About morvan disease -- syringomyelia

What is morvan disease -- syringomyelia?

Syringomyelia is a neurological disorder characterized by the formation of a fluid-filled cyst (syrinx) within the spinal cord. This cyst may, for unknown reasons, expand during adolescence or the young adult years, destroying the center of the spinal cord as it does.

As the syrinx expands, it affects the nerves that stimulate the patient's legs, arms, back and shoulders. In turn, the affected individual may feel considerable pain and weakness, and may lose the ability to distinguish hot from cold objects. The combination of symptoms and signs may vary considerably from person to person, depending on where in the spine the syrinx is located and how much it expands over time. Often, the symptoms develop slowly. Unless the condition is treated surgically, it may lead to neurological deficits and chronic, intense pain.

What are the symptoms for morvan disease -- syringomyelia?

Pain in the neck and arms symptom was found in the morvan disease -- syringomyelia condition

The specific symptoms and severity of syringomyelia can vary greatly from one person to another. Some individuals may not have any noticeable symptoms (asymptomatic); others may have a variety of symptoms that can progress to cause significant disability. It is important to note the highly variable nature of syringomyelia and to realize that affected individuals can have different sets of symptoms and a different rate of progression (or no progression of the disease at all).

Syringomyelia is usually slowly progressive, but rapid onset can occur. Common symptoms include Pain in the neck and shoulders. Pain may also affect the arms and hands and may be described as a burning, tingling or piercing sensation. Some affected individuals also experience numbness or decreased sensation, especially to hot and cold. Muscle Weakness and wasting, especially of the hands, arms, and eventually the shoulders, may also develop. The upper (cervical and thoracic) portions of the spinal cord are often affected in syringomyelia. Affected individuals may first notice a loss of feeling for Pain and temperature in their fingers, hands, arms, and upper chest. In the early stages, a sense of touch is still present. A loss of feeling may spread over the shoulders and back, described as a “cape-like” distribution.

Affected individuals may also develop Pain and stiffness (spasticity) in the legs and uncoordinated movements (ataxia), eventually affecting the ability to walk. In severe cases, Paralysis of the arms or legs can occur. Some affected individuals may develop Muscle contractions such as small, involuntary Muscle contractions or “twitches” (fasciculations).

Skeletal abnormalities can develop including the abnormal side-to-side curvature of the spine (scoliosis). In some children, Scoliosis may be the only symptom. Some individuals may develop Charcot joints, in which chronic, progressive degeneration of the joint occurs because of damage to the nerves that supply the joint. Charcot joints are initially seen as Swelling and Redness of the affected areas. Without treatment, deformity of the affected joints can occur.

Some affected individuals develop symptoms associated with damage to the autonomic nervous system, which is the part of the nervous system that controls involuntary functions. Such symptoms include loss of bowel and bladder control, excessive Sweating (hyperhidrosis), and fluctuating blood pressure levels. Horner’s syndrome, an uncommon condition that develops because of damage to one of the nerves that supplies the eyes and face, may also occur. Horner’s syndrome usually affects one side of the face and is characterized by a droopy eyelid, narrowing of the opening between the eyelids, decreased pupil size, and Decreased Sweating on the affected side of the face.

Individuals with syringomyelia associated with a Chiari malformation may also have a condition called hydrocephalus, in which there is an abnormal accumulation of cerebrospinal fluid in the brain. In infancy, hydrocephalus can cause a variety of symptoms including an abnormally enlarged head, vomiting, headache, sleepiness, irritability, seizures, and downward deviation of the eyes.

What are the causes for morvan disease -- syringomyelia?

The exact, underlying reason for the formation of a syrinx is unknown. Most theories implicate the obstruction or disruption of the flow of cerebrospinal fluid (CSF) as the most common cause. CSF is a clear fluid that surrounds and is within the brain and surrounds the spinal cord. CSF has several functions including protecting and cushioning the brain and giving the brain buoyancy. CSF is also found in the central canal, a small canal that runs through the center of the spinal cord in infants. The central canal eventually collapses over time. A variety of conditions that block or impair the normal flow cerebrospinal fluid have been associated with syringomyelia. The condition most commonly associated with syringomyelia is the Chiari malformations, a group of complex brain abnormalities that affect the area in the lower back of the skull where the brain and spinal cord connect (craniovertebral junction).

Syringomyelia can also develop following spinal cord injury (SCI). These cases are sometimes referred to as posttraumatic syringomyelia. The development of posttraumatic syringomyelia may occur many years after the initial traumatic injury.

Additional conditions that are associated with syringomyelia include certain spinal cord tumors, meningitis, inflammation of the arachnoid membrane (arachnoiditis), one of the membranes that surrounds and protects the spinal cord, and a tethered spinal cord, which is usually a stretch-induced functional disorder associated with the fixation (tethering) effect of inelastic tissue (filum terminale usually infiltrated with fat) found at the caudal end of the spinal cord, limiting its movement.

Spinal dysraphism (spina bifida occulta), which is characterized by malformations of the spinal canal and its contents, can also be associated with syringomyelia. Spinal dysraphism is often associated with a tethered spinal cord.

In some individuals, syringomyelia can develop without any known cause. These cases are referred to as idiopathic syringomyelia.

What are the treatments for morvan disease -- syringomyelia?

The treatment of syringomyelia is directed toward the specific symptoms that are apparent in each individual. Treatment may require the coordinated efforts of a team of specialists. Pediatricians, neurologists, neurosurgeons, surgeons, eye specialists (ophthalmologists) and other healthcare professionals may need to systematically and comprehensively plan an affected child’s treatment.

Specific therapeutic procedures and interventions may vary, depending upon numerous factors, such as disease progression; the presence or absence of certain symptoms; the underlying cause; the impact of symptoms on quality of life; an individual’s age and general health; and/or other elements. Decisions concerning the use of particular drug regimens and/or other treatments should be made by physicians and other members of the health care team in careful consultation with the patient based upon the specifics of his or her case; a thorough discussion of the potential benefits and risks, including possible side effects and long-term effects; patient preference; and other appropriate factors.

Some individuals with syringomyelia who do not have any symptoms may not require treatment, but should be regularly monitored to see whether the disorder progresses.

General therapeutic options include pain medications (analgesics), physical therapy, and a reduction in activities, especially those that require straining such as heavy lifting. The goal of treatment for syringomyelia is to restore the proper flow of cerebrospinal fluid and to remove the pressure that a syrinx places on the spinal cord. Initial treatment is usually targeted at the underlying cause of syringomyelia. There is no specific, agreed-upon therapy or treatment regimen for the most common cause of a syrinx, a Chiari malformation. Neurosurgeons and other physicians may disagree as to the best approach to treat a Chiari malformation. Different neurosurgeons may recommend different surgical techniques or treatment regimens.

Like syringomyelia, individuals with a Chiari malformation who do not have symptoms are generally not treated, but monitored to see whether the disorder progresses. If mild or nonspecific symptoms are present, such as neck pain or headaches, physicians may recommend conservative treatment. Symptomatic Chiari malformations are most often treated by surgery. There are no specific criteria or objective tests that can be used to determine when to undergo surgery or the best procedures to choose. The most common surgery is known as posterior fossa decompression. With this procedure, a surgeon creates room by removing small pieces of bone in the back of the skull, thereby enlarging the foramen magnum. This relieves pressure and reduces compression of the brainstem. The surgeon may also choose to open the covering (dura mater) of the brain in this region and explore the herniated tissue and then sew in a graft (duraplasty).

If hydrocephalus is associated with the Chiari malformation-related syrinx, then the surgeon will usually treat this first. The most common surgical treatment is to place a shunt that connects the dilated ventricles of the brain to another cavity of the body. The most common cavity chosen is the abdomen. Once the hydrocephalus is treated, then the Chiari malformation is addressed.

Surgery may be used to treat other conditions that cause syringomyelia including surgery to remove a tumor. Tethered spinal cord may require surgery to release the tension of the cord.

In some cases, a tiny tube called a shunt/stent may be placed into the syrinx. The shunt allows the fluid within the syrinx to drain to an area outside of the spinal column. Shunting can stop the progression of the disorder and relieve some symptoms such as pain and headaches. However, shunts can be associated with significant side effects including spinal cord injury or infection, bleeding (hemorrhaging), and blockage.

Posttraumatic syringomyelia may be difficult to treat. Surgery is recommended for individuals with neurological deterioration and/or intractable pain. Surgery is aimed at expanding the area around the spinal cord by the site of trauma or injury and to decrease fluid volume. The insertion of shunt may also be used to treat posttraumatic syringomyelia. Shunting in posttraumatic syringomyelia carries risks including further spinal cord injury and may need to be replaced if the shunt becomes clogged or defective. Many physicians consider shunts a last resort for individuals with posttraumatic syringomyelia.

Surgery for syringomyelia can often lead to an improvement of symptoms and stabilization of the disorder in many individuals. However, syringomyelia can recur after successful treatment, thereby necessitating more operations. After surgery, the physician will evaluate the syrinx to make sure that it stabilizes or decreases in size. These evaluations will entail obtaining an MRI.

What are the risk factors for morvan disease -- syringomyelia?

Syringomyelia most commonly presents in young adults between 20 and 40 years of age, but can also develop in young children or older adults. Some reports suggest that syringomyelia is slightly more common in males than females. One estimate places the incidence at 8.4 individuals per 100,000 in the general population in the United States.

Is there a cure/medications for morvan disease -- syringomyelia?

Your doctor would probably advise surgery if the symptoms of Morvan disease, syringomyelia are interfering with your life or if they are rapidly getting worse.

  • Surgery aims to relieve the pressure the syrinx puts on your spinal cord and return the cerebrospinal fluid to its regular flow. Your symptoms and nervous system performance may improve as a result of this. The etiology of your syringomyelia will determine the kind of surgery you require.

Surgery alternatives to relieve pressure on your brain and spinal cord include:

  • Chiari malformation treatment: Surgery may entail removing a small portion of bone from the back of your skull if the Chiari deformity is the root cause of your syringomyelia. This procedure can relieve pressure on your brain and spinal cord, return your cerebrospinal fluid flow to normal, and perhaps treat or cure syringomyelia.
  • Cleaning the throat: A shunt, also known as a drainage device, will be surgically inserted by your doctor. It consists of a flexible tube that directs the flow of fluid from the syrinx. The tubing is inserted with one end in the syrinx and the other in another body part, such as the abdomen.
  • Removing the obstruction: An barrier to the passage of cerebrospinal fluid within your spinal cord, such as a tumor or bone growth, may be surgically removed in order to allow fluid to drain from the syrinx.

Headache,Pain in the neck and arms
Spinal curvatures,Loss of sensitivity to pain, and temperature

Video related to morvan disease -- syringomyelia