Objective:

To present a case of steroid-responsive GFAP astrocytopathy in a young and otherwise healthy patient presenting with meningoencephalitis and conus medullaris syndrome.

Background:

Glial Fibrillary Acidic Protein (GFAP) is a protein present in adult astrocytes. In 2016, antibodies against said protein were identified and were later associated with a clinical syndrome: fevers, cranial nerve neuropathies, meningoencephalitis, myelitis, and involuntary movements. However, no uniform diagnostic criteria for GFAP astrocytopathy has been established and there is no standard of treatment.

Design/Methods:

Case report.

Results:

A 29-year-old man with no past medical history presented to the emergency department with two weeks of fevers, chills, headache with photophobia, vomiting, neck stiffness, and persistent hiccups. Spouse reported irritability, disorientation, and lack of insight. Initial exam was relevant for fluctuating confusion, diffuse hyperreflexia, loss of check, and bilateral eye abduction deficits with right eye esotropia. Several days into the hospital course, he developed urinary retention, constipation, and lower extremity weakness. MRI total spine revealed mild thickening of the cauda equina nerve roots and spinal cord enhancement at L2 consistent with conus medullaris syndrome. MRI brain revealed mild enhancement of bilateral oculomotor, trigeminal, facial and vestibular nerves. Lumbar puncture and CSF studies were notable for high opening pressure (33cmH2O), lymphocytic pleocytosis, elevated protein and normal glucose. CSF GFAP autoantibody titers were positive (1:1920). After five days of high dose steroids, cranial nerve deficits and leg weakness resolved; there was also improvement of constipation and mental status. The patient was discharged to acute rehabilitation with a steroid taper.

Conclusions:

GFAP astrocytopathy can present in young and healthy patients as recurrent fever, cranial nerve neuropathies, meningoencephalitis, and conus medullaris syndrome. Although there is no standard of treatment for GFAP astrocytopathy, patients can have significant improvement after a short course of high dose steroids and physical therapy.