[A case of myotonic dystrophy with intestinal pseudo-obstruction syndrome].

ABSTRACT

We experienced a patient of myotonic dystrophy presenting with intestinal pseudo-obstruction syndrome (IPOS). He was admitted to our hospital because of repetitive vomiting lasting for 24 hours. Abdominal CT revealed dilated stomach, duodenum and jejunum with extensive fluid residues. This patient was successfully treated with conservative therapy by using intravenous administration of metoclopramide. The etiology of IPOS in myotonic dystrophy is not well understood. In addition to normal findings of autonomic function tests such as R-R interval, Schellong test and sympathetic skin response, the efficacy of metoclopramide in treating IPOS suggests that acetylcholine (ACh) release is relatively preserved from the intestinal nerve plexus. Denervation hypersensitivity at the ACh receptors from long-standing dysfunction of autonomic system may be related to the cause of IPOS in myotonic dystrophy.