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Idiopathic syndrome caused by increased intracranial pressure with papilledema of unknown origin, most often presenting with symptoms of cerebral tumor (pseudotumor cerebri) in young obese women in their childbearing years.

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Benign Intracranial Hypertension; Pseudotumor Cerebri.

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Clinical syndrome characterized by increased intracranial pressure of unknown origin with good prognosis if medical treatment (acetazolamide) is regularly taken. Severe cases (visual impairment) require neurosurgery.

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The incidence in the United States has been reported to range from 0.9-1.0:100,000 in the general population; however, this rate is estimated to be 1.6-3.5:100,000 in women and 7.9-19:100,000 in overweight women. Internationally, the incidence varies considerably among countries. However, the relationship between personal habitus such as obesity has been confirmed. In Libya, it has been demonstrated that the incidence was estimated at 2.2:100,000 in the general population, 4.3:100,000 in women, and 21.4:100,000 in obese women.

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Idiopathic intracranial hypertension is a clinical syndrome characterized by an elevated intracranial pressure with normal cerebrospinal fluid (CSF) composition and no evidence of any expanding intracranial mass lesion or significant hydrocephalus. In all cases, increased vitamin A concentration is measured in the CSF of affected women. Females are more affected than males. Most often, no specific cause is found; however, multiple causes have been identified: endocrine and metabolic disorders (hypoparathyroidism, pseudohypoparathyroidism, corticosteroid therapy withdrawal, growth hormone treatment, hypervitaminosis A, Addison disease, obesity, menarche, oral contraceptives, pregnancy, galactosemia), hematologic disorders (iron deficiency and hemolytic anemia, polycythemia, Wiskott-Aldrich syndrome), infections (roseola infantum, chronic otitis media and mastoiditis, Guillain-Barré syndrome), drugs (tetracyclines, nalidixic acid), and obstruction of intracranial drainage by venous thrombosis (head injury, lateral or posterior sagittal sinus thrombosis, obstruction of superior vena cava).

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Clinical course; CT scan or MRI excluding any structural cause for increased intracranial pressure and showing normal ventricles; lumbar puncture revealing elevated CSF opening pressure and normal CSF composition (chemistry, cytology, bacteriology). Lumbar puncture is therapeutic, allowing CSF to escape, thereby reducing the intracranial pressure. Digital subtraction angiography may be performed if there is suspicion of venous thrombosis.

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Some patients are asymptomatic. Infants may present with irritability, bulging fontanelle, and a resonant sound on skull percussion (MacEwen sign). Headache, pulsatile intracranial noises, occasional vomiting, transient visual obscuration, diplopia secondary to abducens nerve paralysis, and intermittent gait ataxia are possible findings in children and adults. Examination reveals papilledema without significant focal neurologic signs. Visual loss from optic neuropathy may occur as a long-term sequela. Treatment is aimed at controlling intracranial pressure in order to preserve the vision.

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Obtain a full history of any associated disorder (endocrine, hematologic, drug-related, infectious, or obstructive secondary to venous thrombosis). Inquire about the use of therapy to control the presence of benign intracranial hypertension, such as corticosteroids, diuretics (acetazolamide with/without furosemide), repeated therapeutic lumbar punctures, and surgical lumboperitoneal shunting. Obtain electrolytes and glucose levels for patients on steroid or diuretic therapy.

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In patients receiving steroids, consider “stress-dose” ...

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