A Clinical Profile of Peripheral Neuropathy in Korean Children / 대한소아신경학회지

PURPOSE: To analyze the clinical features of peripheral neuropathy in Korean children. METHODS: A total of 62 children with acute flaccid paralysis, longstanding weakness of extremities, or abnormal electrophysiological studies, suggestive of peripheral neuropathy, were evaluated retrospectively from the hospital records. The subjects were recruited at the pediatric neurology and endocrine clinic, Kyungpook National University Hospital from 2000 to 2002 and they all went through neurological examination and electrophysiological studies with or without nerve biopsy. RESULTS: Thirty nine children(Male 24:Female 15; Mean age 7.6+/-4.3 years) were found to have clinical peripheral neuropathy. Inflammatory neuropathy(5 children with Guillain Barre syndrome, 1 children with chronic inflammatory demyelinating polyneuropathy, 12 children with Bell's palsy; 46%) was the most common, followed by hereditary neuropathy(4 children, 10%), Chemotherapy induced neuropathy(3 children, 8%), metachromatic leukodystrophy(2 children, 5%), trauma(2 children, 5%), diabetic neuropathy(1 children, 3%) and so on. Thirty two children had motor neuropathy(82%), six children had combined motor and sensory neuropathy(15%), two had pure sensory(5%), but nobody had autonomic neuropathy. With respect to the type of involvement, polyneuropathies constitute 59%(23 children), mononeuropathy simplex accounted for 38%(15 children), mononeuropathy multiplex was found in 3%(1 child). Based on electrophysiological studies and biopsy results, demyelinating neuropathy was seen in 22 children(56%), axonal neuropathy in 12 children(31%), combined neuropathy in 5 children(13%). Eighteen children(46%) were completely or almost completely recovered from the illness. CONCLUSION: Inflammatory neuropathy was the most common among the acquired neuropathies and hereditary motor sensory neuropathy was the most common among the genetic neuropathies. Treatable neuropathies took up 46%. Potentially preventable neuropathies accounted for 36%. Early diagnosis and early intervention may have significant impacts on the prognosis of peripheral neuropathy in children.

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

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.