Rare Rheumatology News

Disease Profile

Arachnoiditis

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

All ages

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ICD-10

G03.9

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Spinal arachnoiditis

Categories

Nervous System Diseases

Summary

Arachnoiditis is a pain disorder caused by inflammation of the arachnoid, one of the membranes that surrounds and protects the nerves of the spinal cord. The inflammation may occur due to irritation from chemicals; infection; direct injury to the spine; chronic compression of spinal nerves; or complications from spinal surgery or other spinal procedures. It may result in scar tissue and adhesions, which cause the spinal nerves to “stick” together. If arachnoiditis affects the function of nerves, it can cause symptoms such as numbness, tingling, and a characteristic stinging and burning pain in the lower back or legs. In some people. it may affect the bladder, bowel, and sexual function. Very severe arachnoiditis can result in paralysis of the legs.[1] Treatment aims to relieve pain and improve symptoms that impair function. Treatment may include pain medications, physical therapy, and psychotherapy. Surgical treatment is controversial because it offers only short-term relief and may increase formation of scar tissue.[1][2]

Symptoms

Arachnoiditis can cause a number of symptoms including numbness; tingling; loss of temperature sensation; and a characteristic stinging and burning pain in the lower back, limbs, and trunk.[1][3] Pain is often made worse by activity.[3] Some people have debilitating muscle cramps or spasms; loss of balance; tinnitus; problems with vision and hearing; or bladder, bowel, or sexual dysfunction.[1][3] In severe cases, arachnoiditis may lead to paralysis of the legs.[1]

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Abnormality of vision
Abnormality of sight
Vision issue

[ more ]

0000504
Arthralgia
Joint pain
0002829
Hearing impairment
Deafness
Hearing defect

[ more ]

0000365
Hyporeflexia
Decreased reflex response
Decreased reflexes

[ more ]

0001265
Meningitis
0001287
Muscle weakness
Muscular weakness
0001324
Paresthesia
Pins and needles feeling
Tingling

[ more ]

0003401
Tinnitus
Ringing in ears
Ringing in the ears

[ more ]

0000360
30%-79% of people have these symptoms
Anhidrosis
Lack of sweating
Sweating dysfunction

[ more ]

0000970
5%-29% of people have these symptoms
Fatigue
Tired
Tiredness

[ more ]

0012378
Hydrocephalus
Too much cerebrospinal fluid in the brain
0000238
Migraine
Intermittent migraine headaches
Migraine headache
Migraine headaches

[ more ]

0002076
Urinary bladder sphincter dysfunction
0002839
Percent of people who have these symptoms is not available through HPO
Abnormality of metabolism/homeostasis
Laboratory abnormality
Metabolism abnormality

[ more ]

0001939
Abnormality of the skeletal system
Skeletal abnormalities
Skeletal anomalies

[ more ]

0000924
Autosomal dominant inheritance
0000006
Spastic paraparesis
0002313

Cause

Arachnoiditis has many possible causes, including:[1][3][2]

  • mechanical injury during spinal surgery, or complications from spinal surgery (about 60% of cases)
  • trauma to the spinal cord
  • one or more spinal taps
  • steroid epidural injections or other injections
  • spinal and epidural anesthesia
  • myelography
  • bacterial or viral spinal infections

The initial symptoms of arachnoiditis are caused by inflammation of the arachnoid, and the formation of scar tissue and adhesions later cause the more severe symptoms as the condition progresses.

Treatment

Arachnoiditis is difficult to treat. Management aims to relieve pain and improve symptoms that impair fuction. Treatment may involve pain management, physical therapy, and psychotherapy. Surgical intervention is controversial because it generally provides only short-term relief and may increase the formation of scar tissue, making the condition worse.[1][2]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Arachnoiditis. Click on the link to view a sample search on this topic.

        References

        1. NINDS Arachnoiditis Information Page. National Institute of Neurological Disorders and Stroke (NINDS). July 8, 2015; https://www.ninds.nih.gov/disorders/arachnoiditis/arachnoiditis.htm.
        2. Arachnoiditis. NORD. 2013; https://rarediseases.org/rare-diseases/arachnoiditis/.
        3. Antonio Aldrete. Arachnoiditis. Orphanet. March, 2010; https://www.orpha.net/consor4.01/www/cgi-bin/OC_Exp.php?lng=EN&Expert=137817.
        4. Spinal Arachnoiditis. Online Mendelian Inheritance in Man (OMIM). July 2, 2012; https://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=182950.
        5. Pasoglou V., et. al. Familial adhesive arachnoiditis associated with syringomyelia. AJNR Am J Neuroradiol. June, 2014; 35(6):1232-1236.
        6. Guyer DW, Wiltse LL, Eskay ML, Guyer BH. The long-range prognosis of arachnoiditis. Spine (Phila Pa 1976). December, 1989; 14(12):1332-1341. https://www.ncbi.nlm.nih.gov/pubmed/2617363.

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