Idiopathic Intracranial Hypertension

This condition has previously been termed benign intracranial hypertension (BIH) or pseudotumor cerebri. The intracranial pressure is raised; however, there are no localising signs, no alteration in level of consciousness, the cere-brospinal fluid composition is normal and there is no evidence of hydrocephalus or other cause of raised ICP on neuroimaging. It is thus a diagnosis of exclusion. Chronic meningitis, venous sinus thrombosis and indeed spinal cord tumors may produce a similar clinical picture. An association with various drugs, metabolic and endocrinological disorders is recognized and this needs to be considered during the clinical evaluation of these patients.

Headache and visual disturbance are the usual clinical features. Papilloedema, optic atrophy and reduced visual fields are often present and permanent visual impairment can result. Close ophthalmological surveillance of these patients is mandatory.

Preservation of vision is the main aim of treatment. Treatment options include medical therapies such as acetazolamide. Drainage of CSF, either intermittently with serial lumbar punctures or continuously by placement of a lumbo-peritoneal shunt (or ventriculo-peritoneal shunt if ventricular size permits), may be required where medical therapy fails. Optic nerve fenestration may lead to visual improvement and may be a useful adjunct in the treatment of this condition. The long-term prognosis is generally good, with spontaneous resolution in a number of cases, though the visual loss may persist.

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