(Kiosk 3) Gfap-astrocytopathy Recovery Through the Combined Efforts of Medical Intervention, Pharmacology, and Acute Spinal Cord Rehabilitation: A Case Report

Case Diagnosis: Functional recovery of a 23 year-old male diagnosed with GFAP-A.

Case Description or Program Description: Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is an inflammatory central nervous system disorder with many reported clinical syndromes. We present a 23-year-old male who was brought to the emergency department endorsing fever, headache, myalgias, cough, vomiting, photophobia, and urinary retention. Eventual diagnosis indicated GFAP-A.

Setting: Acute Inpatient Rehabilitation Hospital

Assessment/Results: Initial CSF revealed lymphocytic predominance, leading to initiation of empiric meningitis treatment, which was discontinued in the setting of negative bacterial, fungal, and viral cultures. MRI was significant for longitudinally extensive transverse myelitis. The patient was then administered intravenous solumedrol for encephalitis. He later developed lower extremity weakness with concern for acute inflammatory demyelinating polyneuropathy, but electromyography was non-diagnostic. Further work-up revealed positive GFAP antibodies and oligoclonal bands, confirming diagnosis of GFAP-A. He subsequently completed rituximab and plasmapheresis, along with a steroid taper. Additionally, he had dysphagia and respiratory compromise that required tracheostomy and PEG placement.

The patient was admitted to an acute rehabilitation hospital with a dedicated spinal cord injury unit and completed 34 days of intensive rehabilitation. Physicians, nurses, and therapists addressed his neurogenic bladder and bowel, compromised respiratory status, dysphagia, and sensorimotor deficits. He made substantial, rapid progress in transfers, ambulation, and grooming, achieving modified independence in most measures - by rehabilitation discharge.

Discussion (relevance): GFAP-A is a rare disease that is believed to affect 0.6/10000. This 23-year-old man with GFAP-A had similar needs to those of an SCI patient, but made remarkable progress in ADL’s, I-ADL’s, and mobility more rapidly than seen in traumatic tetraplegia.

Conclusions: Suggested explanations of his significant improvement include rapid identification of the motor loss with prompt initiation of steroids, along with early mobilization and transition to acute rehabilitation. Further investigation of the value of timely rehabilitation in the neurological recovery of GFAP-A is warranted.