Pseudotumor Cerebri

Published on 27/03/2015 by admin

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Last modified 27/03/2015

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Chapter 597 Pseudotumor Cerebri

Pseudotumor cerebri, also known as idiopathic intracranial hypertension, is a clinical syndrome that mimics brain tumors and is characterized by increased intracranial pressure (ICP; >200 mm H2O in infants and >250 mm H2O in children), with a normal cerebrospinal fluid (CSF) cell count and protein content and normal ventricular size, anatomy, and position documented by MRI. Papilledema is universally present in children old enough to have a closed fontanel.

Etiology

Table 597-1 lists the many causes of pseudotumor cerebri. There are many explanations for the development of pseudotumor cerebri, including alterations in CSF absorption and production, cerebral edema, abnormalities in vasomotor control and cerebral blood flow, and venous obstruction. The causes of pseudotumor are numerous and include metabolic disorders (galactosemia, hypoparathyroidism, pseudohypoparathyroidism, hypophosphatasia, prolonged corticosteroid therapy or rapid corticosteroid withdrawal, possibly growth hormone treatment, refeeding of a significantly malnourished child, hypervitaminosis A, severe vitamin A deficiency, Addison disease, obesity, menarche, oral contraceptives, and pregnancy), infections (roseola infantum, sinusitis, chronic otitis media and mastoiditis, Guillain-Barré syndrome), drugs (nalidixic acid, doxycycline, minocycline, tetracycline, nitrofurantoin), isotretinoin used for acne therapy especially when combined with tetracycline, hematologic disorders (polycythemia, hemolytic and iron-deficiency anemias [Fig. 597-1], Wiskott-Aldrich syndrome), obstruction of intracranial drainage by venous thrombosis (lateral sinus or posterior sagittal sinus thrombosis), head injury, and obstruction of the superior vena cava. When a cause is not identified, the condition is classified as idiopathic intracranial hypertension.