Syringomyelia

Syringomyelia is a generic term referring to a disorder in which a cyst or cavity forms within the spinal cord. This cyst, called a syrinx, can expand and elongate over time, destroying the spinal cord.  The damage may result in pain, paralysis, weakness,  and stiffness in the back, shoulders, and extremities.  Syringomyelia may also cause a loss of the ability to feel extremes of hot or cold, especially in the hands.  The disorder generally leads to a cape-like loss of pain and temperature sensation along the back and arms.  Each patient experiences a different combination of symptoms. These symptoms typically vary depending on the extent and, often more critically, to the location of the syrinx within the spinal cord.

Syringomyelia has a prevalence estimated at 8.4 cases per 100,000 people,  with symptoms usually beginning in young adulthood.  Signs of the disorder  tend to develop slowly, although sudden onset may occur with coughing, straining.

What causes Syringomyelia?

Trauma to the spinal cord or congenital developmental problems of the brain and/or spinal cord may result in SM.
Spinal cord trauma such as a car accident or serious fall may manifest years later as SM.     

Congenital developmental problems, sometimes undetectable may result in syringomyelia.

In either case, the condition may lie dormant and undetected for months or years until a symptom or variety of symptoms become bothersome enough to warrant medical attention.  Many people with SM are not diagnosed until mid-life.

A number of medical conditions can cause an obstruction in the normal flow of cerebrospinal fluid (CSF), redirecting it to the spinal cord itself.  This results in the formation of a syrinx (cyst that fills with CSF). Pressure differences along the spine cause the fluid to move within the cyst.  It is believed that this continual movement of fluid results in cyst growth and further damage to the spinal cord and connecting nerves.

Different Origins

Generally, there are two forms of syringomyelia:  congenital and acquired. (In addition, one form of the disorder involves a part of the brain called the brainstem.  The brainstem controls many of our vital functions, such as respiration and heartbeat.  When syrinxes affect the brainstem, the condition is called syringobulbia.)

The first major form relates to an abnormality of the brain called an Chiari malformation, named after the physician who first characterized it.  This is the most common cause of syringomyelia, where the anatomic abnormality causes the lower part of the cerebellum to protrude from its normal location in the back of the head into the cervical or neck portion of the spinal canal.  A syrinx may then develop in the cervical region of the spinal cord.  Because of the relationship that was once thought to exist between the brain and spinal cord in this type of syringomyelia, physicians sometimes refer to it as communicating syringomyelia.  Here, symptoms usually begin between the ages of 25 and 40 and may worsen with straining or any activity that causes cerebrospinal fluid pressure to fluctuate suddenly.  Some patients, however, may have long periods of stability.  Some patients with this form of the disorder also have hydrocephalus, in which cerebrospinal fluid accumulates in the skull, or a condition called arachnoiditis, in which a covering of the spinal cord—the arachnoid membrane—is inflamed.

Some cases of syringomyelia are familial, although this is rare.

Acquired

The second major form of syringomyelia occurs as a complication of trauma, meningitis, hemorrhage, a tumor, or arachnoiditis.  Here, the syrinx or cyst develops in a segment of the spinal cord damaged by one of these conditions.  The syrinx then starts to expand.  This is sometimes referred to as noncommunicating syringomyelia.  Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma.

The primary symptom of post-traumatic syringomyelia (often referred to using the abbreviation of PTS) is pain,  which may spread upward from the site of injury.  Symptoms, such as pain, numbness, weakness, and disruption in temperature sensation, may be limited to one side of the body.  Syringomyelia can also adversely affect sweating, sexual function, and, later, bladder and bowel control.  A typical cause of PTS would be a car accident or similar trauma involving a whip-lash injury.

What can make PTS difficult to diagnose is the fact that symptoms can often first appear long after the actual cause of the syrinx occurred, e.g. a car accident occurring and then the patient first experiencing PTS symptoms such as pain, loss of sensation, reduced ability on the skin to feel varying degrees of hot and cold, a number of months after car accident.

Signs and tests

A neurologic examination may show loss of sensation or movement caused by compression of the spinal cord.

An MRI of the spine confirms syringomyelia and determines the exact location and extent.  Often, an MRI of the head will be done to look for associated conditions including hydrocephalus (water on the brain).

Rarely, a spinal CT with myelogram may be done.

Symptoms

Syringomyelia causes a wide variety of neuropathic symptoms due to damage of the spinal cord.  Patients may experience chronic pain, abnormal sensations and loss of sensation particularly in the hands.  Some patients experience paralysis or paresis temporarily or permanently.  A syrinx may also cause disruptions in the parasympathetic and sympathetic nervous systems, leading to abnormal body temperature or sweating, bowel control issues, or other problems.  If the syrinx is higher up in the spinal cord or affecting the brainstem as in syringobulbia, vocal cord paralysis, ipsilateral tongue wasting, trigeminal nerve sensory loss, and other signs may occur.  Rarely, bladder stones can occur in the onset of weakness in the lower extremities.  Classically, syringomyelia spares the dorsal column/medial lemniscus of the spinal cord, leaving pressure, vibration, touch andproprioception intact in the upper extremities.  Neuropathic arthropathy, also known as a Charcot joint, can occur, particularly in the shoulders, in patients with syringomyelia.  The loss of sensory fibers to the joint is theorized to lead to damage of the joint over time.

Back pain;  headaches;  stiffness, weakness or pain in the back, shoulders, arms or legs;  loss of the ability to feel extremes of hot or cold, especially in the hands?

Treatment

The goals of treatment are to stop the spinal cord damage from getting worse and to maximize functioning. Surgery to relieve pressure in the spinal cord may be appropriate. Physical therapy may be needed to maximize muscular function.

It may be necessary to drain the fluid build up.
Surgery
The first step after diagnosis is finding a neurosurgeon who is experienced in the treatment of syringomyelia. Surgery is the only viable treatment for syringomyelia. Not all patients will advance to the stage where surgery is needed. Evaluation of the condition is often difficult because syringomyelia can remain stationary for long periods of time, and in some cases progress rapidly.

Surgery of the spinal cord has certain, characteristic risks associated with it and the benefits of a surgical procedure on the spine have to be weighed against the possible complications associated with any procedure. Surgical treatment is aimed at correcting the condition that allowed the syrinx to form. It is vital to bear in mind that the drainage of a syrinx does not necessarily mean the elimination of the syrinx-related symptoms, but rather is aimed at stopping progression. In cases involving an Arnold-Chiari malformation, the main goal of surgery is to provide more space for the cerebellum at the base of the skull and upper cervical spine without entering the brain or spinal cord. This often results in flattening or disappearance of the primary syrinx or cavity, over time, as the normal flow of cerebrospinal fluid is restored. If a tumor is causing syringomyelia, removal of the tumor is the treatment of choice and almost always eliminates the syrinx.

Surgery results in stabilization or modest improvement in symptoms for most patients. Delay in treatment may result in irreversible spinal cord injury. Recurrence of syringomyelia after surgery may make additional operations necessary; these may not be completely successful over the long term.

In some patients it may also be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and valves. This system is also known as a shunt. Shunts are used in both the communicating and noncommunicating forms of the disorder. First, the surgeon must locate the syrinx. Then, the shunt is placed into it with the other end draining cerebrospinal fluid (CSF) into a cavity, usually the abdomen. This type of shunt is called a ventriculoperitoneal shunt and is particularly useful in cases involving hydrocephalus. By draining syrinx fluid, a shunt can arrest the progression of symptoms and relieve pain, headache, and tightness. Without correction, symptoms generally continue.

The decision to use a shunt requires extensive discussion between doctor and patient, as this procedure carries with it greater risk of injury to the spinal cord, infection, blockage, or hemorrhage and may not necessarily work for all patients. Draining the syrinx more quickly does not produce better outcomes, but a shunt may be required if the fluid in the syrinx is otherwise unable to drain.

In the case of trauma-related syringomyelia, the surgeon operates at the level of the initial injury. The syrinx collapses at surgery but a tube or shunt is usually necessary to prevent re-expansion.

Other

Surgery is not always recommended for syringomyelia patients. For many patients, the main treatment is analgesia. A typical treatment of syringomyelia involving severe chronic pain would involve two or more medications. One medication for "classical" back pain such as a weak or strong opioid(e.g. tramadol and Oxycontin respectively) combined with a medication to combat any neuropathic pain symptoms such as shooting and stabbing pains (e.g. Neurontin or Lyrica). In addition, paracetamol (called acetaminophen in the United States) can be used to combat headaches. Such long term treatment of chronic pain should be monitored with blood tests to assess any adverse effects of the medication on the liver, with the dosages being then changed accordingly, depending on the outcome of such blood tests.

Drugs have no curative value as a treatment for syringomyelia. Radiation is used rarely and is of little benefit except in the presence of a tumor. In these cases, it can halt the extension of a cavity and may help to alleviate pain.

In the absence of symptoms, syringomyelia is usually not treated. In addition, a physician may recommend not treating the condition in patients of advanced age or in cases where there is no progression of symptoms. Whether treated or not, many patients will be told to avoid activities that involve straining.

Since the natural history of syringomyelia is poorly understood, a conservative approach may be recommended.  When surgery is not yet advised, patients should be carefully monitored by a neurologist or neurosurgeon.  Periodic MRI's and physical evaluations should be scheduled at the recommendation of a qualified physician.

Prevention

There is no known prevention, other than avoiding trauma to the spinal cord.  Prompt treatment reduces progression of the disorder.

Expectations (prognosis)

Untreated, the disorder gets worse very slowly, but it eventually causes severe disability.  Surgical decompression usually stops the progression of the disorder, with about fifty percent of people showing significant improvement in neurologic function after surgical decompression.

Complications

Without treatment, the condition will lead to:

  • Continued or progressive loss of neurologic function
  • Permanent disability

Possible complications of surgery include:

  • Postoperative infection and other complications common to all surgeries

Testimonials:

Pain - Marijuana acts as an  analgesic or natural pain killer in the bodies basic operational system.  There is a role for the cannaboids  found in marijuana for the body ‘s pain and movement receptors.  A number of areas in the brain that produce sensing and processing receptors respond to the analgesic effects of cannabis.

Cannaboids  are often successful in treating pain and used as adjunct treatments to opiads.. Acting as pain killers  and anti-inflammatories,  medical marijuana is far less addictive than many other pain killing medications.

  Sleep Aid - A common side effect of marijuana is drowsiness.  It is thought to be used for patients who have difficulty sleeping due to their condition or treatment method.  Patients experience a more restful sleep and wake up feeling more refreshed.

Medical marijuana is not toxic to major organs like many other sleep aids and has very few side effects.  Marijuana causes the body to drop temperature by half of a degree.  This allows for a human to become more relaxed and stress free allowing you to fall asleep faster.

Smoking cannabis from a pipe can significantly reduce chronic pain in patients with damaged nerves,  a study suggests.

“ Hi i am 22 yrs old and was diagnosed with SM due to chiari about 1 year ago.  After a failed decompression surgery my only option would be to take out a piece of the cerebellum.  I consume alot of marijuana everyday, and know of its effects on the brain.  Mariuana slowed down the flow of CSF into the spine.  Slowed  down the effects  of the syringomyelia.   about 3 times.  Also smoking takes away every single symptom that i have:  stomach ache, cephalea in my temples, tingling sensations in my hands and feet.”

Marijuana, reduced pain in patients with nerve pain stemming from injuries or surgical complications, new research shows.

Patients smoking the highest potency marijuana (9.4 percent) reported less pain than those smoking samples containing no THC.  Patients also reported better sleep and less anxiety, according to the Canadian study.

Best Strains for symptom relief of Syringomyelia:

Skunk #1, TrainWreck, Apollo 13, Afgani, NYCD, White Rhino, Purple Haze, Super Silver Haze, Sour Diesel, Big Kahuna, Blueberry, Romulan, G13, Blue Fruit, Green Queen, Queen Bee, Sweet Blu, Strawberry Cough,

Symptoms:  tingling in feet and hands, numbness, burning, muscle spasm, pain, headache, stiffness or weakness in back, shoulders, arms/legs, ability to feel  extremes of hot/cold especially in hands, abnormal sensations or loss of sensations especially in hands, paralysis (temporary/permanent), chronic pain.

References



1. Feske SK, Cochrane TI. Degenerative and compressive structural disorders. In: Goetz CG, ed. Textbook of Clinical Neurology. 3rd ed. Philadelphia, PA: Saunders Elsevier; 2007:chap 29.
2. Golden JA, Bonnemann CG. Etiological categories of neurological diseases. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, PA: Saunders Elsevier; 2007:chap 28.
1. "Neurosurgical considerations in posttraumatic syringomyelia - Home Study Program". AORN Journal. January 2003. Retrieved 2009-02-12.
2. Brewis M, Poskanzer DC, Rolland C, et al., "Neurological disease in an English city". Acta Neurologica Scand Suppl 24:1--89, 1966.
3. Schurch B, Wichmann W, Rossier AB (January 1996). "Post-traumatic syringomyelia (cystic myelopathy): a prospective study of 449 patients with spinal cord injury". J. Neurol. Neurosurg. Psychiatr. 60 (1): 61–7. doi:10.1136/jnnp.60.1.61. PMC 486191. PMID 8558154.
4. Greenberg, David A, et al.: Clinical Neurology. 5th ed. Feb 9, 2002.
5. Nishida, Takayasu, et al. "A large bladder stone caused by syringomyelia". Japanese Journal of Clinical Urology, Vol.60, No. 6, pp 413-415, 2006. ISSN:0385-2393.
6. "Neuropathic Arthropathy of the Shoulder (Charcot Shoulder): Clinical Commentary" Medscape. Accessed 9 January 2011.
7. "Information about a Genetic Research Study for Chiari Type I Malformation (CMI) with or without Syringomyelia."
8. Yeom JS, Lee CK, Park KW, et al. (2007). "Scoliosis associated with syringomyelia: analysis of MRI and curve progression". Eur Spine J 16 (10): 1629–35. doi:10.1007/s00586-007-0472-1. PMC 2078323. PMID 17701226.
SOURCES: Mark Ware, M.D., director, clinical research, Alan Edwards Pain Management Unit, McGill University Health Centre, Montreal; Henry McQuay, D.M., professor, chronic pain unit, Oxford University, Oxford, England; Aug. 30, 2010, Canadian Medical Association Journal