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GFAP astrocytopathy — SCE Neurology MCQ

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HardNeurogenetics and NeuroimmunologyGFAP astrocytopathySCE Neurology

A patient develops a febrile meningoencephalomyelitis with tremor, urinary retention and optic-disc swelling. MRI shows radial perivascular enhancement and CSF GFAP-IgG is positive by a validated assay. Infection and malignancy screening are negative. Which treatment is most appropriate?

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Correct answer: E — High-dose corticosteroids followed by a slow taper

The best answer is “High-dose corticosteroids followed by a slow taper”. GFAP astrocytopathy is usually a steroid-responsive autoimmune meningoencephalomyelitis; severe disease warrants acute corticosteroids, and slow tapering is often used because relapse can occur. “Treat with levodopa because tremor is the defining disease mechanism” is less appropriate because dopamine replacement does not treat the inflammatory astrocytopathy “Use long-term aciclovir despite repeatedly negative viral studies” is less appropriate because continued antiviral therapy is not justified after adequate exclusion of infection “Give anticoagulation for presumed cerebral venous thrombosis without venography” is less appropriate because the imaging and antibody phenotype do not establish venous thrombosis “Provide rehabilitation alone without immune treatment” is less appropriate because rehabilitation is valuable but does not suppress active CNS inflammation

Reference: Autoimmune GFAP astrocytopathy: prospective evaluation of 90 patients. https://pubmed.ncbi.nlm.nih.gov/28459450/