Orphanet: Arachnoiditis

Orphanet: Arachnoiditis

Arachnoiditis

Orpha number ORPHA137817 Synonym(s) Adhesive arachnoiditis Chronic arachnoiditis Prevalence Unknown Inheritance Sporadic Age of onset Variable

ICD-10 - OMIM 182950 UMLS C0003708 MeSH D001100 MedDRA 10003074 SNOMED CT 8217007

SUMMARY

Arachnoiditis (ARC) is a chronic inflammation of the arachnoid layer of the meninges, of which adhesive arachnoiditis is the most severe form, characterized by debilitating, intractable neurogenic back and limb pain and a range of other neurological problems.

The prevalence is unknown. About 25,000 cases of arachnoiditis occur each year, mostly in North and South America, Asia and Europe, where spinal operations are more prevalent.

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 hyperpathia. Patients may also experience proprioception alterations (including loss of balance, tinnitus and reduced hearing and vision), motor weakness, muscle cramps, fasciculation, anhydrosis, and bladder, bowel and sexual dysfunction. Arachnoiditis may, in a minority of cases, involve the brain as well as the spinal cord, possibly causing communicating hydrocephalus.

Arachnoiditis can be mechanically (localized) or chemically (diffuse) induced, and is often associated with spinal operations (60% of cases), neuroaxial (spinal and epidural) anesthesia (22%), spinal taps (7%), myelography (3%), pain relief procedures and secondary infections. It can also be caused by bacterial and viral spinal infections (7%) and repeated subarachnoid injections of anticancer drugs or antimetabolites. Syringomyelia, cauda equina syndrome, pseudomeningoceles, intrathecal cysts or tethering of the spinal cord and nerve roots (NR) may complicate lumbosacral arachnoiditis. Arachnoiditis occurs as a progression of inflammatory changes. In the early (inflammatory) phase NR are edematous (enhanced), while in the late proliferation stage (adhesive arachnoiditis) NR are clumped and asymmetric. The flow of cerebrospinal fluid from the distal dural sac to the brain is impeded, intrathecal pressure increases and this causes back pain and postural headache. In some cases the scar tissue calcifies (arachnoiditis ossificans).

Diagnosis is based on patient history, clinical presentation and a causative event, and can be confirmed by MRI with contrast. When MRI is not possible, myelogram followed by CT scan is indicated. The adhesions generally occur on the dorsal segments, are arranged peripherally, and have been described as looking `like the bark of a tree' when viewed by myelography.

Differential diagnoses include intra-spinal hematoma or dislodged disc fragment if the condition presents immediately after surgery, and Failed Back Surgery Syndrome (FBSS). Some patients are diagnosed with fibromyalgia (see this term), but these symptoms are likely to occur as a secondary feature due to the altered spinal dynamics.

In the early phase, treatment includes large doses of IV methylprednisolone for five days, preferably within three months of the causative injury, followed by a protocol directed to control neuropathic pain using a multimodal approach that includes an anti-inflammatory, an anticonvulsant and an antidepressant. Large doses of opiates, that can cause hyperalgesia, hypersensitivity and tachyphylaxis, and lead to dependence, should be discouraged. If necessary, to treat exacerbation ``flare-ups'', IV infusions of NMDA receptors antagonists (including lidocaine, MgSO4, ketamine) can be given.

Once the proliferative stage has begun, arachnoiditis will be permanent and is complicated by the aging process of the spine. Operations, injections or any other invasions of the spine may exacerbate the disease significantly.

Expert reviewer(s)

• Pr Antonio ALDRETE

Last update: March 2010

Orphanet: Perineural cyst Tarlov cyst

Orphanet: Perineural cyst Tarlov cyst

Perineural cyst

Orpha number ORPHA65250 Synonym(s) Tarlov cyst Prevalence Unknown Inheritance Sporadic Age of onset Adulthood

ICD-10 G54.8 OMIM - UMLS - MeSH - MedDRA - SNOMED CT -

SUMMARY

Perineural (or Tarlov) cysts are cerebrospinal fluid-filled nerve root cysts most commonly found at the sacral level of the spine, although they can be found in any section of the spine, which can cause progressively painful radiculopathy.

The annual incidence of perineural cysts is estimated at approximately 5%, although large cysts that cause symptoms are relatively rare with annual incidence estimated at less than 1/2,000. Women are affected more frequently than men.

Patients with perineural cysts present with pain in the area of the nerves affected by the cyst, muscle weakness, difficulty sitting for prolonged periods, loss of sensation, loss of reflexes, pain when sneezing or coughing, swelling over the sacral area, parasthesias, headaches, sciatica, and bowel, bladder and sexual dysfunction. The cysts typically occur along the posterior nerve roots and can be valved or nonvalved. The main feature that distinguishes perineural cysts from other spinal lesions is the presence of spinal nerve root fibres within the cyst wall or in the cyst cavity.

There are a number of conditions that can cause the cysts to become symptomatic, including traumatic injury, heavy lifting, childbirth, epidurals, and trauma to the spinal cord. It has also been observed that the herpes simplex virus can cause the body chemistry to change and that perineural cyst symptoms worsen during herpes virus outbreaks.

Diagnosis is based on magnetic resonance imaging (MRI), computed topography (CT) or myelogram of patients experiencing lower back pain or sciatica.

The main differential diagnoses are meningeal diverticula and long arachnoid prolongations, which can be distinguished by rapid filling on myelography compared to the delayed filling of perineural cysts. Differential diagnoses also include herniated lumbar discs, arachnoiditis and, in females, gynecological conditions.

The majority of perineural cysts are sporadic. However, in some cases cysts have been observed among relatives, suggesting the possibility of a familiar trait with autosomal transmission.

Treatment involves lumbar drainage of the cerebrospinal fluid, CT scanning-guided cyst aspiration, decompressive laminectomy, cyst and/or nerve root excision and microsurgical cyst fenestration and imbrications. However, surgical treatment for perineural cysts is complicated by postoperative pseudomeningocele and intracranial hypotension, and recurrence of the cyst. Pain therapy may offer a nonsurgical alternative for the treatment of symptomatic perineural cysts.

Those who have progressive and prolonged symptoms may experience neurological damage if the cysts continue to compress nerve structures.

Expert reviewer(s)

• Dr A CARLUCCIO
• Pr Antonio FEDERICO
• Dr D MARINO

Last update: February 2010

Sharon Dublin Ireland, Tarlov cyst Disease Patient

• tarlovcysteurope@groups.facebook.com
• https://www.facebook.com/groups/tarlovcysteurope/
• Hi Ya,
• I would just like to say, as a Tarlov cyst Patient, from Dublin Ireland, I was diagnosed in 2000, 
• I have been active on the internet since 2002 researching and educating myself about Tarlov cyst Disease, I often share articles here, on the Blog related to Tarlov cysts Arachnoiditis, Chronic Pain and anything related to both, 
• I started a Facebook group for Europeans the links are above,I would love for anyone from Europe to join share any information they might have about Drs, Pain Specialists etc etc in your Part of the woods or just to get to know each other. Like you who are in the same situation, 
• I also am involved with a Yahoo support Group for many Years, tarlovcystsurvival@yahoogroups.com Its a wonderful Worldwide Support Group, 
• 
  
• You are Not alone with this Rare Disease,
• Best Wishes
• Sharon xx soft hugs xx
• Dublin Ireland
• You can contact me anytime s_gillece@hotmail.com
• 00353 1 8338196 home
• 00353 86 8556557 mobile,

Critics Oppose FDA Approval of Painkiller Zohydro

http://www.webmd.com/pain-management/news/20140226/new-painkiller-zohydro-criticized

Feb. 27, 2014 -- A new narcotic painkiller is due to come on the market in March, and critics want the FDA to reverse its approval of the drug, Zohydro ER. They claim it could become the nextOxyContin, another opioid that’s become a popular drug of abuse.

Critics of the FDA's ruling include attorneys general from 28 states and FED UP!, a union of consumer groups, addiction treatment providers, drug and alcohol prevention programs, and other interested groups. They have petitioned FDA Commissioner Margaret Hamburg, MD, to prevent Zohydro from coming on the market. The FDA approved the drug even though its advisory committee voted 12 to 2 against approval.

"I firmly believe that the benefits of this product outweigh its risks," the FDA’s Bob Rappaport, MD, wrote in the summary review explaining why he approved Zohydro last October. Rappaport is director of the FDA's Division of Anesthesia, Analgesia, and Addiction Products. "Many patients in the U.S. suffer from untreated or poorly treated chronic pain. Further limiting access to potential treatments is not the answer when new treatments are critically needed.”

Opioids are a man-made type of narcotic pain medication. They subdue the central nervous system, ease pain, and induce sleep. They can have serious side effects if not used properly.

Here are some commonly asked questions about Zohydro, made by Zogenix, a San Diego company.

Q. Why do people want the FDA to reverse its approval of Zohydro?

A. Zohydro is too easy to abuse, critics say. It is the only approved extended-release product that contains the man-made opioid hydrocodone. It will be available in doses as high as 50 milligrams per pill -- five times the amount in immediate-release hydrocodone pills. Even though it is meant to release hydrocodone slowly over 12 hours, the pill could be tampered with to release a large dose all at once, says Andrew Kolodny, MD, co-founder of the group Physicians for Responsible Opioid Prescribing.

Two members of the FDA’s controlled substance staff warned about the potential for abuse in materials presented to the advisory committee. “If approved and marketed, Zohydro ER will be abused, possibly at a rate greater than that of currently available hydrocodone combination products,” wrote medical officer Lori Love, MD, PhD, and pharmacologist James Tolliver, PhD.

Zogenix is “currently evaluating two different technologies to ensure we develop the most effective formulation to minimize misuse and abuse,” says company spokeswoman Julie Normart. The company is also taking other actions to lower the risk of abuse, according to a statement supplied by Normart. These include locking pill bottle caps and an external “safe-use board” made up of experts in abuse, misuse, and diversion.

Jason Jerry, MD, an addiction specialist at the Cleveland Clinic, questions why Zogenix doesn’t wait to introduce Zohydro until they develop a version of it that's harder to abuse. “I’m not sure why there’s this great rush to get this to market,” Jerry says.

FDA approves extended-release, single-entity hydrocodone product

http://www.fda.gov/newsevents/newsroom/pressannouncements/ucm372287.htm

FDA NEWS RELEASE

For Immediate Release: Oct. 25, 2013
Media Inquiries: Morgan Liscinsky, 301-796-0397; morgan.liscinsky@fda.hhs.gov 
Consumer Inquiries: 888-INFO-FDA
FDA approves extended-release, single-entity hydrocodone product
First to have updated labeling now required for all ER/LA opioid analgesics

The U.S. Food and Drug Administration today approved Zohydro ER (hydrocodone bitartrate extended-release capsules) for the management of pain severe enough to require daily, around-the-clock, long-term treatment and for which alternative treatment options are inadequate.

Zohydro ER, a Schedule II controlled substance under the Controlled Substances Act, is the first FDA-approved single-entity (not combined with an analgesic such as acetaminophen) and extended-release hydrocodone product.

Zohydro ER will offer prescribers an additional therapeutic option to treat pain, which is important because individual patients may respond differently to different opioids.

Zohydro ER is in the class of extended-release/long-acting (ER/LA) opioid analgesics. Due to the risks of addiction, abuse, and misuse with opioids, even at recommended doses, and because of the greater risks of overdose and death with ER/LA opioid formulations, Zohydro ER should be reserved for use in patients for whom alternative treatment options are ineffective, not tolerated, or would be otherwise inadequate to provide sufficient management of pain. Zohydro ER is not approved for as-needed pain relief.

The approved labeling for Zohydro ER conforms to updated labeling requirements for all ER/LA opioid analgesics announced by the FDA on Sept. 10, 2013.

The new class labeling and stronger warnings will more clearly describe the risks and safety concerns associated with ER/LA opioid analgesics, along with the appropriate use of these medications. These warnings are expected to improve the safety of all such medicines by encouraging more appropriate prescribing, patient monitoring, and patient counseling practices. Zohydro ER is the first opioid to be labeled in this manner.

Schedule II drugs can only be dispensed through a physician’s written prescription and no refills are allowed. There are also stringent recordkeeping, reporting, and physical security requirements for Schedule II controlled substances. 

The FDA is requiring postmarketing studies of Zohydro ER to assess the known serious risks of misuse, abuse, increased sensitivity to pain (hyperalgesia), addiction, overdose, and death associated with long term use beyond 12 weeks. These studies will also be required for other ER/LA opioid analgesics.

The safety of Zohydro ER is based on clinical studies of more than 1,100 people living with chronic pain. The efficacy of Zohydro ER is based on a clinical study that enrolled over 500 patients with chronic low back pain and showed significant improvement in chronic pain compared to placebo.

Zohydro ER will be part of the ER/LA Opioid Analgesics Risk Evaluation and Mitigation Strategy (REMS). Originally approved in 2012, the ER/LA Opioid Analgesics REMS requires companies to make available to health care professionals educational programs on how to safely prescribe ER/LA opioid analgesics and to provide Medication Guides and patient counseling documents containing information on the safe use, storage, and disposal of ER/ LA opioids.

The most common side effects of Zohydro ER are constipation, nausea, drowsiness (somnolence), fatigue, headache, dizziness, dry mouth, vomiting and itching (pruritus).

Zohydro ER is manufactured by San Diego-based Zogenix, Inc.

For more information:

FDA Approved Drugs: Questions and Answers
FDA: Opioid Medications
The FDA, an agency within the U.S. Department of Health and Human Services, protects the public health by assuring the safety, effectiveness, and security of human and veterinary drugs, vaccines and other biological products for human use, and medical devices. The agency also is responsible for the safety and security of our nation’s food supply, cosmetics, dietary supplements, products that give off electronic radiation, and for regulating tobacco products.