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BACKGROUND  Spontaneous intracranial hypotension (SIH) is a well-documented condition that typically follows a defined clinical course. Previously published studies describing the pathophysiology of SIH have demonstrated extensive evidence of low intracranial pressure (ICP) driving the clinical features of the condition. Through lumbar puncture and use of intracranial monitoring devices, however, both low and normal cerebrospinal fluid (CSF) pressures have been documented. This report outlined and discussed the unique finding of elevated ICP associated with clinical features of SIH.    OBSERVATIONS  Here, the authors presented a case of a patient with spontaneous spinal CSF leak who developed tonsillar herniation, cerebral edema, and subsequent episodes of elevated ICP. Although more diverse presentations of SIH are being reported, the authors believed the case to be unique because SIH was accompanied by elevated ICP.    LESSONS  This case adds to the growing body of literature surrounding SIH by demonstrating that patients can develop elevated CSF pressures associated with acute encephalopathy.

Spontaneous intracranial hypotension complicated by diffuse cerebral edema and episodes of severely elevated intracranial pressure: illustrative case