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Hereditary muscular diseases and symptoms from the gastrointestinal tract

Control of gastrointestinal (GI) motor function is complex. Contractions of the intestinal smooth muscles are integrated and modulated by both intrinsic and extrinsic nerves. The enteric nervous system in man is estimated to contain 10 neurons. There are numerous peptides involved in the regulation of the function of the GI tract, acting both as hormones and local transmitter substances. A subtle balance between the excitatory and inhibitory nerve activities as well as the neuroendocrine system of the gut is maintained under normal conditions. Impairment of any regulatory part may give rise to motility disturbances and GI symptoms. Abnormal GI motility can be attributed to myopathic or neuropathic disorders. Diseases giving rise to autonomic neuropathy, e.g. complications to diabetes and systemic neurological diseases, e.g. FAP (Familial Amyloidotic Polyneuropthy), are characterized by motility disturbances of the GI tract causing symptoms of gastroparesis, constipation/diarrhoea, malnutrition and/or malabsorption (1). In clinical practice, the gastroenterologist may meet patients with GI problems secondary to muscular or neurological diseases in two different clinical settings. On the one hand as a consultant in the neurology ward for patients with abdominal problems, on the other in the ordinary work with GI patients with an unknown or known muscular/neurological disorder as the underlying cause of the symptoms. It is therefore important to have a basic knowledge of this clinical problem. Diseases affecting the muscles of the GI tract could be of basically two types: a hereditary, sometimes systemic, disease (i.e. myotonic dystrophy), or an inflammatory disease (i.e. myositis or systemic sclerosis). This review deals with hereditary disorders affecting primarily the muscles of the patient, but in whom GI symptoms may as well be present. A comprehensive review of this problem was published in 1982 (2) and we will stress the new genetical findings (Table I) as well as therapeutical observations that could be of practical value.