[Acute intermittent porphyria with transient paresis and contracture. Evidence for initial myopathic dysfunction?].
A 70-year old man with acute intermittent porphyria had acute transient bibrachial paresis with moderate contracture of the involved muscles, which showed electrical silence in a conventional electromyographic investigation with surface electrodes placed over the contracted muscle belly (filter: 20 Hz-2 Hz, amplifier 50 microV). Slow finger movements were still possible and showed typical muscle-action potentials. This electroclinical correlation points to myopathic localized dysfunction, perhaps similar to rare case reports with patients having metabolic myopathy of McArdle's type.
Poersch
M
M
Dr. von Ehrenwall'sche Klinik, Fachkrankenhaus für Psychiatrie, Psychotherapie und Neurologie, Ahrweiler.
D000368:Aged; D003286:Contracture / Q000175:diagnosis / Q000503:physiopathology*; D004576:Electromyography; D006801:Humans; D007537:Isometric Contraction / Q000502:physiology; D008297:Male; D009048:Motor Skills / Q000502:physiology; D009460:Neurologic Examination; D009469:Neuromuscular Junction / Q000502:physiology; D010291:Paresis / Q000175:diagnosis / Q000503:physiopathology*; D017118:Porphyria, Acute Intermittent / Q000175:diagnosis / Q000503:physiopathology*