Objective:

Not applicable.

Background:

Sarcoidosis is a multisystem inflammatory disorder which can have protean neurologic manifestations. Herein, we report a case of neurosarcoidosis which presented as intracranial hypertension with course complicated by death due to intracranial pressure crisis.

Design/Methods:

Not applicable.

Results:

A 21 year old woman developed subacute headaches, pulsatile tinnitus, and diplopia. Exam demonstrated papilledema and bilateral abducens nerve palsies. Initial MRI revealed T2 hyperintense, nonenhancing lesions of the left optic nerve and dorsal right pons/midbrain junction, and subsequent MRIs revealed leptomeningeal enhancement of the brain and spinal cord. Lumbar puncture was notable for opening pressure greater than 55 cm H2O (exceeded manometer limits) and CSF analysis revealed a mild lymphocytic pleocytosis (16/uL, normal 0-5), severely elevated protein (389 mg/dL, normal 15-45), and hypoglycorrhachia (29 mg/dL). Suspicion was highest for an inflammatory disease. Prednisone and acetazolamide were prescribed.

Symptoms fluctuated and doses of those medications were adjusted. Three infliximab infusions were administered, resulting in significant clinical improvement. Clear rhinorrhea (beta-2 transferrin positive) developed. Erosion of the cribriform plate was identified and repaired via transnasal endoscopic approach. She was re-admitted three days post-operatively due to nausea and vomiting, presumed from infection. Hospitalization was complicated by clinically-suspected status epilepticus and subsequent intracranial pressure crisis resulting in death by neurologic criteria. Autopsy revealed epidural and pulmonary non-caseating granulomas without evidence of infection.

Conclusions:

This case regards a rare presentation of neurosarcoidosis presenting initially with intracranial hypertension, which likely developed due to impaired CSF reabsorption secondary to chronic meningitis. Development of a CSF leak compensated for elevated pressure. Although acute sarcoid inflammation was well controlled by infliximab, CSF physiology remained impaired due to chronic meningeal scarring, resulting in ICP crisis and death after repair of CSF leak.