Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)
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Diagnostic criteria of idiopathic intracranial hypertension (IIH) include the following:
- Signs and symptoms of increased intracranial pressure (e.g., headache, vomiting, ocular manifestations, and papilledema)
- Elevated cerebrocranial fluid pressure but otherwise normal CSF
- Normal neurologic exam except for papilledema (occasional abducens or other motor cranial neuropathy)
- Normal neuroimaging study (or incidental findings only)
- Boys and girls are affected equally in childhood; in adulthood, more women than men are affected.
- IIH has been reported in patients as young as 4 months of age, with a median age of 9 years.
Estimated to be 0.5/100,000 cases/year for children younger than 18 years old
Sporadic, no clear genetic predisposition, unless related to an underlying hormonal, toxic, or inflammatory condition; no data are available in children.
Pathogenesis unknown but may involve decreased CSF absorption owing to arachnoid villi dysfunction or elevated intracranial venous pressure. For example, obesity may lead to increased intra-abdominal, intrathoracic, and cardiac filling pressure, leading to elevated intracranial venous pressure.
- Numerous precipitants of IIH have been reported. In adolescents, it is clearly associated with obesity and weight gain but not clearly linked to obesity in children <11 years of age. Many weaker associations may be due to chance.
- IIH is often linked to minocycline, tetracycline, sulfnamides, isotretinoin, and thyroid replacements and to corticosteroid withdrawal. It is also linked to vitamin A deficiency or intoxication, chronic anemia, and hypothyroidism.
Commonly Associated Conditions
- Visual loss due to optic nerve pressure
- Endocrinopathies, exogenous steroids, lead exposure, and therapy involving tetracycline and several other antibiotics may be associated with IIH.