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Abstract Details

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy masquerading as tuberculosis of the central nervous system
Autoimmune Neurology
P12 - Poster Session 12 (12:00 PM-1:00 PM)
15-001

To describe two patients with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy whose initial presentation and treatment response mimicked those of tuberculous meningitis.

As delayed treatment of tuberculous meningitis can lead to fatal consequences, it is not uncommon for patients to be empirically treated with anti-tuberculosis treatment before diagnostic assay and culture results become available, especially in regions where tuberculosis is endemic. Autoimmune GFAP astrocytopathy, a steroid-responsive disorder, could mimic clinical presentation and initial response to anti-tuberculosis therapy.  

NA

Patient 1: A 56-year-old man presented with 2-week history of fever, headache, dizziness and confusion. He had nystagmus, ataxia and tremors. Cerebrospinal fluid (CSF) examination showed lymphocytic pleocytosis with elevated protein. There was leptomeningeal enhancement of the brainstem and cervical cord on magnetic resonance imaging. Anti-tuberculosis  treatment with tapering steroids were started for tuberculous meningitis. The patient demonstrated clinical, CSF and radiologic improvement initially, but ataxia and confusion later recurred during steroid taper. Investigations showed GFAP-IgG in the CSF. He was reinitiated on high dose steroids with improvement.

 

Patient 2: A 43-year-old man presented with 5 days of fever, vomiting and confusion. He developed seizures and respiratory failure requiring intubation. Opening pressure was 29 cmH20 on lumbar puncture, with lymphocytic pleocytosis and elevated protein. Neuroimaging showed diffuse leptomeningeal enhancement. Treatment for tuberculous meningitis with anti-tuberculosis drugs and steroids was initiated. His mentation, CSF and radiologic changes gradually improved while on treatment. Subsequent fever and myeloradiculitis were attributed to tuberculous paradoxical reaction during steroid taper, and steroids were consequently increased. GFAP-IgG was later detected in the CSF.

Initial clinical features of autoimmune GFAP astrocytopathy may be difficult to distinguish from tuberculous meningitis. Autoimmune GFAP astrocytopathy should be suspected in patients with neurological deterioration and paradoxical reaction of tuberculous meningitis, and confirmed by detection of GFAP-IgG in CSF.

Authors/Disclosures
David K. Tang, MBBS (NUH)
PRESENTER
No disclosure on file
Amanda X. Chin, MBBS (Nuhs) No disclosure on file
No disclosure on file