Primary Sjögren’s syndrome (pSS), a chronic autoimmune disorder primarily affecting the exocrine glands, typically presents with sicca symptoms. Neurological involvement in pSS is uncommon as an initial presentation. Ischemic stroke, in particular, is a rare manifestation, while seizures are more commonly secondary to underlying structural or inflammatory central nervous system involvement. Here, we report a 41-year-old female who presented with a generalized tonic-clonic seizure preceded by left-sided tinnitus and associated with tongue biting, ocular deviation, and postictal confusion. Notably, seven years earlier, she had experienced a neurological event characterized by sudden-onset left-sided weakness and mouth deviation, with neuroimaging at that time reportedly demonstrating an acute infarction in the right temporoparieto-occipital region. During the current admission, brain imaging demonstrated chronic encephalomalacia and gliosis in the same region without evidence of acute infarction. Electroencephalography revealed focal epileptiform discharges in the left temporal lobe. Extensive evaluation of stroke etiologies was unrevealing. Subsequent immunological testing revealed positive anti-Sjögren’s syndrome-related antigen A (SSA)/Ro and antinuclear antibodies (ANA). Although sicca symptoms were not reported at the time of the initial neurological presentation, the patient subsequently developed dry eye and dry mouth symptoms, prompting further evaluation with Schirmer’s test and labial salivary gland biopsy, which confirmed the diagnosis of pSS. This case highlights the importance of considering autoimmune disorders, including pSS, in selected patients with cryptogenic neurological manifestations. It also underscores the diagnostic challenges posed by atypical presentations in the absence of classic sicca symptoms. Further studies are needed to better understand the relationship between pSS and cerebrovascular disease.
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