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Idiopathic intracranial hypertension 

Idiopathic intracranial hypertension

Chapter:
Idiopathic intracranial hypertension
Author(s):

N.F. Lawton

DOI:
10.1093/med/9780199204854.003.0241005

A relevant case history from Neurological Case Histories: Case Histories in Acute Neurology and the Neurology of General Medicine has been added to this chapter.

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date: 27 March 2017

Idiopathic intracranial hypertension is a syndrome of raised intracranial pressure in the absence of an intracranial mass lesion, enlargement of the cerebral ventricles due to hydrocephalus, or venous thrombosis in the dural sinuses. Impaired cerebrospinal fluid absorption and raised cerebral venous pressure have both been implicated. Obese females of childbearing age are predominantly affected. Rarely, provocative factors include tetracycline, vitamin A derivatives, hypervitaminosis A, and vitamin A excess.

Clinical features

Characteristic presentation is with headache which may be typical of raised intracranial pressure but is frequently non-specific. Papilloedema is almost invariably present, but often not severe and may be unilateral; visual field defects are common. There are no focal cerebal signs, and a history of epilepsy is strongly against the diagnosis.

Diagnosis, treatment and prognosis

After brain imaging to exclude other causes of raised intracranial pressure, diagnosis depends on measurement of cerebrospinal fluid pressure higher than 200 mm. Composition of fluid is normal. Spontaneous remission and relapse is common, but treatments including the following aim to prevent symptoms and visual loss, which should be monitored by serial analysis of visual fields: (1) high-dose corticosteroids—for relief of acute crises, but not as long-term treatment; (2) drugs—the carbonic anhydrase inhibitors acetazolamide, and thiazide diuretics are used in mild cases; (3) surgery—some patients require lumboperitoneal shunts, optic nerve decompression, or other procedures; (4) intermittent lumbar puncture—particularly of use in pregnancy. Aside from visual loss, serious complications are rare.

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