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Published on: October 17, 2018
Rheumatoid leptomeningitis: radiological alteration of cerebral hypoperfusion and subarachnoid lesions
Ken Ikeda1, Takanori Takazawa, Hirono Ito
1Department of Neurology, Toho University Omori Medical Center, Tokyo, Japan. keni@med.toho-u.ac.jp
Abstract:
A 56-year-old man with rheumatoid arthritis developed emotional lability and myoclonic seizure in the left arm, followed by fever and generalized convulsion. Brain magnetic resonance imaging (MRI) revealed leptomeningeal lesions with abnormal enhancement. MRI lesions were localized predominantly in the right cerebral subarachnoid spaces. Electroencephalogram showed epileptogenic focus at the right frontal and central points. After administration of valproate sodium improved convulsion and myoclonus, single photon emission computed tomography (SPECT) using N-isopropyl-p-(123)I-iodoamphetamine was performed. Brain SPECT displayed hypoperfusion predominantly in the right cerebral hemisphere. Cerebrospinal fluid (CSF) disclosed mild pleocytosis and marked elevations of interleukin-6 levels. Repeated CSF analyses showed cytology of class I and negative results for infectious pathogens. Methylprednisolone pulse therapy (1 g for 3 days, iv) and subsequent prednisolone administration (daily 50 mg, po) ameliorated neurological symptoms dramatically. Prednisolone was tapered to 20 mg/day for 5 months. Leptomeningeal MRI lesions were attenuated gradually followed by restoration of cerebral hypoperfusion on SPECT. He was diagnosed as rheumatoid leptomeningitis (RLM). Although clinical features of RLM exhibited variable deficits of the central nervous system (CNS), MRI failed to detect the corresponding CNS lesions. We first highlighted neuroradiological changes of cerebral hypoperfusion and leptomeningeal lesions in RLM. These neuroimages of our patient supported that leptomeningeal inflammation and the adjacent cerebrocortical ischemia could cause encephalitis-like symptoms in RLM patients.
Insights
Rheumatoid leptomeningitis (RLM) can cause encephalitis-like symptoms due to inflammation and ischemia. Neuroimaging revealed leptomeningeal lesions and cerebral hypoperfusion, which improved with treatment.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Rheumatoid arthritis (RA) is a systemic autoimmune disease.
- Central nervous system (CNS) involvement in RA, such as rheumatoid leptomeningitis (RLM), is rare.
- RLM can present with diverse neurological deficits.
Observation:
- A 56-year-old man with RA developed seizures, fever, and emotional lability.
- Brain MRI showed leptomeningeal lesions, and EEG indicated an epileptogenic focus.
- Cerebrospinal fluid analysis revealed mild pleocytosis and elevated interleukin-6 levels.
Findings:
- Brain SPECT demonstrated significant cerebral hypoperfusion in the right hemisphere.
- Treatment with methylprednisolone and prednisolone led to dramatic improvement in neurological symptoms.
- Follow-up neuroimaging showed resolution of leptomeningeal lesions and restoration of cerebral blood flow.
Implications:
- This case highlights the neuroradiological features of RLM, including leptomeningeal enhancement and cerebral hypoperfusion.
- Leptomeningeal inflammation and adjacent cortical ischemia may underlie the encephalitis-like symptoms in RLM.
- Early diagnosis and treatment of RLM are crucial for managing CNS complications in rheumatoid arthritis patients.
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