ACUTE PORPHYRIA: REPORT OF TWO CASES WITH ELECTRICAL STUDIES IN ONE

Abstract

Two cases of acute idiopathic porphyria are reported. One was easily recognized by the characteristic red urine. The other case passed as a case of Guillain-Barre syndrome for more than a yr., chiefly because the urine was essentially normal in color when voided. Both cases were characterized during their attacks by acute abdominal symptoms, tachycardia and hypertension. In one of the cases neurologic complications in the form of muscular paralysis (quadriplegia), bulbar symptoms in the form of dysphonia and dysarthria were noted, but the patient eventually made an almost complete recovery. Muscular atrophy and paralysis persisted in the upper extremities and shoulder girdles. The radial and median nerves were most affected. Data from electrodiagnostic tests by means of a constant current impulse stimulator and an electromyograph were compatible with postmortem pathologic findings of patchy degeneration of motor nerves. No evidence of complete degeneration was obtained, although there was some evidence of regeneration. Return of voluntary power was noted in some muscles. Muscles which were weakened showed gradual improvement in strength as the disease was allayed and as they were encouraged to further activity.