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.

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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