Abstract
Purpose: :
To identify cases of neurosarcoidosis mimicking Idiopathic Intracranial Hypertension (IIH)
Methods: :
Retrospective descriptive study of patients presenting with clinical signs and symptoms suggestive of idiopathic intracranial hypertension who were later found to have neurosarcoidosis. With IRB approval, the database of cases of known sarcoidosis from New Jersey Medical School Neuroophthalmology Service 1989 to 2010 was investigated. 125 cases were identified, of which 75 records were recovered. Patients presenting with papilledema and no prior history of sarcoid were chosen, with attention to results on chest x-ray/CT, gallium scan, ACE level and spinal fluid.
Results: :
Three patients were studied, 3 female, 0 male. Findings critical in diagnosis of sarcoid in these patients included abnormal chest-xrays (n=2), clinically enlarged lacrimal glands (n=2), panuveitis (n=1), abnormal gallium scans suggestive of sarcoid (n=2) and high ACE levels (n=2). No cases had spinal fluid findings that were suggestive of sarcoid. One additional patient had a history of sarcoidosis and was thought to have unrelated IIH, and after failing treatment with acetazolamide; was successfully treated with methotextrate and plaquenil to treat what was unsuspected neurosarcoid.
Conclusions: :
Patients who have negative brain imaging and papilledema initially thought to have idiopathic intracranial hypertension may still harbor occult neurosarcoid. Even normal spinal fluid composition may not rule out neurosarcoid as the source of papilledema.
Keywords: neuro-ophthalmology: optic nerve • neuro-ophthalmology: diagnosis