Idiopathic Paroxysmal Myoglobinuria

Abstract

The liberation of myoglobin pigment from striated muscle and its subsequent appearance in the urine is a dramatic, potentially serious, and fortunately rather rare occurrence.Many types of traumatizing damage to muscle fibers may cause a partial or complete dissolution of the muscle sarcoplasm with the release of myoglobin and probably many other of the intracellular components. Some of these substances enter the circulation, and myoglobin, at least, is excreted by the kidney. Hence, myoglobinuria is not associated specifically with any single disease process but is a sign of severe damage to a significant mass of striated muscle.In addition to the traumatic myopathies, there have been reported a number of cases of myoglobinuria, usually recurrent and unassociated with any traumatic or toxic event.About one-third of these have been associated with muscular dystrophy. The remainder of the cases, 20 in number, have been idiopathic and unassociated with any specific or at least constantly recognizable disease process. This report deals with such a case, a man who throughout all of his adult life has suffered from recurrent major and minor attacks of myoglobinuria.Although the pathophysiological mechan¬ isms underlying this disease entity are still not understood, we have been able to con-duct a number of studies not previously reported on this condition. The results, to¬ gether with some recent reports on the physical chemistry of myoglobin, allow us to formulate a fairly clear picture of a number of the events which occur during the course of this disease. To add further emphasis to the potential seriousness of the disorder, our patient, as have others, de¬ veloped acute renal shutdown following his induced acute attack. Recovery from this was fortunately most satisfactory. Report of a CaseA 57-year-old white male office worker of Scotch and American Indian ancestry was first admitted to the Wadsworth Veterans Administra¬ tion Hospital on Feb. 16, 1955, with mild symp¬ toms and chemical evidence of diabetes mellitus.History revealed that he was perfectly well and healthy until the age of 20. Muscular effort in¬ cluding strenuous wrestling in matches prior to this time cause him no discomfort. While in France, in 1918, (age 20) he began to experience aching and cramping in the calves following moderate forced marching with a light pack. On the third such occasion the cramps were so severe that he had to be carried back to the camp.After this episode he noted that any muscle or muscle group would react in the same way with pain, tenderness, and cramps following heavy physical exertion. The cramps would last up to 12 hours and the pain and tenderness up to 48 hours. The quadriceps muscles were most fre¬ quently affected. In 1922, following heavy lifting, he developed lumbar pain and muscle cramping which was followed by the passage of dark red urine for the first time. On many occasions since then he has experienced this sequence of events, and the passage of red urine has followed overexertion of any significant bulk...

Spontaneous Leg Cramps and “Restless Legs” Due to Diabetogenic Hyperinsulinism: Observations on 131 Patients*

“…One such male attended by the writer (not included in the present series) had other features and complications of D.H. Another male patient with striking muscular atrophy and fasciculations, but a questionable diagnosis of amyotrophic lateral sclerosis, also was subject to longstanding recurrent hypoglycemia. . An association between idiopathic paroxysmal myoglobinuria and diabetes mellitus or insulin therapy has been documented (48, 49). .…”

“…. An association between idiopathic paroxysmal myoglobinuria and diabetes mellitus or insulin therapy has been documented (48, 49).…”

Strabismus as a possible sign of subclinical muscular dystrophy predisposing to rhabdomyolysis and myoglobinuria: a study of an affected family

“…Pregnancy, trauma, myocardial infarction and alcoholism are other conditions which need to be remembered. ~ 3 An association has been reported between MH, squint and ptosis. 16't7 The present sudy shows that strabismus patients may be prone to rhabdomyolysis and myoglobinuria.…”