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.
Entire Body System
- Weakness
Keywords: Bilateral lower limb weakness, chronic adhesive arachnoiditis, diastematomyelia, MRI, tubercular radiculomyelitis How to cite this article: Chandak S, Agrawal A, Kumar A. [saudijhealthsci.org]
The patient could walk without a cane 3 years after the shunt operation, although numbness and motor weakness of the lower extremities remained. [ncbi.nlm.nih.gov]
A 42-year-old man presented with gradually worsening bilateral leg pain and weakness over the last 12 months. He was treated previously for L3 and L4 vertebral fractures with internal fixation. View Case [ajnr.org]
- Epilepsy
Brain Boost Sound Advice The link between hearing loss and cognitive decline PICTURES OF YOU The Bright Side After epilepsy derailed her dream of being a dancer, Susan Cruz hopes to teach dance to children. [patients.aan.com]
Restless legs - Stiff person Other degenerative / demyelinating diseases Alzheimer's - Pick's - Alpers' - Dementia with Lewy bodies - Leigh's demyelinating: Multiple sclerosis - Devic's - Central pontine myelinolysis - Transverse myelitis Seizure / epilepsy [wikidoc.org]
BOARD乳癌 CARDIAC PRACTICE Cardio-Coagulation Cardio-Lipidology Cardio-Renal Diabetes Clinical Respiration Coagulation & Inflammation DEPRESSION JOURNAL Diabetes Frontier Diabetes Frontier Online Diabetes Horizons -Practice and Progress- DIABETES UPDATE Epilepsy [med.m-review.co.jp]
- Hypersomnia
Cluster - Vascular - Tension Vascular Transient ischemic attack ( Amaurosis fugax, Transient global amnesia ) Cerebrovascular disease ( MCA, ACA, PCA, Foville's, Millard-Gubler, Lateral medullary, Weber's, Lacunar stroke ) Sleep disorders Insomnia - Hypersomnia [wikidoc.org]
Gastrointestinal
- Nausea
Narcotic Pain Relievers Dizziness Constipation Nausea and vomiting Sedation NSAIDS Stomach upset Stomach ulcers. [marijuanadoctors.com]
[…] arachnoiditis include: numbness, tingling, or a pins-and-needles feeling crawling sensation on the skin, as if ants are walking up and down your back muscle cramps or spasms weakness trouble walking severe headaches vision problems hearing problems dizziness nausea [healthline.com]
[…] cauda equina’s nerve root and the sympathetic nerve fibers connected to them., 2 In addition to pain sensory alteration and motor deficits, it may also be accompanied by other symptoms such as low-grade fever, profuse diaphoresis, heat intolerance, nausea [practicalpainmanagement.com]
- Vomiting
We report a boy aged 17, presenting with hearing impairment and recurrent vomiting for 18 months, weight loss for 12 months, dysphagia, dysarthria, hypophonia for 6 months, and gait unsteadiness for 5 months. [ncbi.nlm.nih.gov]
[…] insects crawling on the skin or water trickling down the leg, Severe shooting pain (which some liken to an electric shock sensation), Positional, often severe, headaches as a result of obstruction to the flow & rotation of cerebrospinal fluid (CSF), vomiting [artforarachnoiditis.org]
定義される好中球減少がない場合)[全身症状CONSTITUTIONAL-Fever]; Grade 3-4 の好中球減少を伴う感染(臨床的または微生物学的に確認)-選択[感染INFECTIONInfection]; 好中球数が正常またはGrade 1-2 の好中球減少を伴う感染-選択[感染INFECTION- Infection]; 好中球数が不明な感染-選択[感染INFECTION- Infection]; 疼痛-選択[疼痛PAIN-Pain]; 嘔吐[消化管GASTROINTESTINAL- Vomiting [osaka-med.ac.jp]
Skin
- Sweating
Patients with this disease have severe low back and leg pain, sweating and low grade fever. This case had aberrant skin temperature and sweating in different parts of the body. [ncbi.nlm.nih.gov]
[…] to those felt in the lips/face after dental anesthetic), tingling and numbness, loss of sense of limb position, dizziness, loss of balance, Tingling, numbness, or weakness in the legs or arms, sometimes accompanied by restrictive neck pain, Profuse sweating [artforarachnoiditis.org]
If i get too hot i overheat and sweat uncontrollably. [painsupport.co.uk]
[…] 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 ) inability to sweat [healthline.com]
Musculoskeletal
- Back Pain
L3-S1 L5 Low back pain; weakness Conservative Worsen 2 41/F 20 Myelography; diskectomy; laminectomy; fusion 4 L5-S1 L3–L5 Low back pain; sciatica Conservative Unchanged 3 50/M 11.5 Myelography; laminectomy; fusion 1 L4–L5 L4–L5 Back pain; leg pain Conservative [doi.org]
Typical symptoms include back pain, urinary urgency and/or incontinence, sensory loss and weakness in the lower extremities. [n.neurology.org]
Lower back pain is a very common cause for visit to a physician that affects approximately 80% of the population at some point during their lives. The majority of lower back pain results from injuries; but it can also result from other diseases. [omicsonline.org]
The clinical presentation featured leg pain (90%), low-back pain (80%), and sphincter disturbance (25%). [ncbi.nlm.nih.gov]
- Muscle Cramp
Some people with arachnoiditis will have debilitating muscle cramps, twitches, or spasms. It may also affect bladder, bowel, and sexual function. [ninds.nih.gov]
This syndrome was characterized by transient violent spasms in the legs, muscle cramps, increased radicular pain, and often fever and chills. The recognition of this syndrome and a proposed method of treatment is discussed. [ncbi.nlm.nih.gov]
cramps or spasms, and unusual sensations such as the feeling of water trickling down the leg or insects crawling on the skin. [thepaincenter.com]
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]
[…] headaches, vision changes, incontinence Generalized weakness, leans toward L; +Babinski, brisk reflexes. 258 ml 112 18 233 _ 342 1.33 1:128 1:256 154 LAmB + Alive; residual wide-based gait 3 53 M FL Seizure disorder neoformans Dizziness, hearing loss, tinnitus [academic.oup.com]
- Hearing Impairment
We report a boy aged 17, presenting with hearing impairment and recurrent vomiting for 18 months, weight loss for 12 months, dysphagia, dysarthria, hypophonia for 6 months, and gait unsteadiness for 5 months. [ncbi.nlm.nih.gov]
Eyes
- Visual Impairment
Table 1: Severity of visual impairment in patients with symptomatic optochiasmatic arachnoiditis Click here to view Nineteen of 23 patients (82%) were blind according to the standard WHO definition for visual impairment. [neurologyindia.com]
Victims of the dye's effects can suffer burning back pain, shooting pains in limbs, deep muscle pains in back and limbs, spasms and twitches, burning feet, Joint pains, numbness and tingling sensations, seizures and paralysis, visual impairment and feelings [whale.to]
Neurologic
- Headache
This serious but rare complication should encourage caution when treating parturients with postdural puncture headache with a repeat epidural blood patch. [ncbi.nlm.nih.gov]
Case Example A 23-year-old Hispanic male ( Table 1, case 2 ) developed night sweats, weakness, headache, and neck stiffness over 3 months. [academic.oup.com]
The symptoms of arachnoiditis resemble those associated with other spinal conditions including back pain, muscle spasms, referred pain, headache, dizziness and alterations of balance. [rarediseases.org]
- Sciatica
The common clinical presentation consisted of low-back pain and sciatica, and radicular pain was usually a continuation or a recurrence of the preoperative sciatic pain. [doi.org]
They speculated that their patient's “lumbago and sciatica” were produced by “inflammation.” [jnnp.bmj.com]
- Dysesthesia
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]
[…] 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]
- 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. Patients may not be able to do straight leg raises or flex one or both feet. Range of motion of both upper and lower extremities may be restricted. [edsinfo.wordpress.com]
- Hypesthesia
On admission to the authors' institution, physical examination demonstrated no abnormal findings except for perianal hypesthesia. An MRI study obtained at admission demonstrated a cystic lesion in the peridural space from the level of S-2 to S-4. [ncbi.nlm.nih.gov]
Neurological examination during the first visit to our clinic revealed hypesthesia of the lower right extremity below L-4 and of the lower left extremity below S-1, reinforcement of the bilateral patellar and Achilles tendon reflexes, bilateral ankle [doi.org]
Urogenital
- Sexual Dysfunction
Other symptoms include lower limbs weakness and bladder, bowel, and sexual dysfunction Causes: Infection, intrathecal steroids or anaesthetics, intrathecal haemorrhage, trauma or prior surgery. Arachnoiditis complicates 6-16% of spinal surgeries. [ajnr.org]
dysfunction If the disease progresses, symptoms may become more severe or even permanent. [spineuniverse.com]
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]
dysfunction Symptoms may become more severe or even permanent if the disease progresses. [my.clevelandclinic.org]
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.
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].
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.
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.
References
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- Aldrete JA. Anatomopathology. In: Aldrete JA, ed.Arachnoiditis: the Silent Epidemic. Denver: Futuremed, 2000; 7-18.
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