Background: Central nervous system involvement with primary Sjogren's syndrome (pSS) is rare, and often undiagnosed. Sjogren's syndrome and concomitant intracranial hypertension is a very rare event. Case: We describe a patient with pSS who presented with intracranial hypertension as the initial symptom of the disease. Conclusion: This is the first case report of a patient with pSS that presented with intracranial hypertension only.
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