Clinical and electrophysiological studies of subacute combined degeneration

J Korean Neurol Assoc > Volume 15(5); 1997 > Article

Journal of the Korean Neurological Association 1997;15(5): 1085-1096.

Subacute combined degeneration의 임상적 및 전기생리학적 특징

배희준, 이한보, 이광우

서울대학교병원 신경과, 강북삼성병원 신경과

Clinical and electrophysiological studies of subacute combined degeneration

Hee-Joon Bae, M.D. , Han Bo Lee, M.D.* , and Kwang Woo Lee, M.D.

Dept of Neurology, Seoul National University Hospital, Kangbuk Samsung Hospital

Abstract

Background & Objectives : Subacute combined degeneration (SCD) is a disease of spinal cord involving the posterior and lateral column due to vitamin B12 deficiency. The clinical and electrophysiologic findings of SCD are various. Methods : From 1989 to 1996, 7 patients were diagnosed with SCD in our hospitals. The diagnosis was made by the neurologic and laboratory findings and electrophysiolgic studies such as nerve conduction studies(NCS) and evoked potential(EP). Result ; Four patients received gastrectomy. Two had chronic atrophic, gastritis; one of them was assumed to have food-cobalamin malabsorrtion. The remaining one was a heavy drinker. The mean duration of neurologic symptoms was 35.7 months. The most common initial complaint was paresthesia (in 4) and impairment of cutaneous sensation was the most common neurologic sign At the time of diagnosis, 5 patients had myelopathy with was supported by EP in 3(60%). There were abnormal NCS findings in 5 f 6 patients with peripheral neuropathy. In one patient, there was no symptom and sign compatible with myelopathy but median nerve SEP showed bilateral central conduction delay. No one had visual symptoms but prolongation of P 100 was detected in 2 patients. Sural nerve biopsy was done in 2 case, which revealed chronic nonspecific neuropatby in one and chronic axonopathy in the other. Megaloblastic anemia was found in 4 cases and improved by cobalamin therapy in all the parents, in which the follow up hematologic data were available. There as a tendency that nonanemic patients had more severe neurologic symptoms than anemic ones. We could not find any relationship between the duration and severity of neurologic menifestations was best in the patients with the shortest duration of neurologic manifestations and hematologic feature festations. Conclusions : The authors suggest that early detection and treatment is very important for the improvement of symptoms in SCD.