Arachnoiditis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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Overview
Arachnoiditis is a term used to describe thickening, inflammation, and scaring of the arachnoid membrane, which is the middle layer surrounding the central nervous system. These abnormalities may be self-limited or may cause compression of the nerve roots and spinal cord. Patients may report a variety of clinical symptoms, including severe back pain that persists at rest, perineal/saddle numbness, neurological deficits, skin rashes, and sympathetic or vascular changes. The cause of arachnoiditis is unknown but may occur as a result of invasion (surgery), neuraxial injections, certain myelograms, infections, blood, a herniated disc, or tumors. Diagnosis is based on symptoms, and magnetic resonance imaging. Unfortunately, the treatment of arachnoiditis is difficult and limited and should focus on symptomatic management.
Historical Perspective
The phenomenon of adhesive arachnoiditis was first described by Quinke in 1893 in a case report. In 1897, Schwarz wrote about signs and symptoms of arachnoiditis caused by syphilis. Elkington wrote about the classic description of arachnoiditis in 1936 and termed it as meningitis serosa circumscripta spinalis. He also described various presentations of arachnoiditis in 1951. Foix and Alajouanine described a noninfectious spinal arachnoid scar reaction in 1926 and termed it as hypertrophic vascular ascending myelitis. Ransome and Monterio mentioned that tuberculous meningitis might present as extensive spinal arachnoiditis. The first case of arachnoiditis was associated with syphilis.[1][2][3]
Classification
The radiologists classify arachnoiditis, according to Delamarter's MRI classification.
• Type I—appears as an adhesive mass of adherent roots centrally in the thecal sac, considered mild arachnoiditis.
• Type II—the empty sac, the roots adhere peripherally to the meninges.
• Type III—a soft-tissue mass replaces the subarachnoid space.
Wilkinson developed another classification system that divided the myelographic appearances into four groups.
• Type I: A unilateral focal defect centered on the nerve root exit pouch adjacent to the intervertebral disc space.
• Type II: An annular or a circumferential defect with a bilateral notch and only filiform passage of the medium.
• Type III: Complete transverse obstruction with the picture of stalagmites, candle-guttering, or paintbrush defects.
• Type IV: An infundibuliform cul-de-sac with loss of radicular striation, a vitreous appearance, and cutting-off of the root sleeves.
Pathophysiology
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Causes
Common causes of arachnoiditis include the following.[4]
- Mechanical injury during spinal surgery ( about 60% cases)
- Spinal trauma
- neuroaxial (spinal and epidural) anesthesia ( about 22% cases)
- one or more spinal tabs (7% cases)
- myelography
- bacterial and viral spinal infections (7%)
- Spinal tumors
- Repeated subarachnoid injections of anticancer drugs or antimetabolites
- Spinal cord contamination
Differentiating arachnoiditis from other Diseases
Arachnoiditis is a clinical diagnosis that is supported by a combination of clinical history, physical examination, and radiography. Following pain syndromes may mimic arachnoiditis and should be considered in the differential diagnosis.[5][6][7]
- spinal cord tumors
- cauda equina syndrome
- arachnoiditis ossificans,
- failed back syndrome
- syringomyelia
- Spinal cord infections
Epidemiology and Demographics
Arachnoiditis is a rare disorder. Its exact incidence is hard to determine as its difficult-to-diagnose condition with a wide range of clinical presentations. Some cases of arachnoiditis may go undiagnosed or misdiagnosed. Therefore the exact incidence of arachnoiditis remains not only unknown but also likely significantly underestimated. However, recently the number of lumbar arachnoiditis cases are increasing due to lumbar supine surgeries and spinal anesthesia.[8] [9]
Arachnoiditis affects more females than males because two-thirds of pregnant females receive spinal or epidural anesthesia during child delivery.
Diagnosis
History and Symptoms
Patients suggestive of arachnoiditis should be asked about any history of spinal trauma, spinal cord infections, spinal surgery, or spinal anesthesia. Sometimes an inciting event can not be identified. Arachnoiditis symptoms vary from subclinical to advance and serve. Symptoms of arachnoiditis are challenging to distinguish from other types of compressive neurological diseases because many of the symptoms are similar, and the disease onset can be months to years from the initial injury. Patients will complain about pain, numbness, paraesthesia, and tingling in affected nerve root or roots.
- Chronic and persistent pain in the lower back that may radiate down the legs
- Bowel/bladder, sexual dysfunction
- Muscle cramps, spasms, and uncontrolled twitching
- Burning in the ankles and feet
- Unexplained skin rashes and itching
- Loss of sensation below the area afflicted
- Partial or complete paralysis of the lower extremities
- Neurologic deficits
Physical Examination
Laboratory Findings
Imaging Findings
Other Diagnostic Studies
Treatment
Arachnoiditis is a difficult condition to treat. Treatment is limited to alleviation of pain and other symptoms. Surgical intervention generally has a poor outcome and only provides temporary relief. Steroid injection is generally discouraged and may worsen the condition.
Medical Therapy
Surgery
Prevention
Future or Investigational Therapies
Recent research has indicated that a group of chemicals called cytokines that are produced by various cells in the body may be responsible for generating the pain response. Medications that affect the release of cytokines or block the action of cytokines may reduce the pain response. Various anti-cytokine medications are now being used to treat painful disease states such as Rheumatoid Arthritis and Crohn's Disease. In a recent study the anti-cytokine medication, Thalidomide, is being evaluated for its effect in treating pain associated with Arachnoiditis.
External links
- Circle Of Friends With Arachnoiditis (COFWA)
- American Chronic Pain Association (ACPA)
- National Chronic Pain Outreach Association (NCPOA)
- National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
- National Institute of Dental and Craniofacial Research (NIDCR)
- Arachnoiditis
- Arachnoiditis, if you have ever suffered from back pain or know anyone who has then this is where to find help and information.
- Support for those with disorders of the Arachnoid Membrane
- http://www.burtonreport.com/InfSpine/AdhesArachAnatomy.htm
References
- ↑ Elkington, J. St. C. (1951). Arachnoiditis. In Modern Trends in Neurology, ed. Anthony Feiling, ch. 5, pp. 149-161. Butterworths, London.
- ↑ ELKINGTON, J. St. C. (1936). Meningitis serosa circumscripta spinalis (spinal arachnoiditis). Brain, 59, 181-203
- ↑ Foix C, Alajouanine T . La myélite nécrotique subaigue. Rev Neurol (Paris) 1926; 2: 1–42.
- ↑ "Antonio Aldrete. Arachnoiditis. Orphanet. March, 2010".
- ↑ Vloeberghs M, Herregodts P, Stadnik T, Goossens A, D'Haens J. Spinal arachnoiditis mimicking a spinal cord tumor: a case report and review of the literature. Surg Neurol. 1992;37(3):211-215. doi:10.1016/0090-3019(92)90233-d
- ↑ Wright MH, Denney LC. A comprehensive review of spinal arachnoiditis. Orthop Nurs. 2003;22(3):215-221. doi:10.1097/00006416-200305000-00010
- ↑ Martin RJ, Yuan HA. Neurosurgical care of spinal epidural, subdural, and intramedullary abscesses and arachnoiditis. Orthop Clin North Am. 1996;27(1):125-136.
- ↑ Anderson TL, Morris JM, Wald JT, Kotsenas AL. Imaging Appearance of Advanced Chronic Adhesive Arachnoiditis: A Retrospective Review. AJR Am J Roentgenol. 2017;209(3):648-655. doi:10.2214/AJR.16.16704
- ↑ Todeschi J, Chibbaro S, Gubian A, Pop R, Proust F, Cebula H. Spinal adhesive arachnoiditis following the rupture of an Adamkiewicz aneurysm: Literature review and a case illustration. Neurochirurgie. 2018;64(3):177-182. doi:10.1016/j.neuchi.2017.11.003