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- $Unique_ID{BRK04148}
- $Pretitle{}
- $Title{Pseudotumor Cerebri}
- $Subject{Pseudotumor Cerebri Benign Intracranial Hypertension Arachnoiditis
- Epiduritis Meningitis}
- $Volume{}
- $Log{}
-
- Copyright (C) 1989 National Organization for Rare Disorders, Inc.
-
- 640:
- Pseudotumor Cerebri
-
- ** IMPORTANT **
- It is possible that the main title of the article Pseudotumor Cerebri is
- not the name you expected. Please check the SYNONYM listing to find the
- alternate names and disorder subdivisions covered by this article.
-
- Synonyms
-
- Benign Intracranial Hypertension
-
- Information on the following diseases can be found in the Related
- Disorders section of this report:
-
- Arachnoiditis
- Epiduritis
- Meningitis
-
- General Discussion
-
- ** REMINDER **
- The information contained in the Rare Disease Database is provided for
- educational purposes only. It should not be used for diagnostic or treatment
- purposes. If you wish to obtain more information about this disorder, please
- contact your personal physician and/or the agencies listed in the "Resources"
- section of this report.
-
- Pseudotumor cerebri is a syndrome of increased pressure inside the skull
- (intracranial). It is most often seen in obese females between the ages of
- 20 and 50. Symptoms can mimic those of a brain tumor. However, no tumor is
- involved.
-
- Symptoms
-
- Symptoms of Pseudotumor cerebri include headache of varying degrees of
- severity (often mild), and usually unrelieved by medication. A swelling of
- the optic disc (papilledema) with progressive visual loss occurs in a small
- percentage of the patients. Occasionally, there is a loss of strength and
- energy (asthenia) and memory disturbances that may interfere with daily life.
-
- A lumbar puncture (spinal tap) may show spinal fluid pressure to be
- elevated, but the fluid content is normal or possibly will contain a small
- amount of protein. Tests of electrically activity in the brain (EEG) usually
- are normal.
-
- Causes
-
- There is no known cause for the majority of the cases of Pseudotumor cerebri.
- However, some cases may be caused by pregnancy, steroid drugs and sex hormone
- use. A parathyroid or adrenal gland disorder, venous sinus thrombosis (clot
- in the channel of the brain for venous blood) or excessive use of Vitamin A
- are other possible causes. Use of the antibiotic, tetracycline or Nalidixic
- acid (NegGram) for urinary tract infections may also cause symptoms of this
- disorder.
-
- Pseudotumor cerebri may also be caused by an increased intracranial
- pressure secondary to chronic carbon dioxide retention and a lack of oxygen
- (hypoxia).
-
- Other cases may result from iron deficiency anemia or a abnormal function
- of the parathyroid glands.
-
- Affected Population
-
- Pseudotumor cerebri is most often seen in overweight females between the ages
- of 20 to 50, but it also occurs less frequently in men of similar ages.
- Black men appear to be at a greater risk for loss of vision. The general
- population appears to be affected 0.9 per 100,000 persons. However, when
- obesity was considered, this incidence increased to 13-15 per 100,000 persons
- who are 10% overweight, and to 19.3 per 100,000 when they were 20% or more
- over their ideal weight. The female to male ratio is 8:1, and the average
- weight is 38% over ideal weight.
-
- Related Disorders
-
- Symptoms of the following disorders can be similar to those of Pseudotumor
- cerebri. Comparisons may be useful for a differential diagnosis:
-
- Arachnoiditis is a progressive inflammatory disorder affecting the middle
- membrane surrounding the spinal cord and brain (arachnoid membrane). It may
- affect both the brain and the spinal cord and may be caused by foreign
- solutions (such as dye) being injected into the spine or arachnoid membrane.
- Symptoms may include severe headaches, vision disturbances, dizziness, nausea
- and/or vomiting. If the spine is involved, pain, unusual sensations,
- weakness and paralysis can develop. (For more information on this disorder,
- choose "Arachnoiditis" as your search term in the Rare Disease Database).
-
- Epiduritis is characterized by inflammation of the outer tough canvas-
- like covering surrounding the brain and spinal cord known as the dura mater.
- Symptoms of this disorder can be similar to pseudotumor cerebri.
-
- Meningitis is an inflammation of the membranes around the brain and the
- spinal cord. It may occur as three different forms; adult, infantile and
- neonatal. It may also be caused by a number of different agents such as
- infectious bacteria, virus, or fungi, and malignant tumors. Meningitis may
- develop suddenly or have a gradual onset. Symptoms may include fever,
- headache, a stiff neck, and vomiting. The patient may also be irritable,
- confused and go from drowsiness, to stupor to coma. (For more information on
- this disorder, choose "Meningitis" as your search term in the Rare Disease
- Database).
-
- Brain tumors may also cause symptoms similar to Pseudotumor cerebri. The
- term "pseudo" means that the disorder mimics a brain tumor, but is not caused
- by a tumor.
-
- Therapies: Standard
-
- Treatment of Pseudotumor cerebri varies according to the cause. Symptoms are
- treated and relieved, but in a small percentage of the patients it may
- reoccur. Repeated lumbar punctures at daily intervals may lower pressure,
- and may relieve some of the symptoms. Occasionally a lumbar-peritoneal shunt
- (tube to drain the excess fluid) may be required to facilitate drainage from
- the brain (cranial) area into the abdomen. Other treatment may consist of
- use of diuretics and discontinuation of medications that may have caused the
- symptoms to develop.
-
- Therapies: Investigational
-
- This disease entry is based upon medical information available through April
- 1989. Since NORD's resources are limited, it is not possible to keep every
- entry in the Rare Disease Database completely current and accurate. Please
- check with the agencies listed in the Resources section for the most current
- information about this disorder.
-
- Resources
-
- For more information on Cerebri Pseudotumor, please contact:
-
- National Organization for Rare Disorders (NORD)
- P.O. Box 8923
- New Fairfield, CT 06812-1783
- (203) 746-6518
-
- NIH/National Institute of Neurological Disorders & Stroke (NINDS)
- 9000 Rockville Pike
- Bethesda, MD 20892
- (301) 496-5751
- (800) 352-9424
-
- References
-
- INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and
- Co., 1987. Pp. 2222.
-
- OPTIC NERVE HEAD DRUSEN AND PSEUDOTUMOR CEREBRI. B. Katz, et. al; Arch.
- Neurol. (January, 1988, issue 45(1)). Pp. 45-7.
-
- THE INCIDENCE OF PSEUDOTUMOR CEREBRI. POPULATION STUDIES IN IOWA AND
- LOUISIANNA. F.J. Durcan, et. al; Arch. Neurol. (August, 1988, issue 45(1)).
- Pp. 875-7.
-
- CLINICAL COURSE AND PROGNOSIS OF PSEUDOTUMOR CEREBRI. A PROSPECTIVE STUDY
- OF 24 PATIENTS. P.S. Sorenson, et. al.; Acta Neurol. Scand. (February, 1988,
- issue 77 (2)). Pp. 164-72.
-
- PSEUDOTUMOR CEREBRI IN MEN. K.B. Digre, et. al.; Arch. Neurol. (August,
- 1988, issue 45(8)). Pp. 866-72.
-
-