We report a rare case of encephalitis in a woman with new onset seizures and autonomic instability.

A 29-year-old black woman was admitted with subacute onset of encephalopathy and myoclonus. She was recently diagnosed with type I diabetes mellitus, hypothyroidism, and drug reaction with eosinophilia and systemic symptoms (DRESS) due to minocycline. On admission, she was treated with antiepileptics and prophylactic antibiotics for suspected meningitis. She also developed episodes of autonomic instability with supraventricular tachycardia. This led to a pulseless electrical activity (PEA) arrest and she was successfully resuscitated.  Electroencephalography (EEG) monitoring showed nearly continuous generalized rhythmic delta activity (GRDA) and rare generalized periodic discharges (GPDs) but no electrographic seizures. An MRI brain demonstrated leptomeningeal enhancement and cerebral edema of the hippocampi and amygdala. Cerebrospinal fluid (CSF) studies were remarkable for mild lymphocytic pleocytosis, elevated protein, positive oligoclonal bands and elevated IgG index. A diagnosis of autoimmune encephalitis was suspected and she was initially treated with high doses of intravenous steroids. She did not clinically improve until the addition of  intravenous immunoglobulins (IVIG) and was discharged on oral steroids. The CSF autoimmune panel was positive for anti-GFAP antibody.  She was diagnosed with GFAP astrocytopathy.

GFAP astrocytopathy is a novel CNS autoimmune syndrome. This case shows severe autonomic system involvement. While most patients respond briskly to steroids, our case shows the effectiveness of IVIG, which suggests a steroid-refractory phenotype. Finally, we show here GFAP astrocytopathy which  was simultaneous with the onset of multiple autoimmune syndromes in response to tetracyclines.