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Delayed immune response in chorea-amyotrophy with spherocytosis
H L Lagrèze1, S E Kornguth, B R Brooks
1Neurology Service, William S. Middleton Memorial Hospital, Madison, WI.
Neurology
|October 1, 1988
Summary
Familial neurodegenerative diseases with spherocytosis show late-stage immune responses, including cerebrospinal fluid (CSF) pleocytosis and intrathecal IgG synthesis. Antibodies against glial fibrillary acidic protein suggest a secondary autoimmune trigger.
Area of Science:
- Neuroimmunology
- Neurodegenerative Diseases
- Genetics
Background:
- Familial neurodegenerative disorders present complex clinical and pathological features.
- Understanding the interplay between neurodegeneration and immune responses is crucial.
Observation:
- Patients with familial amyotrophy, neuropathy, chorea, and dementia with spherocytosis exhibited progressive cerebrospinal fluid (CSF) lymphocytic pleocytosis.
- Intrathecal IgG synthesis was a late-occurring feature in these patients.
Findings:
- Immunoblotting detected a specific antibody in both serum and CSF targeting glial fibrillary acidic protein (GFAP).
- This suggests an autoimmune component directed against GFAP.
Implications:
- The findings indicate a potential secondary autoimmune response triggered during the neurodegenerative process.
- This highlights GFAP as a potential target in understanding disease pathogenesis and developing therapeutic strategies.