Autoimmune glial fibrillary acidic protein astrocytopathy mimicking acute disseminated encephalomyelitis: A case report.
Medicine (Baltimore)
; 100(25): e26448, 2021 Jun 25.
Article
en En
| MEDLINE
| ID: mdl-34160439
ABSTRACT
INTRODUCTION:
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an increasingly recognized type of steroid-responsive autoimmune disease of the nervous system. Defined in 2016, it is associated with the presence of anti-GFAP immunoglobulinG in the serum or cerebrospinal fluid (CSF) of affected patients. PATIENT CHARACTERISTICS Herein, we report a case of acute neurological symptoms, including headache, fever, confusion, and paralysis of the lower extremities. CSF analysis revealed lymphocytic pleocytosis and elevated protein levels, indicating acute disseminated encephalomyelitis, and the patient was given immunotherapy. Cranial magnetic resonance imaging showed multifocal T2/fluid-attenuated inversion recovery hyperintense signal changes in the periventricular white matter, and electromyography testing showed changes consistent with severe sensorimotor neuropathy, indicating the involvement of the brain and peripheral nerves. DIAGNOSES Finally, a diagnosis of autoimmune GFAP astrocytopathy was confirmed due to the presence of GFAP-immunoglobulinG in the patient's CSF.INTERVENTIONS:
The patient was treated with one course of intravenous immunoglobulin therapy, then followed with intravenous methylprednisolone (1.0âg/d for 3âdays) and oral prednisolone.OUTCOMES:
At 1âweek after intravenous immunoglobulin therapy, his level of consciousness improved. However, flaccid paralysis persisted without substantial improvement.CONCLUSION:
In conclusion, the provision of an accurate early diagnosis and appropriate treatment are crucial for improving the prognosis of patients with autoimmune GFAP astrocytopathy. Further, this case highlights the importance of recognizing the role of peripheral nerve involvement in GFAP autoimmunity.
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Polineuropatías
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Autoanticuerpos
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Encefalomielitis Aguda Diseminada
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Sustancia Blanca
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Proteína Ácida Fibrilar de la Glía
Tipo de estudio:
Diagnostic_studies
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Prognostic_studies
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Screening_studies
Límite:
Humans
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Male
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Middle aged
Idioma:
En
Año:
2021
Tipo del documento:
Article