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Arachnoiditis
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Arachnoiditis

Important
It is possible that the main title of the report Arachnoiditis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms

* Arachnitis
* Cerebral Arachnoiditis
* Chronic Adhesive Arachnoiditis
* Serous Circumscribed Meningitis
* Spinal Arachnoiditis

Disorder Subdivisions

* Adhesive Arachnoiditis
* Arachnoiditis Ossificans
* Spinal Ossifying Arachnoiditis
* Neoplastic Arachnoiditis
* Optochiasmatic Arachnoiditis
* Postmyelographic Arachnoiditis
* Rhinosinusogenic Cerebral Arachnoiditis

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

* Leptomeningitis
* Epiduritis
* Pseudotumor Cerebri

General Discussion

Arachnoiditis is a general term for several progressive regional disorders all of which result in the inflammation of parts of the middle membrane surrounding the spinal cord and brain (arachnoid membrane) and the space defined by this membrane (subarachnoid space). Either the spinal cord or the brain may be involved; in some cases, both are affected. This disorder can also be associated with meningitis. The condition may be caused by foreign agents such as anesthesia drugs or testing dyes injected into the spine or arachnoid membrane.

Since the subarachnoid space is continuous, it would be expected that a noxious agent introduced in one place would distribute itself throughout the space. However, such is not the case. The lower spinal roots and/or the spinal cord may be affected, while regions close by remain free of inflammation; hence, the term "spinal arachnoiditis". Similarly, the optic nerve and optic chiasm (crossing of nerve fibers) may be affected, giving rise to the term "opticochiasmatic arachnoiditis".
Symptoms

When the brain is involved, symptoms of Arachnoiditis include severe headaches, vision disturbances, dizziness, nausea and/or vomiting. When the spine is affected, pain, unusual sensations, weakness, and paralysis can develop. The disorder usually begins unexpectedly with gradual loss of sensations and movement of the lower back and legs. Inflammation, muscle atrophy, weakness, and involuntary twitching of muscles often occur. In the most severe cases, loss of vision and/or paralysis may develop. Fibrous tissue may thicken the arachnoid membrane which can also harden or ossify in some cases.

Causes

Arachnoiditis may arise as an uncommon complication of meningitis or other bacterial infection, or it may come about as a result of noxious agents, such as liquid anesthetics or radio-opaque dyes, reach the membranes. An immune deficiency in the blood may also contribute to development of this disorder.
Affected Populations

Arachnoiditis is a rare disorder affecting males and females in equal numbers. Individuals who have had spinal surgery, injection of foreign fluids into the spinal area such as dye or anesthesia, or injuries to the spine or head may be at greater risk to develop this disorder.

Related Disorders

Symptoms of the following disorders can be similar to those of Arachnoiditis. Comparisons may be useful for a differential diagnosis:

Leptomeningitis is characterized by inflammation of the soft membranes surrounding the brain and spinal cord including the pia mater and arachnoid membrane. Inflammation is centered in membranes at the base of the brain. This disorder is thought to be a complication of chronic meningitis.

Epiduritis is characterized by inflammation of the outer tough fibrous membrane surrounding the brain and spinal cord known as the dura mater.

Pseudotumor Cerebri is a condition simulating the presence of a tumor inside the skull (intracranial), probably related to congested vessels or swelling of the brain. The exact cause of this disorder is usually not known. Onset and symptoms often appear and resolve spontaneously. Headaches, vision problems, and some degree of paralysis may be associated with increased pressure inside the skull. This condition is more common in women between twenty and fifty years of age.

Standard Therapies

Arachnoiditis is usually treated by a combination of surgery and drug therapy. Surgical removal of adhesions and accumulations of foreign fluids from the arachnoid membrane and spaces surrounding the brain and spinal cord may be helpful in some cases, especially if pressure increases. In some instances, the anti-tumor drugs cyclophosphamide or nitrogen mustard are administered to ameliorate headaches and to improve vision. Anti-inflammatory drugs may be recommended. Other treatment is symptomatic and supportive.
Investigational Therapies

In very rare case, when Arachoniditis is caused by a parasitic infection, the drug praziquinatel showed benefits for at least a year in forty-seven percent of arachnoiditis patients in one study. However, in the United States, most cases of Arachnoiditis are not caused by parasites.

References

McKusick VA., ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No: 182950; Last Edit: 3/25/1995.

TEXTBOOKS
Thoene JG., ed. Physicians’ Guide to Rare Diseases. Montvale, NJ: Dowden Publishing Company Inc; 1995:268-69.

Bennett JC, Plum F., eds. Cecil Textbook of Medicine. 20th ed. Philadelphia, PA: W.B. Saunders Co; 1996:2148.

Adams, RD, et al., eds. Principles of Neurology. 6th ed. New York, NY: McGraw-Hill, Companies; 1997:208-09, 638-39, 1262.

REVIEW ARTICLES
Ribeiro C, et al., [Adhesive lumbar arachnoiditis]. Acta Med Port. 1998;11:59-65. Portugese.

Wedel DJ., Evaluation of neurologic complications after regional anesthesia. Anesth Analg. 1998;Suppl:147-52.

Kriss TC, et al., Symptomatic spinal intradural arachnoid cyst development after lumbar myelography. Case report and review of the literature. Spine. 1997;22:568-72.

JOURNAL ARTICLES
Hosseini H, et al., Sarcoid related optochiasmatic arachnoiditis: favorable outcome confirmed with MRI. J Neurol Neurosurg Psychiatry. 1999;67:690.

Bilgen IG, et al., Adhesive arachnoiditis causing cauda equina syndrome in ankylosing spondylitis: CT and MRI demonstration of dural calcification and a dorsal dural diverticulum. Neuroradiology. 1999;41:508-11.

Parker F, et al., [Non-traumatic arachnoiditis and syringomyelia. A series of 32 cases]. Neurochirurgie. 1999;45 Suppl 1:67-83. French.

Jean WC, et al., Cervical arachnoid cysts after craniocervical decompression for Chiari II malformations: report of three cases. Neurosurgery. 1998;43:941-44; discussion 944-45.

Tseng Sh, et al., Surgical treatment of thoracic arachnoiditis with multiple subarchnoid cysts caused by epidural anesthesia. Clin Neurol Neurosurg. 1997;99:256-58.
Resources

American Syringomyelia Alliance Project, Inc.
P.O. Box 1586
Longview, TX 75606-1586
USA
Tel: 9032367079
Fax: 9037577456
Tel: 8002727282
Email: info@asap.org
Internet: http://www.asap.org

Spinal Cord Society
19051 County Hwy. 1
Fergus Falls, MN 56537
USA
Tel: 2187395252
Fax: 2187395262
Internet: http://users.aol.com/scsweb

American Paraplegia Society
75-20 Astoria Boulevard
Jackson Heights, NY 11370-1177
USA
Tel: 7188033782
Fax: 7188030414
Email: aps@UnitedSpinal.ORG
Internet: http://www.apssci.org

National Spinal Cord Injury Association
6701 Democracy
Suite 300-9
Bethesda, MD 20817
USA
Tel: 3012144006
Fax: 3015889414
Tel: 8009629629
Email: NSCIA2@aol.com
Internet: http://www.spinalcord.org

NIH/NINDS Brain Resources and Information Network
PO Box 5801
Bethesda, MD 20824
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Internet: http://www.ninds.nih.gov/

Canadian Syringomyelia Network
69 Penny Crescent
Markham
Ontario, L3P 5X7
Canada
Tel: 9054718278
Fax: 9059444844
Email: barb@csn.ca
Internet: http://www.csn.ca

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. 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.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated: 2/7/2000
Copyright 1988, 1990, 2000 National Organization for Rare Disorders, Inc.

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