CSF findings in adrenoleukodystrophy: correlation between measures of cytokines, IgG production, and disease severity.
The childhood-onset cerebral form of adrenoleukodystrophy has a devastating neurologic prognosis. Unfortunately, there is no early method of distinguishing it from the more benign forms of adrenoleukodystrophy, such as adrenomyeloneuropathy. To evaluate the manner in which this disease entity may be reflected in the cerebrospinal fluid, we studied a consecutive series of 19 patients, all with biochemically proved adrenoleukodystrophy. total protein, immunoglobulin production, cytokine levels, and cerebrospinal fluid pressure were measured. In this single sample of cerebrospinal fluid, a significant correlation existed between clinical stage of the illness and cerebrospinal fluid myelin basic protein. No correlation existed with total protein, cytokines, or measures of immunoglobulin production.
Phillips
J P
JP
Division of Pediatric Neurology, University of Minnesota Medical School, Minneapolis.
Lockman
L A
LA
Shapiro
E G
EG
Blazar
B R
BR
Loes
D J
DJ
Moser
H W
HW
Krivit
W
W
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000134:cerebrospinal fluid* / Q000175:diagnosis; D015170:Cerebrospinal Fluid Pressure; D002556:Cerebrospinal Fluid Proteins / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D016207:Cytokines / Q000134:cerebrospinal fluid*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000134:cerebrospinal fluid*; D008297:Male; D004676:Myelin Basic Protein / Q000134:cerebrospinal fluid; D011379:Prognosis; D012720:Severity of Illness Index
D002556:Cerebrospinal Fluid Proteins; D016207:Cytokines; D007074:Immunoglobulin G; D004676:Myelin Basic Protein