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Pseudotumor cerebri.

Pseudotumor cerebri is a clinical syndrome in which signs and, sometimes, symptoms of raised intracranial pressure are present but in which mental and neurological function are unaffected. Therefore, the diagnosis is reached after mass and other structural causes of raised pressure have been excluded. Many causes of pseudotumor have been suggested, not all of them well documented. Pathogenesis, however, includes cerebral edema, increased cerebral blood volume, and decreased cerebro spinal fluid (CSF) absorption. Except for the risk of vision in a minority of cases, the prognosis is excellent.

Abducens Nerve

Utilization, reliability, and cost effectiveness of cranial computed tomography in evaluating pseudotumor cerebri.

Cranial computed tomography (CT) has reduced the frequency of cerebral angiography from 95% to 32% and pneumoencephalography from 71% to 11% in patients with pseudotumor cerebri. Total hospital stay has been significantly reduced from a mean of 19.6 to 13.3 days and the hospital days required for diagnosis from a mean of 5.8 to 3.4. The diagnostic reliability of CT in pseudotumor cerebri was estimated using Bayes's decision theorem. The combination of CT and radionuclide brain scans provide diagnostic accuracy similar to radiologic contrast studies. The diagnosis of pseudotumor cerebri can be adequately established without cerebral angiography and air studies. CT has significantly reduced the morbidity, time, and cost of diagnostic evaluation in these patients.

Brain

Cerebrospinal fluid dynamics in patients with pseudotumor cerebri.

CSF dynamics were determined as a function of intracranial pressure in patients with pseudotumor cerebri. Servocontrolled variable rate lumbar infusions were used to determine net CSF-absorptive capacities and resting pressures in 10 patients; serial studies were done in 5 of the patients. Nearly all of the patients had abnormally low CSF-absorptive capacities. On the other hand, marked elevations in resting pressure were not a constant feature of the disease. Concurrent changes in the cerebrovascular bed could introduce errors into this manometric determination of CSF dynamics; the significance of this potential artifact is examined. The results of this study suggest that the CSF compartment may be of etiological importance in the pathophysiology of pseudotumor cerebri.

Adolescent

Plasma renin activity and aldosterone determination in patients with pseudotumor cerebri.

A group of 6 women diagnosed as suffering from pseudotumor cerebri had measurement of their plasma renin activity and aldosterone levels at baseline, following plasma volume contraction by furosemide and after dexamethasone therapy. In each instance, the values recorded fell within the normal range and a normal renin-aldosterone axis is postulated.

Adolescent

An acquired abnormal fibrinogen associated with thromboembolic disease and pseudotumor cerebri.

An abnormal fibrinogen was found in a patient associated with disabling recurrent phlebitis and pulmonary emboli, pseudotumor cerebri, gout and endometriosis. The fibrinogen is characterized by (1) abnormal side-to-side and end-to-end polymerization, (2) abnormal fibrinopeptide release, (3) a delayed gamma-gamma dimerization of the non cross-linked fibrin, (4) a pH optimum of 7--7.8, and (5) a deviation from normal amino acid composition with regard to lysine, aspartic acid, glutamic acid and serine. Since no defect has been found in any of her three children, and since the prothromin and partial thromboplastin times vary from time to time, it is assumed that the defect is acquired. Liver disease, usually associated with acquired abnormal fibrinogen, has been excluded as an etiological cause since liver function tests and biopsy are completely normal.

Amino Acids

Subretinal neovascularization and papilledema associated with pseudotumor cerebri.

A 31-year-old man, on routine ocular examination, was found to have bilateral papilledema. Neurologic evaluation confirmed elevated cerebrospinal pressure with no mass lesion and a diagnosis of pseudotumor cerebri was made. A tiny subretinal hemorrhage adjacent to the right optic disk was found to be secondary to subretinal neovascularization. Over the course of several months, the papilledema resolved. However, the neovascular membrane extended further toward the fovea and was subsequently obliterated with argon laser photocoagulation.

Adult

An unusual presentation of pseudotumor cerebri.

A 25-year old grossly obese woman presented with decreased visual acuity. She had bilateral total (internal and external) ophthalmoplegia, a left-sided seventh cranial nerve palsy, and florid bilateral papilledema. The diagnosis of pseudotumor cerebri was made on the basis of high cerebrospinal fluid pressure and the absence of other abnormalities on systemic, neurologic, and neuroradiologic examination. The patient responded favorably to corticosteroid treatment; however, bilateral optic atrophy occurred with persistent loss of visual field and acuity.

Adult

Pseudotumor cerebri.

The authors evaluate the case of a 20-year-old, four-month-pregnant woman who presented with papilledema and visual field loss, and developed headache, intermittent diplopia and impairment of color vision over the next month. The procedures and considerations leading to the diagnosis of pseudotumor cerebri are discussed, as are the various modes of therapy and their indications.

Adult