Objective:

NA

Background:

While Sjogren’s disease occasionally affects the central nervous system (CNS), it is rare for it to be the initial and sole symptom. Here, we describe a rare case of a young patient that developed meningoencephalitis and focal seizures preceding the diagnosis of Sjogren's disease.  

Design/Methods:

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Results:

A previously healthy 20-year-old woman who presented with fever, headache, and neck stiffness for two weeks with subsequent episodes concerning for focal seizures, described as staring episodes followed by right arm posturing and generalized convulsions. During admission, the patient was increasingly encephalopathic with clinical signs concerning meningoencephalitis for which she was started on empiric antimicrobials. Lumbar puncture revealed cerebrospinal fluid pleocytosis (29 Cells/uL) with lymphocytic predominance (48%), but normal protein (18 mg/dL), glucose (90 mg/dL), and negative infectious workup. MRI of the brain was notable for a small developmental venous anomaly in the left inferior frontal lobe with prominence of ventricular size and sulci for age. Upon further review, there was faint FLAIR-hyperintense signal within the left temporal lobe parenchyma and possible meningeal enhancement along the left frontal and insular regions. Her EEG demonstrated focal slowing in the left frontal lobe. Antiepileptic treatment was initiated. Rheumatological workup revealed high anti-nuclear antibody (ANA) titer (>1:640) with positive anti-SSA/Ro antibodies (>8 immunoassay) after which high-dose methylprednisolone was started. Her overall neurologic function improved significantly, and she was able to be tapered off anti-seizure medications.  

Conclusions:

This case highlights the importance of thorough rheumatological workup, as new onset seizures and meningoencephalitis can be the first manifestation of Sjogren's disease.