title: [Distigmine bromide improves chronic intestinal pseudo-obstruction in a case of MELAS].
abstract: 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.
authors.affiliation: Department of Neurology, Yokohama City University, School of Medicine.
authors.forename: Yoshiharu
authors.initials: Y
authors.lastname: Nakae
authors.forename: Hitaru
authors.initials: H
authors.lastname: Kishida
authors.forename: Yasuhito
authors.initials: Y
authors.lastname: Hakii
authors.forename: Shigeru
authors.initials: S
authors.lastname: Koyano
authors.forename: Yume
authors.initials: Y
authors.lastname: Suzuki
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Kuroiwa
mesh_terms: D000328:Adult; D002908:Chronic Disease; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000188:drug therapy*; D017241:MELAS Syndrome / Q000150:complications*; D008297:Male; D011726:Pyridinium Compounds / Q000494:pharmacology / Q000627:therapeutic use*; D011950:Receptors, Cholinergic / Q000187:drug effects
chemical_list: D011726:Pyridinium Compounds; D011950:Receptors, Cholinergic; C084645:distigmine