Decreased cerebrospinal fluid levels of substance P in Machado-Joseph disease.
To clarify the mechanism of brain impairment in Machado-Joseph disease (MJD), we measured the cerebrospinal fluid (CSF) levels of substance P in 7 patients (mean age 45.7 +/- 12.09 years) with this disease. Four patients had type I and three had type II disease. Findings were compared with those obtained in 14 age-matched controls, 8 patients with Parkinson's disease, 7 patients with multiple system atrophy, and 6 patients with myopathy. The CSF level of substance P was significantly (p = 0.0000) lower in the patients with MJD, being 44.5% of the control value. However, the mean CSF levels of substance P in the patients with Parkinson's disease, multiple system atrophy, or myopathy did not differ significantly from that in the control subjects. The alteration in the CSF level of substance P may be related to the neurological impairment observed in MJD.
Matsuishi
T
T
Department of Pediatrics and Child Health, Kurume University School of Medicine, Fukuoka, Japan.
Sakai
T
T
Nagamitsu
S
S
Shoji
H
H
Ueda
N
N
Kaneko
S
S
Kano
T
T
Iwashita
H
H
Kato
H
H
D000328:Adult; D002452:Cell Count; D002555:Cerebrospinal Fluid / Q000737:chemistry / Q000166:cytology / Q000276:immunology; D002556:Cerebrospinal Fluid Proteins / Q000032:analysis; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D017827:Machado-Joseph Disease / Q000134:cerebrospinal fluid*; D008297:Male; D008875:Middle Aged; D013373:Substance P / Q000134:cerebrospinal fluid*; D005719:gamma-Globulins / Q000032:analysis
D002556:Cerebrospinal Fluid Proteins; D007074:Immunoglobulin G; D005719:gamma-Globulins; D013373:Substance P