NMOSD or GFAP astrocytopathy? A case report.
Yue Zhang1, Abhijeet Kumar Bhekharee2, Xiang Zhang1
1Department of Neurology, Huashan Hospital, Fudan University, 12 Wulumuqi Zhong Road, Shanghai 200040, China.
Multiple Sclerosis and Related Disorders
|June 1, 2020
Summary
This case study highlights a patient with aquaporin-4 IgG negative neuromyelitis optica spectrum disorders (NMOSD) and high glial fibrillary acidic protein (GFAP) antibody levels. The patient showed significant improvement with corticosteroid treatment.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Neuromyelitis Optica Spectrum Disorders (NMOSD) are inflammatory demyelinating diseases of the central nervous system.
- Aquaporin-4 IgG seronegative NMOSD presents diagnostic challenges.
- Glial fibrillary acidic protein (GFAP) antibodies are increasingly recognized as a distinct autoimmune encephalitis marker.
Observation:
- A 43-year-old female presented with symptoms consistent with NMOSD.
- Cerebrospinal fluid analysis revealed high titers of GFAP antibodies.
- The patient exhibited a mixed clinical presentation overlapping NMOSD and GFAP antibody-associated disorders.
Findings:
- The patient met diagnostic criteria for aquaporin-4 IgG negative NMOSD.
- Elevated GFAP antibody levels were confirmed, suggesting a co-existing or related autoimmune process.
- Corticosteroid therapy resulted in a favorable clinical response.
Implications:
- This case expands the understanding of NMOSD heterogeneity.
- It underscores the importance of testing for GFAP antibodies in seronegative NMOSD cases.
- Early diagnosis and treatment with corticosteroids can be effective in managing these complex neurological conditions.
