Delayed immune response in chorea-amyotrophy with spherocytosis.
Progressive CSF lymphocytic pleocytosis and intrathecal IgG-synthesis occurred late in familial amyotrophy, neuropathy, chorea, and dementia with spherocytosis. Immunoblotting showed a serum and CSF antibody apparently directed against glial fibrillary acidic protein. A secondary autoimmune response was probably triggered during the evolution of the neurodegenerative process.
Neurology Service, William S. Middleton Memorial Hospital, Madison, WI.
H L
HL
Lagrèze
S E
SE
Kornguth
B R
BR
Brooks
R L
RL
Levine
D002819:Chorea / Q000276:immunology*; D004913:Erythrocytes, Abnormal / Q000276:immunology*; D005904:Glial Fibrillary Acidic Protein / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000096:biosynthesis; D008297:Male; D009468:Neuromuscular Diseases / Q000276:immunology*; D011086:Polycythemia / Q000276:immunology*; D013102:Spherocytes / Q000276:immunology*
D005904:Glial Fibrillary Acidic Protein; D007074:Immunoglobulin G