A Case of Chronic Inflammatory Demyelinating Polyneuropathy in a 13-year-old Girl with Pes Cavus Deformity

J Korean Neurol Assoc > Volume 18(4); 2000 > Article

Journal of the Korean Neurological Association 2000;18(4): 503-507.

요족변형을 동반한 소아에서 진단된 만성 염증성 탈수초성 다발신경병증 1예

강남제, 김은주 김대성 정대수 박규현

부산대학교 의과대학 신경과학교실

A Case of Chronic Inflammatory Demyelinating Polyneuropathy in a 13-year-old Girl with Pes Cavus Deformity

Nam-Je Kang, M.D., Eun-Joo Kim, M.D., Dae-Seong Kim, M.D., Dae-Soo Jung, M.D., Kyu-Hyun Park, M.D.

Department of Neurology, Pusan National University Hospital

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated polyneuropathy with a relaps-ing or progressive course. Although the occurrence of CIDP is very rare in childhood compared to the occurrence in adulthood, it does occur in children. In childhood CIDP, the main clinical features are somewhat different from that of adults, which includes a more precipitous onset of symptoms, a high frequency of gait abnormalities, and significant neurological dysfunction. Also, the response to immune-modulating therapy is more reliable and rapid in children than in adults. The recognition of childhood CIDP is important as it may mimic hereditary neuropathies which usually pre-sent with progressive gait disturbance associated with pes cavus deformity. A careful history, clinical examination, and electrophysiological study is crucial for the accurate diagnosis of this treatable disease. Here, we report a case of CIDP in a 13-year-old Korean girl whose initial clinical presentation strongly suggested hereditary motor-sensory neuropathy. J Korean Neurol Assoc 18(4):503~507, 2000 Key Words : Chronic inflammatory demyelinating polyneuropathy, Hereditary motor-sensory neuropathy, Childhood, Pes cavus, Nerve conduction study