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Radiographic evolution of myelitis in a case of glial fibrillary acidic protein (GFAP) astrocytopathy
Christopher E Goyne1, David Piccioni2, Jason Handwerker3
1Neurology, University of California, La Jolla, California, USA cgoyne@health.ucsd.edu.
Abstract:
Autoimmune glial fibrillar acidic protein (GFAP) astrocytopathy is a rare autoimmune neuroinflammatory disorder that affects the central nervous system. We present a case of GFAP astrocytopathy in a middle-aged male who presented with constitutional symptoms, encephalopathy and lower extremity weakness and numbness. Initially MRI of the spine was normal, but he subsequently developed longitudinally extensive myelitis and meningoencephalitis. Workup for infectious aetiologies was negative and the patient's clinical course worsened despite broad antimicrobial coverage. Ultimately, he was found to have anti-GFAP antibodies in his cerebral spinal fluid consistent with GFAP astrocytopathy. He was treated with steroids and plasmapheresis with clinical and radiographic improvement. This case demonstrates the temporal evolution of myelitis on MRI in a case of steroid-refractory GFAP astrocytopathy.
Insights
Autoimmune glial fibrillar acidic protein (GFAP) astrocytopathy is a rare neuroinflammatory disorder. This case highlights its evolving presentation and successful treatment with steroids and plasmapheresis.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Glial fibrillar acidic protein (GFAP) astrocytopathy is a rare autoimmune disorder impacting the central nervous system.
- It is characterized by inflammation affecting astrocytes, the star-shaped glial cells supporting neurons.
Observation:
- A middle-aged male presented with constitutional symptoms, encephalopathy, and lower extremity neurological deficits.
- Initial spinal MRI was unremarkable, but the patient later developed longitudinally extensive myelitis and meningoencephalitis.
- Infectious workups were negative, and the patient's condition deteriorated despite empirical antimicrobial treatment.
Findings:
- Cerebrospinal fluid analysis revealed the presence of anti-GFAP antibodies, confirming the diagnosis of GFAP astrocytopathy.
- The patient exhibited steroid-refractory disease, necessitating aggressive treatment.
- Treatment with corticosteroids and plasmapheresis led to significant clinical and radiographic improvement.
Implications:
- This case underscores the importance of considering GFAP astrocytopathy in patients with unexplained neuroinflammation.
- It illustrates the dynamic changes in MRI findings, particularly the evolution of myelitis.
- Early diagnosis and prompt immunotherapy are crucial for favorable outcomes in GFAP astrocytopathy.
