No Rheum at The Insula: Rheumatoid Lepto-meningitis as Cause of Focal Seizure
Background:
Patient is a 65 year old female with history of rheumatoid arthritis, MGUS, SJogrens disease, CAD, aortic aneurysm with dissection; who presented with recurrent episodes of leg weakness and shaking. Initial symptoms began 1 month prior to presentation, with interim progression and increasing frequency. The symptoms would start without prodrome, involve uncontrollable movements, and could last several hours prior to spontaneous resolution.
Results:
Neurologic exam revealed LLE hyperreflexia with upgoing babinski and was otherwise normal. Initial differential included stroke with subsequent epileptogenic focus, transverse myelitis, methotrexate toxicity, Behcets, infectious process, and atlantoaxial subluxation. MRI of the spine without evidence of disease. MRI brain showed leptomeningeal enhancement of the right frontoparietal vertex, with MRA showing multifocal hyperintensities with wall irregularities concerning for CNS vasculitis. Lumbar puncture and MR black blood imaging was performed, along with vasculitis workup. MR black blood returned with continued concerns for vasculitis. Lumbar puncture showed 22 wbc, with xanthochromia, normal protein. Rheumatologic workup with Complement levels and SPEP were all reassuring, with only minimal elevation of CRP at 10.4 and ESR 31. Infectious work-up, including for opportunistic infections, was negative. Neurosurgery was consulted for biopsy which was positive for amyloid angiopathy, with rheumatology indicating probable diagnosis of rheumatoid-associated vasculitis. While in the hospital, the patient was witnessed to have an episode of her presenting symptoms of left leg shaking which was observed to be rhythmic and non-distractible. Routine EEG, showed no evidence of epileptiform activities, however the patient was treated with levetiracetam without return of her symptomatology.
Conclusions:
Rheumatoid arthritis is a known systemic disease that can potentially and rarely affect other organs including the CNS. In this case, the patient presented with focal seizures secondary to cortical irritation caused by leptomeningeal enhancements of her right hemisphere.