DECREASED CEREBROSPINAL-FLUID LEVELS OF SUBSTANCE-P IN PATIENTS WITH RETT-SYNDROME

To clarify the mechanism of brain and spinal cord impairment in Rett s yndrome (RS), we measured the cerebrospinal fluid (CSF) levels of subs tance P in 20 patients with RS including 16 childhood patients and 4 a dult patients. Findings were compared with those obtained in age-match ed controls and diseased controls. The CSF level of substance P was si gnificantly lower in patients with RS compared with controls. The alte ration in the CSF level of substance P may he related to the neurologi cal impairment, especially autonomic dysfunction, and neuropathologica l involvement of dorsal root ganglia and peripheral nerve observed in RS.