Lupus cerebritis

Case contributed by Ryan Key
Diagnosis almost certain

Presentation

New-onset seizures.

Patient Data

Age: 30 years
Gender: Female
ct

Extensive patchy hypodensities throughout the cerebellum. Multifocal cortical and subcortical hypodensities in the cerebral hemispheres. 

mri

Innumerable subacute lacunar infarcts scattered throughout the cerebellum, and a subacute lacunar infarct in the superior aspect of the right thalamus. Extensive edema in the cortex and subcortical white matter of the cerebral hemispheres, cerebellum, and pons. Moderate narrowing of the supraclinoid ICAs and carotid termini, and questionable mild focal narrowing of the middle of the basilar artery. Findings are most consistent with a cerebritis/encephalitis and vasculitis. Differential considerations include inflammatory/autoimmune cerebritis/vasculitis or infectious meningoencephalitis. Superimposed PRES and/or seizure-related changes may contribute to the edema, although some atypical features make them unlikely to be the primary pathologic process. 

Case Discussion

The patient was diagnosed with lupus cerebritis and lupus CNS vasculitis. She had a history of lupus and lupus nephritis, resulting in end-stage renal disease and the need for hemodialysis. She presented with new-onset generalized seizures. High-dose steroids were given for the cerebral edema. Lumbar puncture performed after edema subsided was significant for a lymphocytosis and oligoclonal bands, nonspecific findings which can be seen with lupus cerebritis 1. CSF and blood bacterial, viral, and fungal studies were negative. 

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