BACKGROUND: Peroneal muscular atrophy (PMA) is characterized by insidious onset, gradually progressive course of disease, very mild disability degree and easily subjecting to missed diagnosis and misdiagnosis. Nerve c...BACKGROUND: Peroneal muscular atrophy (PMA) is characterized by insidious onset, gradually progressive course of disease, very mild disability degree and easily subjecting to missed diagnosis and misdiagnosis. Nerve conductive velocity is helpful in the diagnosis of atypical cases. OBJECTIVE: To retrospectively analyze the characteristics of clinical manifestation, electromyogram (EMG), motor and sensory nerve conduction velocity of patients with PMA. DESIGN: Retrospective case analysis. SETTING: Department of Neurology, Guangzhou First People's Hospital. PARTICIPANTS: Twenty-four patients with PMA, including 16 males and 8 females, aged 5-68 years old, admitted to Guangzhou First People's Hospital between March 1996 and January 2006 were recruited. Informed consents were obtained from all the patients. METHODS: All the patients subjected to EMG and detection of nerve conduction velocity at distal end of four extremities with a Keypoint evoked potential/ EMG instrument (Denmark). Sensory and motor conduction velocity, EMG changes of upper and lower extremities were observed, and relationship of neuroelectrophysiological characteristics and clinical symptoms was analyzed. MAIN OUTCOME MEASURES: Changes in sensory and motor conduction velocity, EMG and clinical manifestations of 24 patients. RESULTS: ① All the patients suffered from insidious onset and gradually progressive course of PMA. Muscular atrophy of lower extremity was found in 14 patients, and that of upper extremity in 5 patients. ②Routine nerve conduction study showed that sensory and motor conduction velocity were stepped down, especially in 16 patients with typeⅠPMA (demyelinating pattern, nerve conduction velocity below normal level 50%). Motor nerve conduction velocity of median nerve, ulnar nerve, common peroneal nerve and tibial nerve averaged 34.8 m/s, 37.2 m/s, 16.5 m/s and 17.4 m/s, respectively; Sensory nerve conduction velocity of median nerve, ulnar nerve and sural nerve averaged 27.9%, 24.6 m/s and 3.1 m/s, respectively. Slowing conduction velocity and muscular strength involvement were disproportionate, i.e. myasthenia was relatively lessened, sensory and motor conduction velocities were greatly decreased. Nerve conduction velocity in distal end of two lower extremities was not detected in 8 patients, but who could still walk. CONCLUSION: ①PMA of patients is characterized by insidious onset and gradually progressive course of disease. Clinical symptom is the base to diagnose PMA. ②Neuroelectrophysiological study is a simple and easy-to-operate means with good reproducibility in diagnosing PMA. Patients with abnormal myasthenia in lower extremity can be detected in the early stage.展开更多
文摘BACKGROUND: Peroneal muscular atrophy (PMA) is characterized by insidious onset, gradually progressive course of disease, very mild disability degree and easily subjecting to missed diagnosis and misdiagnosis. Nerve conductive velocity is helpful in the diagnosis of atypical cases. OBJECTIVE: To retrospectively analyze the characteristics of clinical manifestation, electromyogram (EMG), motor and sensory nerve conduction velocity of patients with PMA. DESIGN: Retrospective case analysis. SETTING: Department of Neurology, Guangzhou First People's Hospital. PARTICIPANTS: Twenty-four patients with PMA, including 16 males and 8 females, aged 5-68 years old, admitted to Guangzhou First People's Hospital between March 1996 and January 2006 were recruited. Informed consents were obtained from all the patients. METHODS: All the patients subjected to EMG and detection of nerve conduction velocity at distal end of four extremities with a Keypoint evoked potential/ EMG instrument (Denmark). Sensory and motor conduction velocity, EMG changes of upper and lower extremities were observed, and relationship of neuroelectrophysiological characteristics and clinical symptoms was analyzed. MAIN OUTCOME MEASURES: Changes in sensory and motor conduction velocity, EMG and clinical manifestations of 24 patients. RESULTS: ① All the patients suffered from insidious onset and gradually progressive course of PMA. Muscular atrophy of lower extremity was found in 14 patients, and that of upper extremity in 5 patients. ②Routine nerve conduction study showed that sensory and motor conduction velocity were stepped down, especially in 16 patients with typeⅠPMA (demyelinating pattern, nerve conduction velocity below normal level 50%). Motor nerve conduction velocity of median nerve, ulnar nerve, common peroneal nerve and tibial nerve averaged 34.8 m/s, 37.2 m/s, 16.5 m/s and 17.4 m/s, respectively; Sensory nerve conduction velocity of median nerve, ulnar nerve and sural nerve averaged 27.9%, 24.6 m/s and 3.1 m/s, respectively. Slowing conduction velocity and muscular strength involvement were disproportionate, i.e. myasthenia was relatively lessened, sensory and motor conduction velocities were greatly decreased. Nerve conduction velocity in distal end of two lower extremities was not detected in 8 patients, but who could still walk. CONCLUSION: ①PMA of patients is characterized by insidious onset and gradually progressive course of disease. Clinical symptom is the base to diagnose PMA. ②Neuroelectrophysiological study is a simple and easy-to-operate means with good reproducibility in diagnosing PMA. Patients with abnormal myasthenia in lower extremity can be detected in the early stage.
