Arachnoiditis (Adhesive arachnoiditis)

ARACHNOIDITIS[1]

Arachnoiditis is chronic inflammation of the arachnoid, one of the three meninges that surround and protect the central nervous system.

Presentation

Although there is no consistent pattern, the symptoms of arachnoiditis mostly involve the nerves between the lower back and the legs, and they tend to become more severe and permanent as the illness progresses. The predominant symptom is undoubtedly the chronic and unrelenting pain, which occurs mainly in the limbs and, as indicated, the lower back, although it can spread up the spine and down the arms in the later pathological phases. Numbness is also a major symptom, and can often involve other body parts, which can become extremely sensitive to touch. Among other frequent symptoms there are muscle complications such as cramps, twitches and stiffness, together with neurological disorders that affect the bladder, bowel, and sexual functions. The less frequent, but nevertheless particularly insidious, problems can include depression, difficulty in thinking clearly, and sleep disturbance, all due to the constant chronic pain.

Skin
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  • urogenital
    Sexual Dysfunction
    • dysfunction Symptoms may become more severe or even permanent if the disease progresses.[my.clevelandclinic.org]
    • dysfunction Direct injury to the spine Possible Causes: Chemicals: Dye used in myelograms (diagnostic tests in which a dye called radiographic contrast media is injected into the area surrounding the spinal cord and nerves) have been blamed for some[europeanmedicaltourist.com]
    • dysfunction As the disease progresses, the symptoms may become more severe or even permanent.[webmd.boots.com]
    • Symptoms & Signs neuropathic pain, often non-dermatomal*; mostly lower limbs, low back but may also affect upper half of the body secondary musculoskeletal pain /- fibromyalgic symptoms; joint pains; headaches bladder/bowel control dysfunction /- sexual[arachnoiditiscanada.com]
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  • Ears
    Tinnitus
    • Patients may also experience proprioception alterations (including loss of balance, tinnitus and reduced hearing and vision), motor weakness, muscle cramps, fasciculation, anhidrosis, and bladder, bowel and sexual dysfunction.[orpha.net]
    • […] back muscle cramps or spasms weakness trouble walking severe headaches vision problems hearing problems dizziness nausea bladder or bowel problems trouble sleeping fatigue joint pain loss of balance sexual dysfunction depression ringing in the ears ( tinnitus[healthline.com]
    • Other less common problems experienced include: Tinnitus (ringing in the ears), dental problems (tooth decay may be worsened by dry mouth due to loss of the protective power of saliva), abnormalities in the menstrual cycle, eyesight problems (difficulty[cofwa.org]
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  • Eyes
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  • musculoskeletal
    Back Pain
    • Forest Tennant, MD, a chronic pain specialist and lead author of the study, stated “Every pain specialist should be aware that if they see a back pain patient who claims to have severe, constant back pain with the hallmark signs of not being able to stand[njmetropain.com]
    • Experts have started to alert the medical community that some cases of back pain may be undiagnosed arachnoiditis.[pnarx.com]
    • […] started complaining about back pain and headache immediatly after birth of first child in Aug 2008.[empowher.com]
    Muscle Cramp
    • Arachnoiditis Arachnoiditis is a condition that can cause stinging, burning pain feelings, and muscle cramps and spasms.[webmd.boots.com]
    • cramps, twitching and spasms.[clinicaltrials.gov]
    • cramps, spasms and uncontrollable twitching · Bladder, bowel and/or sexual problems · As the disease progresses, symptoms may become more severe or even permanent.[powerofpain.org]
    • cramps, spasms, and uncontrollable twitching Bladder, bowel, and/or sexual dysfunction Symptoms may become more severe or even permanent if the disease progresses.[my.clevelandclinic.org]
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  • gastrointestinal
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  • respiratoric
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  • neurologic
    Confusion
    • Learn the correct uses of these two commonly confused homophones.[dictionary.reference.com]
    • Arachnoiditis is frequently confused with epidural fibrosis, a condition where scarring occurs outside the meninges.[painmanagement.org.uk]
    • Adhesive arachnoiditis is an advanced form of arachnoiditis and is most often confused with the latter.[burtonreport.com]
    • Why, fourteen years from first putting cursor to screen, am I still working to help people worldwide who are lost and confused by denial, suffering in pain and disability, yearning for a diagnosis and/or justice, because of medical procedures that the[arachnoiditis.co.uk]
    Hyporeflexia
    • Clinical description Patients present with chronic, persistent deafferentiation pain in the lower back, limbs and trunk that is increased by activity, hyporeflexia, loss of temperature sensation, numbness, and often widespread allodynia, dysesthesia and[orpha.net]
    • Some physical signs of AA include lower extremity weakness, hyporeflexia, and abnormal gait.[edsinfo.wordpress.com]
    • Criteria for diagnosis are: back pain that increases with exertion, with or without leg pain, which may be bilateral; some neurological abnormality on examination, most often hyporeflexia; and characteristic MRI findings.[academic.oup.com]
    • Serial clinical examination over 24 h revealed a rapidly ascending sensory level that stabilised at the T10 dermatome, 3/5 power, hypotonia and hyporeflexia in the lower limbs and reduced anal tone.[onlinelibrary.wiley.com]
    Dysesthesia
    • […] spine An applicant with spinal arachnoiditis must have a compromise of a nerve root or the spinal cord with: Verification of the arachnoiditis by appropriate medically acceptable imaging or, an operative note or pathology report of tissue biopsy Severe dysesthesia[disabilitycarecenter.org]
    • Clinical description Patients present with chronic, persistent deafferentiation pain in the lower back, limbs and trunk that is increased by activity, hyporeflexia, loss of temperature sensation, numbness, and often widespread allodynia, dysesthesia and[orpha.net]
    • One or more of the following may occur: Chronic neuralgia, frequently occurring in the lower back Tingling/burning/throbbing sensations, often described as a general dysesthesia Crawling sensation on the skin (patients have reported a sensation of insects[painmanagement.org.uk]
    Vertigo
    • Types of Arachnoiditis Arachnoiditis Message Boards Related forums and medical stories: Brain Conditions (288) arachnoid cyst Pain/Pressure in back of head when I laugh "ice Pick" Headaches Fluid running down back of my head feeling vertigo other symptoms[rightdiagnosis.com]
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  • Entire body system
    Weakness
    • Home , Search , Index , Links , Pathology , Molecules , Syndromes , Muscle , NMJ , Nerve , Spinal , Ataxia , Antibody & Biopsy , Patient Info SPINAL ARACHNOIDITIS Clinical syndrome Pain: Low back & radiating down both legs Weakness: One or multiple lumbar[neuromuscular.wustl.edu]
    • Generalized weakness and fatigue.[epainassist.com]
    • I needed to adjust my entire life learning to live with numbness and weakness in my left leg, bladder dysfunction and severe pain.[themighty.com]
    • They may complain of leg weakness and limited range of motion.[pnarx.com]
    • Weakness and muscle atrophy may also occur, and bowel or bladder sphincter dysfunction is not uncommon.[clinicalgate.com]
    Falling
    • Spinal Cord Society. 19051 County Hwy 1, Fergus Falls, MN 56537. (218) 739-5252.[encyclopedia.com]
    • Normally nerve roots of cauda equina should fall freely in the dependent portions of thecal sac appreciated most easily against the background of high signal intensity Csf on Axial T2 images.[neuroradiologycases.com]
    • In 2009, I had a bad fall, and landed hard on my tailbone.[powerofpain.org]
    • The course of the condition is such that it tends to fluctuate, with intermittent flare-ups, but overall most patients will plateau out' and remain fairly stable unless there is an event such as a fall, accident or further surgery, which can cause a rapid[arachnoiditiscanada.com]
    Malaise
    • Neurological problems often accompany arachnoiditis, leading to muscle jerks, spasms and muscle weakness, which in turn leads to bladder, bowel and sexual dysfunction, a swelling of the limbs, and cold extremities from poor circulation, as well as fatigue, malaise[caringmedical.com]
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  • Workup

    The diagnostic procedure of choice to identify arachnoiditis is magnetic resonance imaging (MRI). Where MRI is contraindicated, computed tomography myelography appears to be a valid alternative, although the images provided do not have the same quality as those for MRI, and thus they are not as informative. In both cases, the main pathophysiological features observed are subarachnoid space narrowing or blockage, nerve root thickening, and lack of nerve root sleeve filling. Physical examination of the patient should be added to these classical imaging findings, to determine their physiological alterations, such as for sensory deficit, reflexes, and weakness. As the symptoms of arachnoiditis vary greatly, and sometimes appear in relatively confusing combinations, this condition is often mixed up with simple irritation or compression of a single nerve.

    Treatment

    Unfortunately, there is no cure for arachnoiditis. Treatment is difficult and mostly based on relieving the pain and improving the symptoms that negatively affect the daily routine of the patient. The options are thus those that are usually chosen to cope with other chronic pain conditions, such as non-steroidal anti-inflammatory drugs, or narcotic pain relievers, together with a general program of pain management and psychotherapy. As steroid injections have been reported to cause and at least to worsen arachnoid inflammation, their use is generally discouraged by the medical community. Surgery is also strongly discouraged, as it can only cause further development of scar tissue and the outcomes are frequently poor and short-lasting.

    Prognosis

    Arachnoiditis remains an incurable disease that causes chronic pain and neurological deficits, and that shows little or no significant improvement with treatment. The general outlook for the patient is complicated by the absence of any specifically predictable progression pattern and the apparent lack of correlation between the beginning of the illness and the start of the symptoms (as it can take many years for the disease to be manifest). The prognosis for patients with arachnoiditis is still very poorly defined and the relevant literature is not sufficient. A book by Guyerin 1989 appears to have been the most cited study to date, in which he contends that, on average, arachnoiditis shortens life expectancy by as much as 12 years. There has been deep controversy over the question of whether or not arachnoiditis has a progressive nature. Many patients show increasing pain levels and loss of function over a period of many years, while other patients appear to remain stable after reaching a plateau. Rapid decline has only been observed in cases of injury from violent incidents, like car accidents. Indeed, the majority of patients who have suffered arachnoiditis for up to 20 years still remain in reasonably good condition, and relatively independent.

    Other complications can appear during the disease progression, like arachnoid cysts, syringomyelia, and hydrocephalus, all of which are usually coupled to depression and osteoporosis (due to the lack of mobility). Unrelieved pain and the consequent depression can in turn lead to despair and suicide, indirectly making arachnoiditis a fatal disease.

    Complications

    Meningeal Tuberculosis
    • .- Smith-Fo x Causes Arachnoiditis most commonly arises from spinal surgery (especially multiple operations), severe trauma to the spine, myelographic agents, especially the older oil-based dyes, viral and bacterial meningitis, tuberculosis, syphilis,[arachnoiditiscanada.com]
    • , tuberculosis and syphilis affect the spine.[physio-pedia.com]
    • Tuberculosis infection of the thoracic region of the spinal cord.[epainassist.com]
    Fungal Meningitis
    • Infection from bacteria or viruses: Infections such as viral and fungal meningitis or tuberculosis can affect the spine.[my.clevelandclinic.org]
    • Causes Arachnoiditis may be caused by a number of things, but some of the most common occurrences or incidents include: A direct injury to the spine A bacterial or viral infection affecting the spine, such as fungal meningitis or tuberculosis Exposure[southwestspineandpain.com]
    • Infection by bacteria or viruses: Infections such as viral and fungal meningitis or tuberculosis can affect the spine.[webmd.boots.com]
    Spina Bifida
    • Bifida and Filing for Disability Syringomelia and Filing for Disability Chiari Malformation and Filing for Disability Meningitis and Filing for Disability Laminectomy and Filing for Disability Narrowing of the Spine from Spinal Stenosis and Filing for[ssdrc.com]
    • No CSF was present at this level and there was no evidence of spina bifida.[onlinelibrary.wiley.com]
    Hashimoto's Thyroiditis
    • Patients develop a high prevalence of arthralgia, myalgia, and such autoimmune phenomenon as Hashimoto’s thyroiditis and carpal tunnel syndrome.[edsinfo.wordpress.com]
    • Their activation would produce proinflammatory or neurotoxic factors such as cytokines (interleukins 6, 8) and substance P that lead to various clinical manifestations and even clinical syndromes such as Sjogren's syndrome, Hashimoto's thyroiditis, and[surgicalneurologyint.com]
    Cystitis
    • Fibromyalgia is also commonly associated with a number of other symptoms such as disturbance in memory, concentration, mood and sleep, fatigue, depression, cystitis-like symptoms, irritable bowel syndrome, dyspareunia, morning stiffness, dry mouth and[surgicalneurologyint.com]
    Syringomyelia
    • Other complications can appear during the disease progression, like arachnoid cysts , syringomyelia , and hydrocephalus , all of which are usually coupled to depression and osteoporosis (due to the lack of mobility).[symptoma.com]
    • In some individuals, CSF flow is impaired, and they may develop syringomyelia which should, therefore, be sought in the cord.[radiopaedia.org]
    • American Syringomyelia Alliance Project, Inc.[encyclopedia.com]
    • It may also lead to other spinal cord conditions, such as syringomyelia .[wikidoc.org]

    Etiology

    Initially, arachnoiditis was recognized in cases of post-spinal surgery complications, and it was long assumed to be a surgery-induced problem. Broadly speaking, there are three types of arachnoiditis, which depend on the cause of the inflammation. The first type includes all cases of arachnoiditis due to mechanical stress, such as trauma [2] [3] [4] and the already mentioned surgical difficulties [5] [6] [7]. The second type includes the cases of irritation by foreign substances, particularly like old radiographic contrast agents that were widely used for myelography in Europe and North America up to the 1980s [8]. The third and last type of arachnoiditis includes all of the cases of arachnoiditis that are triggered by infectious diseases of the spinal cord, such as pyogenic infection [9] [10], tuberculosis [11] and leutic meningitis [3].

    Causes

    Parasite
    • Possible Etiology of Arachnoiditis Recognizing that arachnoiditis of the lumbosacral spine can also occasionally be caused by infections like meningitis, viruses like HIV, and parasites like echinococcus, readers are referred to a review on that subject[practicalpainmanagement.com]
    • Infectious includes bacterial, viral, fungal, and parasitic agents.[neuroradiologycases.com]
    • Causes of arachnoiditis include infections (bacterial, viral, fungal, and parasitic agents), irritation from chemicals, direct injury to the spine, chronic compression of spinal nerves, or complications of spinal surgery or other invasive spinal procedures[myvmc.com]

    Epidemiology

    Arachnoiditis is considered a rare disease by the National Institutes of Health. It may develop in patients who have undergone spinal surgery and intrathecal injections of toxic fluids, or who have suffered head and/or spine injuries. Arachnoiditis tends to be more frequent in females than males, which appears to be due to the spinal or epidural anesthesia that pregnant women receive during delivery. Many cases of arachnoiditis go misdiagnosed or undiagnosed for different reasons, which makes it extremely difficult to determine its precise prevalence and incidence. Burton attempted to estimate these figures for lumbo-sacral arachnoiditis in an online article in 1997 [12], where he indicated that there have been at least one million cases over the last few decades in the USA alone. If this estimate can be proven correct, the people suffering worldwide from some type of arachnoiditis must be of the order of at least tens of millions.

    Sex distribution
    Age distribution

    Pathophysiology

    Arachnoiditis shows an initial inflammation phase that lasts around two months. This phase is usually characterized by the appearance of scarring, fibrosis and adhesion, together with nerve swelling and vasodilatation, as happens in other organs and tissues. If this is not treated, these changes can become permanent [13], and the inflammation can continue into the chronic proliferative phase. The appearance of profound structural alterations to the nerve roots in the anterior half of the dural sac can be typical of the first phase, whereby these nerve roots appear “enhanced” during the inflammation; these can then become “clumped” during the later chronic proliferative stage [14] [15]. The condition can further degenerate with the formation of permanent scar tissue along with the adhesions that cause the nerves to stick together in intricate patterns. This pathological condition is known as chronic adhesive arachnoiditis, and it represents the end of the inflammatory stage, and is particularly painful for patients. If the inflammation is serious enough, arachnoiditis can begin to interfere with the functioning of the surrounding nerves, which can cause complications for the lower body parts, such as loss of full limb control.

    Prevention

    The prevention of arachnoiditis mainly consists in preventing post-surgical complications. Many guidelines have been published in this regard which underline a series of measures and surgical principles such as scar prevention or the necessity of gently handling tissues while performing surgical procedures. A good physician should know these measures and keep updated with the new versions of specific guidelines, to minimize the risk of possible port-operative complications.

    Summary

    Arachnoiditis is defined as chronic inflammation of the arachnoid, one of the three meninges. The arachnoid has the appearance of a spider-web-like layer, and it lies between the other two meningeal membranes, the dura mater (the outermost membrane) and the pia mater (the deepest membrane). Together with the classical role of protection, the arachnoid has the additional function of containing the cerebrospinal fluid, and it acts as an elastic absorber and a physiological filter for the cerebrospinal fluid as it passes from the subarachnoid space into the venous sinuses. Depending on the situation, arachnoiditis can have severe consequences, which include movement and other neurological disorders. Although arachnoiditis is now regarded as a rare disease, back in 1978 Charles V. Burton [1] defined it as common when he noted that it is often found in cases of functional impairment due to failed back surgery syndrome. Among these patients, arachnoiditis now represents the third most common pathological condition, after stenosis and recurrent disc complications.

    Patient Information

    Arachnoiditis is inflammation of the arachnoid, which is the central membrane of the three meninges that surround and protect the central nervous system (i.e., the brain and spinal cord). The causes of arachnoiditis are the same mechanical, chemical and biological stimuli that usually trigger inflammation, which here include trauma and surgically related complications, chemical irritants, and pathogens that attack the spinal cord. The symptoms of arachnoiditis mostly involve the nerves between the lower back and the limbs, and they are normally manifest as intense chronic pain, numbness, muscle complications, and neurological disorders that affect the bladder, bowel, and sexual functions. There is no cure for arachnoiditis, and treatment of the patients with arachnoiditis is limited to the relief and improvement of their symptoms through conveniently organized pain-management programs.

    Other symptoms

    Arachnoiditis
    • External links [ edit ] Arachnoiditis Information Page at NINDS Arachnoiditis Arachnoiditis; Familial spinal arachnoiditis (subtype); Spinal tuberculous arachnoiditis (subtype) at NIH 's Office of Rare Diseases Online Mendelian Inheritance in Man (OMIM[en.wikipedia.org]
    • Arachnoiditis: Introduction Arachnoiditis: Arachnoiditis is an inflammatory response of the arachnoid, one of three coverings, or meninges, that envelop the brain and spinal ... more about Arachnoiditis .[rightdiagnosis.com]
    • Arachnoiditis is a condition when the arachnoid membrane inflames.[epainassist.com]
    • Arachnoiditis is inflammation of the arachnoid mater.[spinecenteratlanta.com]
    Herniated Disc
    • In many cases, other spinal disorders such as degenerative disc disease, herniated disc, and nerve root compression may influence the development of arachnoiditis.[disabilitycarecenter.org]
    • Arachnoiditis can occur on its own or in conjunction with another back condition such as degenerative disc disease, nerve root compression from arthritis or herniated disc , failed back syndrome (also known as failed back surgery syndrome), fracture of[disabilitysecrets.com]
    • Common pathologic conditions of the spine, including herniated discs, spinal stenosis, and degenerative arthritis, may cause enough irritation to produce neuroinflammation in cauda equina nerve roots in the lumbar region.[practicalpainmanagement.com]
    • One of the investigations of back pain which was commonly used until MRI scans became available, was a myelogram, where contrast dye was injected into the spinal fluid to help image any compromise to the spinal cord (say from a herniated disc).Unfortunately[caudaequina.org]
    Calcifications
    • Rarely ossification / dystrophic calcification occurs and this is known as arachnoiditis ossificans .[radiopaedia.org]
    • These nerves are being progressively strangled by the progression of scar calcification.[burtonreport.com]
    • A CT scan (not shown) demonstrated high density within the mass compatible with calcification/ossification.[radsource.us]
    • This appears to be due to progressive calcification of the scar tissues as illustrated in the cases below.[burtonreport.com]
    Triamcinolone
    • Corticosteroids used include: hydrocortisone acetate, methylprednisolone acetate (MPA), methylprednisolone succinate, and triamcinolone.[academic.oup.com]
    Poor Outcome
    • Surgical intervention generally has a poor outcome and only provides temporary relief.[wikidoc.org]
    • Surgical intervention generally has a poor outcome, and only provides temporary relief.[disabled-world.com]
    Very Rare

    Self-assessment

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    References

    1. Burton CV. Lumbosacral arachnoiditis. Spine (Phila Pa 1976).1978 Mar; 3(1):24-30.
    2. Holmes G. The Goulstonian lectures on Spinal Injuries of Warfare. Br Med J 1915;2:769-74.
    3. Schiep G. Syringomyelia and syringobulbia. In: Vinken G, Bruyn G, eds. Handbook of clinical neurology Vol 32, Congenital malformations of the spinal cord. Amsterdam: North Holland Publishing Co, Amsterdam, 1978:255-327.
    4. Barnett H, Botterell E, Jousse A, Wynn-Jones M. Progressive myelopathy as a sequel to traumatic paraplegia. Brain 1966;89:159-73.
    5. Guyer DW, Wiltse LL, Eskay ML, Guyer BH.The long-range prognosis of arachnoiditis.Spine.1989;14:1332-41.
    6. Heary RF, Northrup BE, Barolat G. Arachnoiditis. In: Benzel EC (ed) Spine surgery: techniques, complication, avoidance and management. Churchchill Livingstone, Philadelphia, pp 2004–2012., 2005
    7. Wright MH, Denney LC. A comprehensive review of spinal arachnoiditis. Orthop Nurs 2003;22:215-9. 
    8. Laitt R, Jackson A, Isherwood I. Patterns of chronic adhesive arachnoiditis following myodil myelography: the significance of spinal canal stenosis and previous surgery. Br J Radiol, 1996;69:693-8.
    9. Barnett H. Syringomyelia associated with spinal arachnoiditis. In: Barnett H, Foster J, Hudgson P. Syringomyelia. London: Saunders, 1973:220-44.
    10. Jenik F, Tekle-Haimanot R, Hamory B. Non-traumatic adhesive arachnoiditis as a cause ofspinal cord syndromes. Investigations of 507 patients. Paraplegia 1981;19:140-54.
    11. Suzuki M, Davis C, Symon L, Gentili F. Syringoperitoneal shunt for treatment of cord cavitation. J Neurol Neurosurg Psychiatry. 1995;48:620–7.
    12. Burton CV, Internet article “Adhesive arachnoiditis: The Global Economic Liability” 1997.
    13. Aldrete JA. Anatomopathology. In: Aldrete JA, ed.Arachnoiditis: the Silent Epidemic. Denver: Futuremed, 2000; 7-18.
    14. Aldrete JA, Ghaly RF. Postlaminectomy pseudomeningocele: an unexpected cause of low back pain. Reg Anesth 1995; 20:75–9.
    15. Aldrete JA, Brown TL. Laboratory and radiological diagnosis. In: Aldrete JA, ed. Arachnoiditis: the Silent Epidemic. Denver:Futuremed,2000; 221-52.



    Media References

    1. ARACHNOIDITIS, Public Domain

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    Symptoma
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