Intracranial Hypertension, Idiopathic

Alternative Names

  • IIH
  • Pseudotumor Cerebri
  • Benign Intracranial Hypertension
  • BIH
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WHO-ICD-10 version:2010

Diseases of the nervous system

Other disorders of the nervous system

OMIM Number

243200

Mode of Inheritance

Autosomal recessive

Description

Idiopathic Intracranial Hypertension, as the name suggests, is a condition characterized by an abnormal elevation in the intracranial pressure, in the absence of any expansive lesion or ventricular dilation. Since the signs and symptoms of the condition are very similar to that of brain tumor, the disease is also known as pseudotumor cerebri (PC). Most of the symptoms are non-specific, such as headache, nausea, transient visual obscurations and double vision, rhythmic sound in the ears (pulsatile tinnitus), lethargy, mood change and dizziness. The primary hallmark of IIH is bilateral, asymmetric, or unilateral papilloedema, caused by atrophy of the optic nerve. If left untreated, the papilloedema may result in blindness. The pathophysiology of the disease is unclear. However, it is proposed that increased resistance to absorption of CSF across the arachnoid villi may be the causative factor of the increased fluid pressure.

This disease predominantly affects obese women of child bearing age, or reaching menarche. Diagnosis involves imaging techniques like CT and MRI scans of the brain, along with orbital ultrasound. The primary aim of treatment is to reduce the intracranial pressure. Diuretics and hyperosmotic drugs are administered to promote loss of fluid. Due to the condition's relationship with obesity, weight loss is a necessary part of the treatment strategy. A majority of the patients respond to medication within three to five months, failing which surgical intervention is considered. Surgery involves therapeutic shunting, wherein the spinal fluid is made to drain into the abdominal cavity via a tube.

Molecular Genetics

A genetic predisposition to the disease is likely; especially considering the reports of familial IIH. However, the gene or the locus responsible for the condition has not been identified.

Epidemiology in the Arab World

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Other Reports

Libya

Radhakrishnan et al. (1993) conducted an epidemiologic survey of idiopathic intracranial hypertension (IIH) in Benghazi, Libya, over a period from September 1982 through August 1989. The group was comprised 81 patients (76 females and 5 males). Ages ranged from 8 to 55 years. The average crude annual incidence rates for IIH per 100,000 persons were 2.2 for the total and 4.3 for females for all ages. In females aged 15-44 years, IIH occurred at a rate of 12.0 per 100,000 per year; for those defined as obese, the rate rose to 21.4. Moderate to severe visual loss occurred as a sequela in 20% of the patients. The extent of visual loss did not correlate with age at diagnosis, duration of symptoms, degree of obesity, use of oral contraceptive pills, cerebrospinal fluid (CSF) opening pressure, steroid treatment, or recurrence. Radhakrishnan et al. (1993) found no correlation between CSF protein and opening pressure. Radhakrishnan et al. (1993) also conducted a case-control study on 40 consecutive female incident IIH patients and 80 age-matched female control subjects. Obesity and recent weight gain occurred more frequently in patients. More patients were married and more had irregular menses.

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