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What is syringomyelia? -

Syringomyelia is a condition in which a cyst filled with fluid (called a syrinx) forms within the spinal cord.

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What is syringomyelia? -

The syrinx can grow over time and can lead to damage to the spinal cord. A bigger and elongated syrinx can also compress and damage the nerve fibers in the area, which carry important information to and from the brain.

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What is the fluid in the cyst? -

The fluid that fills the cyst is cerebrospinal fluid (CSF). This is the fluid that protects the spinal cord and the brain.

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How does it occur? -

CSF collects within the spinal cord and then expands the central canal, forming a syrinx. This happens when the flow of CSF fluid is disturbed, leading to the development of syringomyelia.

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Who’s at risk? -

The condition affects mainly people aged between 20 and 50, though young children and older adults can also be diagnosed with syringomyelia.

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How common is it? -

Syringomyelia affects approximately eight out of 100,000 people, making it a pretty rare condition.

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Symptoms -

Symptoms of syringomyelia worsen over time, and those with a small syrinx may not even experience them for a long time. Though if the syrinx gets large enough, a variety of symptoms may appear. Let’s take a look at some of the most common.

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Symptoms -

Symptoms of syringomyelia include headaches, radiculopathy (nerve pain down the limbs or back), muscle atrophy and weakness, and muscle tightness and spasms (mostly in the back, shoulders, neck, and limbs).

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Symptoms -

Other symptoms include limb tingling or numbness, as well as loss of sensitivity to temperature or pain (usually in the hands).

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Symptoms -

Balance problems and difficulty walking can also occur in people with syringomyelia, as well as loss of bladder and bowel control. Sexual function may also suffer as a result of the condition.

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Symptoms in children -

Children may develop specific symptoms of syringomyelia, including scoliosis (curvature of the spine) and toe walking.

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Causes -

The exact reason why syringomyelia develops remains unknown. The disruption or obstruction of the flow of cerebrospinal fluid (CSF) is the most likely cause. Why this happens can vary.

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Associated causes of syringomyelia -

There are, however, two main types of associated causes; congenital (from birth) and acquired (occurring later in life). Chiari malformation type 1 is one of the most common associated congenital causes.

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Congenital causes of syringomyelia: Chiari malformation type 1 -

Chiari malformation type 1 occurs when the lower part of the cerebellum extends into a hole located at the base of the skull. The spinal cord passes through this hole, so when the normal flow of CSF is obstructed, it can result in syringomyelia.

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Congenital causes of syringomyelia: myelomeningocele -

Myelomeningocele, aka open spina bifida, is a birth defect that can lead to syringomyelia. Myelomeningocele occurs when the spine and spinal canal don’t close before birth.

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Congenital causes of syringomyelia: tethered cord syndrome -

Tethered cord syndrome is when the spinal cord is attached to the surrounding tissues of the spine. This rare condition is often linked with scoliosis and spina bifida.

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Acquired causes of syringomyelia: spinal cord injuries -

Syringomyelia can also develop later in life, and spinal cord injuries are one of the most common causes. This post-traumatic syringomyelia is usually caused by scar tissue buildup, which ends up blocking the normal flow of CSF.

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Acquired causes of syringomyelia: spinal cord tumors -

Spinal cord tumors, such as ependymomas and hemangioblastomas, can affect the normal flow of CSF, and consequently result in syringomyelia.

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Acquired causes of syringomyelia: Inflammation of the arachnoid membrane (arachnoiditis) -

This membrane surrounds and protects the spinal cord, so when it gets inflamed, it can cause syringomyelia. Inflammation can be caused by a number of conditions, including multiple sclerosis (MS), sarcoidosis, and transverse myelitis.

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Acquired causes of syringomyelia: meningitis -

Meningitis occurs when there is an infection in the protective lining around the brain and spinal cord (called the meninges). This too can cause syringomyelia.

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Diagnosis -

Patients are usually referred to a neurologist, who will go through the person’s medical history and likely prescribe a number of exams, including medical imaging tests of the spine or brain.

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Diagnosis -

Common imaging tests used to diagnose syringomyelia include a CT scan, myelogram, and MRI.

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Treatment -

Treatment will depend on a number of factors, namely the severity of symptoms, as well as the cause of syringomyelia. If the patient shows no symptoms, it's usually left untreated. Though periodic monitoring is usually recommended.

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Treatment -

Looking at the underlying cause of the syrinx and managing the symptoms is, however, the course of action with some patients. The goal is to prevent further damage to the spinal cord and restore the normal flow of CSF.

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Treatment -

This may involve taking pain medication, physiotherapy, and lifestyle changes (e.g. restricting activities that may cause further damage). If the syrinx gets too big and/or the patient is experiencing severe symptoms, sometimes surgery is recommended.

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Surgery: Chiari malformation -

Posterior fossa decompression is a common surgery performed to treat Chiari malformation. It involves removing tiny pieces of bone from the back of the skull to reduce pressure on the brain and spinal cord and restore the regular flow of CSF.

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Surgery: post-traumatic syringomyelia -

Expansive duraplasty surgery is usually performed to remove scar tissue around the spinal cord, allowing CSF to flow normally.

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Surgery: obstruction removal -

In addition to bone and scar tissue, sometimes the culprit is a tumor. If this is the case and radiation therapy doesn’t shrink the tumor enough, surgery is performed to remove it.

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Surgery: draining -

When there is no obvious underlying cause, draining the syrinx is an option to reduce its size and consequently any symptoms it may be causing.

Sources: (Cleveland Clinic) (NINDS) (Mayo Clinic) (WebMD)

See also: Stars who suffer from rare diseases

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