title: [The idiopathic paroxysmal myoglobinuric myopathy (Meyer-Betz) (author's transl)].
abstract: The idiopathic paroxysmal myoglobinuric myopathy (IPMM) as a genuine disease can be differentiated from other myoglobinurias by clinical criterias. Concerning the course of the disease two different types of IPMM are to be seen, sporadic cases are observed as well as familiar, autosomal recessive inherited ones. Regarding the pathogeny of IPMM several metabolic disorders are discussed: Provocation of the disease by physical exertion and glucose deficiency point to a disturbance of carbohydrate metabolism. Disorders of lipid metabolism are reported, too. So we tried to analyse the results of a case study in relation to the presently discussed - still hypothetical - pathogenetic ideas. Our laboratory, histological and electromyographic findings suggest a primary myopathy, which might be based on a peripheral glucose utilisation disorder (ATP-deficiency). Under the condition of exertion this disorder leads to a decompensation of the muscular functional and structural metabolism manifested by an excessive efflux of myoglobin and enzymes.
mesh_terms: D000255:Adenosine Triphosphate / Q000172:deficiency; D000328:Adult; D004576:Electromyography; D005947:Glucose / Q000172:deficiency; D006801:Humans; D008297:Male; D009212:Myoglobinuria / Q000175:diagnosis* / Q000209:etiology / Q000235:genetics; D010243:Paralysis / Q000175:diagnosis; D005082:Physical Exertion; D013577:Syndrome
chemical_list: D000255:Adenosine Triphosphate; D005947:Glucose
vernacular_title: Die idiopathische paroxysmale myoglobinurische Myopathie (Meyer Betz).