stopped. Although the administration of domperidone, mosapride citrate, butyric acid bacteria, sodium picosulfate, prostaglandin F2 alpha, pantothenic acid, dioctyl sodium sulfosuccinate, and so on, was ineffective, the administration of distigmine bromide improved his bowel motion disturbance and abnormal distention. The present case is the first MELAS patient with CIPO to be ameliorated by distigmine bromide, which might work acetylcholine receptor on the interstitial cells of Cajal.

(CLINICA NEUROL, 47: 177|179, 2007)
key words: mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), chronic intestinal pseudo-obstruction (CIPO), distigmine bromide, interstitial cells of Cajal (ICC)

(Received: 10-Aug-06)