Small Intestinal Manometric Studies in Patients with Familial Visceral Myopathies

We studied jejunal manometry on 10 patients with type 1 familial visceral myopathy (FVM), and one patient each with types II and III. Two patients of type I and both patients of types II and III had intestinal pseudo-obstruction syndrome. The record was obtained in each patient for 4 to 5 hours during fasting, and 1 hour after feeding. In type I FVM, migrating motor complexes were present in six and absent in four patients. In these four patients (two with intestinal pseudo-obstruction syndrome) with absent migrating motor complexes, there was infrequent low-amplitude contractions during fasting, and after feeding. In six patients with migrating motor complexes, the motility indices of phases 2, 3, and fed period were 59%, 49%, and 24% of those of control subjects, respectively, and the frequency of contractions of phases 2, 3 and the fed period were 70%, 79%, and 32% of those of control subjects, respectively. In both patients with types II and III FVM, only infrequent low-amplitude contractions were recorded during fasting and after feeding. We concluded that intestinal contractions in patients with familial visceral myopathies were weak, and the weakness was more severe in patients with intestinal pseudo-obstruction syndrome.