title: [Two patients with congenital insensitivity to pain with anhidrosis showing marked prolongation of central conduction time in short latency somatosensory evoked potential].
abstract: 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.
authors.affiliation: Department of Child Neurology, National Center Hospital of Neurology and Psychiatry, Kodaira, Tokyo. r-tanaka@md.tsukuba.ac.jp
authors.forename: Ryuta
authors.initials: R
authors.lastname: Tanaka
authors.forename: Kenji
authors.initials: K
authors.lastname: Sugai
authors.forename: Yoshinao
authors.initials: Y
authors.lastname: Fujikawa
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Komaki
authors.forename: Eiji
authors.initials: E
authors.lastname: Nakagawa
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Saito
authors.forename: Tatsuyuki
authors.initials: T
authors.lastname: Ohto
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Hashimoto
authors.forename: Masayuki
authors.initials: M
authors.lastname: Sasaki
mesh_terms: D005073:Evoked Potentials, Somatosensory; D005260:Female; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D008297:Male; D009431:Neural Conduction / Q000502:physiology*; D013036:Spasms, Infantile / Q000503:physiopathology