Syringomyelia

Symptoms and Treatments
1

When I saw my NS last week, she said they also found a syrinx. I hadn't even thought about having that. It is new to me and the research that I have done doesn't really give me much information. I am having a lot of pressure in that specific area. It isn't painful, just really uncomfortable. Does anyone else have this issue? What other symptoms can that cause?

2

Its a cyst on the spine that’s formed from the fluid build up I’m being told. Mine actually went away with the stent. It can cause bone desenigration over time. Hope this helps a bit.

3

Syringomyelia and Syringobulbia

In syringomyelia there is fluid-filled tubular cavitation (syrinx formation) within the central, usually cervical, spinal cord. The syrinx can elongate, enlarge and expand into the grey and white matter and, as it does so, it compresses the nervous tissue of the corticospinal and spinothalamic tracts and the anterior horn cells. This leads to the various neurological symptoms and signs. If the syrinx extends into the brainstem, syringobulbia results.

Aetiology
  • A blockage in the circulation of cerebrospinal fluid (CSF) is the most common cause of syringomyelia. The most common reason for this blockage is the presence of a Chiari malformation. This is a developmental abnormality occurring in the fetus causing the cerebellum to protrude from its normal position through the foramen magnum and into the cervical portion of the spinal canal. There are 2 main types - type I and type II. Type I has no other associated cause and type II is associated with spina bifida.

    Other causes of disruption/blockage of CSF circulation include:
  • Spinal dysraphism: this is incomplete closure of the neural tube (neural tube defects including spina bifida). It is associated with syringomyelia. Identification and treatment of the dysraphism can arrest progression of syringomyelia.
  • Spinal cord injury: this can occur secondary to trauma, radiation, haemorrhage, ischaemic injury or infection.
  • Idiopathic syringomyelia: this occurs in some cases.
Associations
Epidemiology
  • Prevalence is 8.4 per 100,000 in the USA.1
  • It is more common in men than in women.
Presentation
  • Most commonly presents in the 20s and 30s but can present in childhood or later life.
  • The primary lesion may have been present since birth but the condition progresses very slowly. Progression of symptoms and deterioration seem to occur over many years.
  • Syringomyelia usually affects the cervical region.
  • Sudden exacerbations can occur and are thought to be caused by rupture of the syrinx because of raised venous pressure, as seen in sneezing or violent coughing.2

Sensory features

  • Pain and temperature sensation are lost due to spinothalamic tract damage. One side may be affected more than the other. Classically, the sensation loss is experienced in a shawl-like distribution over the arms, shoulders and upper body.
  • Dysaesthesia (pain experienced when the skin is touched) is common.
  • Light touch, vibration and position senses in the feet are affected as the syrinx enlarges into the dorsal columns.

Motor features

  • These begin to occur as the syrinx extends and damages the lower motor neurones of the anterior horn cells.
  • There is muscle wasting and weakness that begins in the hands and then affects the forearms and shoulders.
  • Tendon reflexes are lost.
  • Claw hand may be present.
  • There may be respiratory muscle involvement.

Autonomic features

4

i also found this i hope it helps to explain things.

Syringomyelia

Definition:

Syringomyelia (sear-IN-go-my-EEL-ya) is a disorder in which a cyst forms within the spinal cord. This cyst, called a syrinx, expands and elongates over time, destroying the center of the spinal cord. Since the spinal cord connects the brain to nerves in the extremities, this damage results in pain, weakness, and stiffness in the back, shoulders, arms, or legs. Other symptoms may include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Each patient experiences a different combination of symptoms. Magnetic resonance imaging (MRI) has significantly increased the number of syringomyelia cases diagnosed in the beginning stages of the disorder. Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing or straining. If not treated surgically, syringomyelia often leads to progressive weakness in the arms and legs, loss of hand sensation, and chronic, severe pain. In most cases, the disorder is related to a congenital abnormality of the brain called a Chiari I malformation. This malformation occurs during the development of the fetus and 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. Syringomyelia may occur as a complication of trauma, meningitis, hemorrhage, a tumor, or arachnoiditis. Symptoms may appear months or even years after the initial injury, starting with pain, weakness, and sensory impairment originating at the site of trauma. Some cases of syringomyelia are familial, although this is rare

Symptoms:

The symptoms of SM are numerous and a person may have various combinations of different symptoms. Symptoms tend to develop slowly, although sudden onset may occur with coughing and straining. Some common symptoms include: loss of sensitivity, especially to hot and cold, muscle weakness and spasticity, motor impairment, loss of bowel and bladder control, as well as osteoporosis and scoliosis. The majority of patients suffer from headaches and chronic pain.

Although Syringomyelia was first described over 400 years ago, very little research was performed and as a result little was known about the disorder. Only recently, with the advent of MRI and the dramatic rise in diagnosed cases, have significant advancements been made. In the past, SM was considered a disorder that always resulted in slow, progressive degeneration. Researchers now believe it is possible that some patients may never progress.

Treatment:

Surgery is usually recommended for syringomyelia patients. 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 be necessary to drain the syrinx, which can be accomplished using a catheter, drainage tubes, and valves.

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.

Prognosis:

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. Some patients, however, may have long periods of stability. Surgery results in stabilization or modest improvement in symptoms for most patients. Delay in treatment may result in irreversible spinal cord injury.