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Rheumatoid disease: an unusual cause of relapsing meningoencephalitis
Sian K Alexander1, Maria Di Cicco2, Ute Pohl3
1Department of Neurology, Queen's Hospital, Romford, UK.
Abstract:
A 73-year-old man presented with three episodes of dysphasia and disinhibited behaviour, a single seizure and transient ischaemic attack-like events characterised by right arm and/or leg weakness. These episodes were separated by month-long asymptomatic intervals. Medical history included rheumatoid arthritis, which was clinically quiescent on leflunomide.Repeated cerebrospinal fluid examination showed a persistent lymphocytosis with mildly reduced glucose and elevated protein; oligoclonal bands and viral PCR were negative. MRI of the brain was initially normal, but 7 months after initial presentation revealed meningeal enhancement with bifrontal cortical hyperintensities on T2/fluid-attenuated inversion recovery. Brain biopsy demonstrated necrotising granulomatous meningitis with mixed T cell and B cell infiltrates and without evidence of vasculitis or infection. Serum anticyclic citrullinated peptide antibodies were strongly positive.The diagnosis of rheumatoid meningoencephalitis was made on the basis of brain biopsy findings and serological evidence of active rheumatoid disease. Steroids and rituximab therapy were started leading to clinical stabilisation.
Insights
Rheumatoid meningoencephalitis, a rare neurological complication of rheumatoid arthritis, can present with varied symptoms. Early diagnosis and treatment with steroids and rituximab are crucial for clinical stabilization.
Area of Science:
- Neurology
- Immunology
- Rheumatology
Background:
- Rheumatoid arthritis (RA) is a chronic autoimmune disease primarily affecting joints.
- Neurological complications of RA are uncommon but can be severe.
- Rheumatoid meningoencephalitis (RME) is a rare manifestation involving central nervous system inflammation.
Observation:
- A 73-year-old male with quiescent RA presented with episodic neurological symptoms including dysphasia, disinhibited behavior, seizures, and transient ischemic attack-like events.
- Cerebrospinal fluid analysis revealed persistent lymphocytosis, mildly reduced glucose, and elevated protein.
- Brain MRI showed meningeal enhancement and bifrontal cortical hyperintensities, with biopsy confirming necrotizing granulomatous meningitis.
Findings:
- The patient had strongly positive anti-cyclic citrullinated peptide (anti-CCP) antibodies.
- Brain biopsy revealed necrotizing granulomatous meningitis with mixed T and B cell infiltrates, excluding vasculitis or infection.
- Diagnosis of RME was established based on clinical presentation, biopsy, and serological markers of active RA.
Implications:
- This case highlights the importance of considering RME in RA patients with unexplained neurological symptoms.
- Prompt diagnosis and initiation of immunosuppressive therapy, including steroids and rituximab, can lead to clinical stabilization.
- Further research into the pathogenesis and optimal management of RME is warranted.
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