[Two patients with congenital insensitivity to pain with anhidrosis showing marked prolongation of central conduction time in short latency somatosensory evoked potential].
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.
Tanaka
Ryuta
R
Department of Child Neurology, National Center Hospital of Neurology and Psychiatry, Kodaira, Tokyo. r-tanaka@md.tsukuba.ac.jp
Sugai
Kenji
K
Fujikawa
Yoshinao
Y
Komaki
Hirofumi
H
Nakagawa
Eiji
E
Saito
Yoshiaki
Y
Ohto
Tatsuyuki
T
Hashimoto
Toshiaki
T
Sasaki
Masayuki
M
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