Understanding Syringomyelia

Understanding Syringomyelia

Syringomyelia is a neurological disease that involves the formation of a cyst filled with cerebrospinal fluid in the spinal cord. This disease, in addition to damaging the spinal cord, surrounding tissues and nerves, can cause many symptoms, more or less severe.

What is syringomyelia?

Syringomyelia is a rare disease characterized by the abnormal formation of a cavity (called a syrinx) inside the spinal cord.

Syringomyelia is a generic term referring to a disorder in which a cyst or cavity forms within the  spinal cord. Often, syringomyelia is used as a generic term before an etiology is determined. Over time, this cyst, called a syrinx, can expand,  enlarge, elongate, and damage or destroying the spinal cord. A large, elongated syrinx can compress and damage nerve fibres in the area, which transmit important information to and from the brain. The fluid in the cavity is called cerebrospinal fluid (CSF). It serves to protect the spinal cord and brain. 

The damage may result in loss of feeling, 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. It may also lead to a cape-like bilateral loss of pain and temperature sensation along the upper chest and arms. The combination of symptoms varies from one patient to another depending on the location of the syrinx within the spinal cord, as well as its extent.

How does this disease appear?

Cerebrospinal fluid accumulates in the spinal cord, spreads into the central canal and forms a syrinx. Syringomyelia often appears as a result of poor circulation of cerebrospinal fluid.

People at risk:

This disease mainly affects people between the ages of 20 and 50, although young children and older people can also be affected.

Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing, straining, or myelopathy.

A fairly rare disease 

Syringomyelia has a prevalence estimated at 8.4 cases per 100,000 people, with symptoms usually beginning in young adulthood, making it a fairly rare disease.

Symptoms:

Syringomyelia symptoms get worse over time. Also, people with a small syrinx may not experience symptoms for a while. If the syrinx gets bigger, it can cause different symptoms.  

Even if some patients may experience severe chronic pain, the symptoms of syringomyelia include headache, radiculopathy (pain caused by compression of a nerve), muscle atrophy and weakness, and muscle stiffness and spasms (mainly in the back, shoulders, neck, and limbs).

Numbness or tingling in the limbs may also be noticed, as well as a decreased sensitivity to pain or temperature, often felt in the hands.

People with syringomyelia may also have problems with balance and gait, as well as problems controlling urine and stool. Sexual problems may also occur.

A syrinx may also cause disruptions in the parasympathetic and sympathetic nervous systems, leading to Horner syndrome, 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 be present.

Rarely, bladder stones can occur at the onset of weakness in the lower extremities. 

 Classically, syringomyelia spares the dorsal column/medial lemnicus of the spinal cord, leaving pressure, vibration, touch and proprioception intact in the upper extremities. Neuropathic arthopathy, also known as a Charcot joint, can occur, particularly in the shoulders, in patients with syringomyelia. The loss of sensory fibres to the joint is theorized to lead to degeneration of the joint over time.

Symptoms in children

Associated causes

• Chiari malformation type 1 is one of the most common associated congenital causes. Chiari malformation type 1 occurs when the lower part of the cerebellum extends into a hole at the base of the skull. Because the spinal cord passes through this hole, any obstruction to the normal flow of cerebrospinal fluid can cause syringomyelia.

• Congenital causes: myelomeningocele. Myelomeningocele, also known as spina bifida, is an embryological malformation characterized by non-closure of the neural tube before birth. 
• Tethered cord syndrome occurs when the spinal cord remains attached to the tissues of the spine. This rare condition is often associated with scoliosis or myelomeningocele.
• Arachnoiditis is an inflammation of the arachnoid membrane, which covers the spinal cord. This inflammation can be caused by a number of diseases, such as sarcoidosis, multiple sclerosis, or transverse myelitis. Arachnoids can trigger syringomyelia.

Acquired causes:

• spinal cord injuries Syringomyelia can also appear later in life, often following spinal cord injuries, which are one of the most common causes. This type of syringomyelia, post-traumatic, is usually due to the accumulation of scar tissue, which eventually hinders the circulation of cerebrospinal fluid.
• Spinal cord tumours, such as ependyma or hemangioblastoma, can affect the circulation of cerebrospinal fluid, and therefore trigger syringomyelia.
• Meningitis is an inflammation of the tissues surrounding the brain and spinal cord (the meninges). Meningitis can also trigger syringomyelia.

Diagnosis 

Patients are usually referred to a neurologist, who will review the person's medical history and likely order a battery of tests, such as imaging tests of the spine or brain. 

To diagnose this disease, a CT scan, a myelogram, or an MRI can be performed.

Treating the condition:

Treatment will depend on a number of factors, including the severity of symptoms and the cause of syringomyelia. If patients do not have symptoms, they are usually not treated. It is recommended to monitor the progression of the disease regularly. 

However, for some patients, finding the underlying cause of the syrinx and managing the symptoms is the course of action. The goal is to prevent further spinal cord injury and restore normal cerebrospinal fluid flow.

Treatment may include pain medication, physical therapy, or lifestyle changes (e.g., avoiding activities that can cause further damage). If the syrinx becomes enlarged and/or the patient experiences severe symptoms, surgery may be necessary.

The Surgery is need for this fourth case: 

• Surgery for Chiari Malformation.  The goal is to decompress the posterior fossa to free up enough space around the malformation to restore normal CSF circulation.
• Post-traumatic syringomyelia:  Duroplasty usually aims to remove scar tissue surrounding the spinal cord, which allows normal circulation of cerebrospinal fluid.
• Removing the tumour: In addition to bones and scar tissue, a tumour can also be responsible for the problem. In this case, the patient can opt for surgery to have it removed, if it has not shrunk after radiation therapy.
• Draining the Syrinx: If there is no underlying cause, the syrinx should be drained to reduce its size and the symptoms it may cause.

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