A Clinical Study on Amyotrophic Lateral Sclerosis

J Korean Neurol Assoc > Volume 4(2); 1986 > Article

Journal of the Korean Neurological Association 1986;4(2): 200-208.

근위축성 측색경화증에 대한 임상적 고찰

박성호, 명호진

서울대학교 신경과.

A Clinical Study on Amyotrophic Lateral Sclerosis

Seong-Ho Park, M.D., Ho-Jin Myung, M.D.

Department of Neurology, College of Medicine, Seoul National University

Abstract

The authors reviewed the medical records of 78 patients diagnosed as amyotrophic lateral sclerosis (ALS) at the Department of Neurology of Seoul National University Hospital from January 1980 to June 1986. The clinical features, serum CPK value, electromyographic findings and muscle biopsy findings were analysed. Results obtained were as follows: 1. The mean age of onset was 44.4 (17-68) which was about 10 years earlier than that in the reports from other countires. The sex ratio (male to female) was two to one (52:26). 2. The most common from was conventional type (48.7%), followed by pseudopolyneuritic type (30.8%) and bulbar type (20.5%). 3. Muscle weakness (100%), muscle atrophy (97%), hyperreflexia (94%), fasciculations (82%), bulbar signs (67%), frontal release signs (31%), Babinski signs (27%) and sensory deficits (5%) were observed. Four cases with sensory deficits proved that they were accompanied with carpal tunnel syndromes. 4. The mean interval from onset to generalization of symptoms was shorter in bulbar type (10.6 months) than in non-bulbar type (16.6 months), suggesting a more progressive course in the former. 5. Serum CPK value was checked in 62 cases. An increase up to four times the normal value was observed in 17 cases (27%). 6. EMG (N=73) revealed such abnormal findings in 70 cases (95.9%) as increased insertional activities, fibrillations, positive sharp waves, reduced interfernece patterns, fasciculations and giant motor unit potentials. No abnormal findings were found in three cases, however. 7. Muscle biopsy (N=61) showed neurogenic atrophy with various changes in 56 cases (92%), massive fatty infiltration in 4 cases and muscle fascia only in one case.