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Two patients with congenital insensitivity to pain with anhidrosis showing marked prolongation of central conduction time in short latency somatosensory evoked potential

Tanaka, R.; Sugai, K.; Fujikawa, Y.; Komaki, H.; Nakagawa, E.; Saito, Y.; Ohto, T.; Hashimoto, T.; Sasaki, M.

No to Hattatsu 41(6): 452-456

2009

ISSN/ISBN: 0029-0831
PMID: 19928545
Document Number: 636509
We examined the evoked potentials in 2 patients, a 6-month-old girl and a 3-year-old boy, with congenital insensitivity to pain with anhidrosis (CIPA). While auditory brainstem response (ABR) in both patients showed normal latencies, flash visual evoked potential (FVEP) revealed delayed latency of wave IV (P100), and short latency somatosensory evoked potential (SSEP) demonstrated marked prolongation of the central conduction time (CCT; N13-N20 interval). The boy had West syndrome and his prolonged CCT might have been influenced by abnormal cortical activities. The girl did not have epilepsy and the abnormalities of her F-VEP and SSEP might have been caused by the developmental deficit of the central nervous system associated with the pathogenesis of CIPA.

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