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#Hospital Medicine #Internal Medicine #Pediatrics
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy, a type of steroid-responsive autoimmune disease of the nervous system, is associated with the presence of anti-GFAP immunoglobulin G in the serum or cerebrospinal fluid (CSF) of affected patients.
A case report demonstrated acute neurological symptoms, including headache, fever, confusion, and paralysis of the lower extremities. The CSF analysis revealed lymphocytic pleocytosis and elevated protein levels, indicating acute disseminated encephalomyelitis, and the patient was initiated immunotherapy. Cranial magnetic resonance imaging revealed multifocal T2/fluid-attenuated inversion recovery hyperintense signal changes in the periventricular white matter, and electromyography testing showed changes consistent with severe sensorimotor neuropathy, indicating the involvement of the brain and peripheral nerves.
Thus the patient was rendered the diagnosis of autoimmune GFAP astrocytopathy due to the presence of GFAP-immunoglobulin G in the patients CSF. He was treated with one course of intravenous immunoglobulin therapy, followed by intravenous methylprednisolone (1.0 g/d for 3 days) and oral prednisolone.
1 week of intravenous immunoglobulin therapy showed improvement in the level of consciousness. However, flaccid paralysis persisted without substantial improvement.
It was thus concluded that an accurate early diagnosis and appropriate treatment are crucial for improving the prognosis of patients with autoimmune GFAP astrocytopathy. Further, recognizing the role of peripheral nerve involvement in GFAP autoimmunity is important.
Source: Medicine (Baltimore). 2021;100(25):e26448. doi:10.1097/MD.0000000000026448