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Aseptic meningitis followed by mononeuritis multiplex in a patient with primary Sjögren's syndrome
Hideki Nakamura1, Yutaka Tanikawa1, Masahiro Nishihara1
1Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan.
Abstract:
A 38-year-old woman was admitted to hospital because of fever and headache. Increased cerebrospinal cell count and protein without evidence of infection led to a diagnosis of aseptic meningitis. Although she improved with acyclovir and glyceol, she experienced left forearm pain and sensory disturbance with drop fingers. Poor derivation of compound muscle action potentials in the left radial nerve was observed, leading to a diagnosis of mononeuritis multiplex with sensorimotor neuropathy. Because the patient had primary Sjögren's syndrome with anti-Ro/SS-A antibody and salivary gland hypofunction, treatment with methylprednisolone, intravenous immunoglobulin, and intravenous cyclophosphamide was followed by oral glucocorticoid therapy. After these intensive therapies, her drop fingers gradually improved, although sensory disturbance remained. In conclusion, we report a case of aseptic meningitis and subsequent mononeuritis multiplex that was successfully treated with intensive immunotherapy in a patient with primary Sjögren's syndrome.
Insights
A patient with primary Sjögren's syndrome developed aseptic meningitis and mononeuritis multiplex. Intensive immunotherapy, including cyclophosphamide, successfully treated these neurological complications.
Area of Science:
- Neurology
- Rheumatology
- Immunology
Background:
- Primary Sjögren's syndrome is an autoimmune disorder primarily affecting exocrine glands.
- Neurological manifestations in Sjögren's syndrome can be diverse, including meningitis and peripheral neuropathy.
- Aseptic meningitis and mononeuritis multiplex are rare but serious complications.
Observation:
- A 38-year-old woman presented with fever, headache, and aseptic meningitis.
- She subsequently developed left forearm pain, sensory disturbance, and drop fingers due to mononeuritis multiplex.
- Electrophysiological studies confirmed sensorimotor neuropathy in the left radial nerve.
Findings:
- The patient was diagnosed with primary Sjögren's syndrome, positive for anti-Ro/SS-A antibodies.
- Treatment involved methylprednisolone, intravenous immunoglobulin, and cyclophosphamide, followed by oral glucocorticoids.
- Neurological symptoms, particularly drop fingers, showed significant improvement after intensive immunotherapy.
Implications:
- This case highlights the importance of recognizing and managing neurological complications in primary Sjögren's syndrome.
- Intensive immunotherapy can be effective in treating aseptic meningitis and mononeuritis multiplex associated with Sjögren's syndrome.
- Early diagnosis and aggressive treatment are crucial for improving outcomes in patients with Sjögren's-related neurological disorders.
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