title: [Motor nerve conduction study in McArdle disease: case report].
abstract: McArdle disease (glycogenosis type V) is a metabolic myopathy with symptoms of exercise intolerance caused by deficiency of the enzyme myophosphorylase. In these patients, the motor nerve conduction studies after a short period of maximal voluntary muscle contraction or repetitive stimulation reveals characteristic findings of the disease. A 37-year-old man presented symptoms of exercise intolerance, muscular fatigue and cramps in the beginning of the physical activity with "second wind" phenomenon. The motor nerve conduction studies after a voluntary contraction of 30 and 90 seconds presented decrease in the amplitude of the compound muscle action potential in median, ulnar and deep peroneal nerves; and decrement after 200 stimulation at the 40 Hz in deep peroneal nerve. The electromyography presented myopathic pattern and during the ischemic exercise electric silence was not proven. The characteristic of electrophysiological studies are discussed with emphasis at the importance of the motor nerve conduction studies in the patients with suspicion of metabolic myopathy.
authors.affiliation: Serviço de Doenças Neuromusculares, Hospital de Clínicas, Universidade Federal do Paraná, Curitiba, PR, Brasil.
authors.forename: Paulo José
authors.initials: PJ
authors.lastname: Lorenzoni
authors.forename: Marcos Cristiano
authors.initials: MC
authors.lastname: Lange
authors.forename: Cláudia Suely Kamoi
authors.initials: CS
authors.lastname: Kay
authors.forename: Rosana Herminia
authors.initials: RH
authors.lastname: Scola
authors.forename: Lineu César
authors.initials: LC
authors.lastname: Werneck
mesh_terms: D000200:Action Potentials / Q000502:physiology; D000328:Adult; D001706:Biopsy; D004576:Electromyography; D015444:Exercise / Q000502:physiology; D006012:Glycogen Storage Disease Type V / Q000473:pathology* / Q000503:physiopathology*; D006801:Humans; D008297:Male; D009046:Motor Neurons / Q000502:physiology*; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology; D009431:Neural Conduction / Q000502:physiology*