Decreased cerebrospinal fluid levels of substance P in patients with Rett syndrome.
To clarify the mechanism of brain and spinal cord impairment in Rett syndrome (RS), we measured the cerebrospinal fluid (CSF) levels of substance P in 20 patients with RS including 16 childhood patients and 4 adult patients. Findings were compared with those obtained in age-matched controls and diseased controls. The CSF level of substance P was significantly lower in patients with RS compared with controls. The alteration in the CSF level of substance P may be related to the neurological impairment, especially autonomic dysfunction, and neuropathological involvement of dorsal root ganglia and peripheral nerve observed in RS.
Matsuishi
T
T
Department of Pediatrics and Child Health, Kurume University School of Medicine, Kurume City, Japan.
Nagamitsu
S
S
Yamashita
Y
Y
Murakami
Y
Y
Kimura
A
A
Sakai
T
T
Shoji
H
H
Kato
H
H
Percy
A K
AK
D000328:Adult; D015415:Biomarkers / Q000134:cerebrospinal fluid*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D011237:Predictive Value of Tests; D015518:Rett Syndrome / Q000134:cerebrospinal fluid* / Q000150:complications; D013373:Substance P / Q000134:cerebrospinal fluid*
D015415:Biomarkers; D013373:Substance P