syringomyelia

What is Syringomyelia?

Syringomyelia is a rare neurological disorder characterized by the formation of a fluid-filled cavity or cyst, called a syrinx, within the spinal cord. This condition can lead to damage and compression of the nerve fibers that carry information from the brain to the rest of the body and vice versa.

Formation of Syrinx

The syrinx is a fluid-filled cavity that forms within the spinal cord parenchyma or central canal. It can get bigger and elongate over time, resulting in damage to the surrounding spinal cord tissue and compression of the nerve fibers. This can lead to various symptoms, including pain, sensory disturbances, weakness, and impaired motor function.

Types of Syringomyelia

Syringomyelia can be classified into two main types:

• Acquired syringomyelia: Also known as primary spinal or noncommunicating syringomyelia, this type occurs when a cyst forms in a damaged part of the spinal cord.
• Congenital syringomyelia: This type is present at birth and is often associated with other congenital anomalies.

Causes and Risk Factors

While the exact cause of syringomyelia is still unknown, it is believed to be related to various factors, including:

• Chiari malformation: An abnormal condition in which brain tissue extends through the hole at the bottom of the skull (foramen magnum) and into the spinal canal.
• Spinal cord injuries
• Spinal cord tumors
• Damage caused by trauma or infection

Symptoms and Diagnosis

The symptoms of syringomyelia can vary depending on the location and size of the syrinx. Common symptoms include:

• Pain: Pain is a common symptom, often described as burning, stabbing, or aching.
• Sensory disturbances: Patients may experience numbness, tingling, or loss of sensation in the affected area.
• Weakness: Weakness or paralysis can occur if the syrinx compresses the spinal cord.

Diagnosis is typically made through a combination of:

• Imaging studies: MRI and CT scans are used to visualize the spinal cord and detect any abnormalities.
• Clinical examination: A thorough physical examination and medical history are essential in diagnosing syringomyelia.

Treatment and Prognosis

While there is no cure for syringomyelia, treatment options aim to alleviate symptoms and prevent further damage. These may include:

• Surgery: Surgical intervention can help relieve pressure on the spinal cord and improve symptoms.
• Pain management: Medications and other interventions are used to manage pain and discomfort.
• Physical therapy: Physical therapy can help maintain or improve motor function.

The prognosis for syringomyelia varies depending on the severity of the condition, the effectiveness of treatment, and individual factors. In some cases, patients may experience significant improvement in symptoms, while others may require ongoing management and care.

Common Signs and Symptoms of Syringomyelia

Syringomyelia, a rare neurological disorder, can cause a range of symptoms that vary depending on the location and size of the syrinx (cyst-like structure) in the spinal cord. Here are some common signs and symptoms:

• Pain: Pain is one of the most common symptoms of syringomyelia, often concentrated in the neck, upper back, and shoulders [3]. The pain can be severe and may occur in other areas such as the lower back, stomach, or legs [5].
• Muscle Weakness: Muscle weakness, particularly in the arms and hands, is another common symptom [4, 7].
• Numbness or Decreased Sensation: Numbness or decreased sensation, especially to pain and temperature, can occur on one or both sides of the body [4, 6].
• Loss of Balance: Some people may experience loss of balance or dizziness (vertigo) due to the syrinx affecting the spinal cord's ability to regulate balance [6].
• Loss of Bladder Control: In some cases, syringomyelia can cause loss of bladder control or other neurological symptoms such as weakness in extremities [4].

Other Possible Symptoms

In addition to these common symptoms, some people may experience:

• Flaccid weakness of the hands and arms
• Deficits in pain and temperature sensation in a capelike distribution over the back and neck
• Rapid, involuntary rolling of the eyeballs (nystagmus)
• Pain, weakness, and stiffness in the back, shoulders, arms, or legs

It's essential to note that symptoms can occur suddenly after coughing or straining in some cases [8]. If you're experiencing any of these symptoms, it's crucial to consult a healthcare professional for proper diagnosis and treatment.

References: [3] - Symptoms of Syringomyelia · Pain (sometimes severe, and usually concentrated in the neck, upper back, and shoulders; pain may occur in the lower back, stomach, ... [4] - Neurological symptoms like pain, numbness, weakness in extremities, loss of balance, or loss of bladder control can develop based on where the syrinx is located ... [5] - Common signs/symptoms include pain in the neck and shoulders, muscle weakness, pain and stiffness in the legs, numbness or decreased sensation, especially to ... [6] - Syrinx Symptoms · Dizziness (vertigo) · Involuntary rapid movement of the eyeballs (nystagmus) · Loss of sensitivity to pain and temperature on one or both sides ... [7] - Symptoms include flaccid weakness of the hands and arms and deficits in pain and temperature sensation in a capelike distribution over the back and neck; light ... [8] - Pain, weakness and stiffness in the back, shoulders, arms or legs; Rapid, involuntary rolling of the eyeballs. Symptoms can occur suddenly after coughing or ...

Diagnostic Tests for Syringomyelia

Syringomyelia, a rare condition characterized by the formation of fluid-filled cavities (syrinxes) within the spinal cord, can be diagnosed through various tests. The primary goal of these diagnostic tests is to confirm the presence of syringomyelia and rule out other conditions that may cause similar symptoms.

Common Diagnostic Tests

• Magnetic Resonance Imaging (MRI): An MRI scan of the spine and spinal cord is the most reliable tool for diagnosing syringomyelia. This test provides a detailed view of the spine and can clearly show tumors or syrinxes [1]. MRIs are also used to rule out other conditions that may cause similar symptoms, such as multiple sclerosis or spinal cord injuries [5].
• Physical Examination: A physical exam focusing on neurological function is an essential part of diagnosing syringomyelia. This includes a thorough examination of the nervous system and assessment of symptoms such as numbness, weakness, or pain in the arms or legs [2].
• Medical History Review: Your healthcare provider will review your medical history to identify any previous conditions that may be related to syringomyelia.

Additional Tests

In some cases, additional tests may be ordered to confirm the diagnosis of syringomyelia. These include:

• Computed Tomography (CT) scans: CT scans can provide detailed images of the spine and spinal cord, but they are not as effective as MRI scans in diagnosing syringomyelia [9].
• Electromyography (EMG): EMG tests can help rule out other conditions that may cause similar symptoms, such as peripheral nerve damage or muscle disorders.

References

[1] Mar 20, 2024 — Tests may include: MRI. An MRI scan of the spine and spinal cord is the most reliable tool for diagnosing syringomyelia. [2] To diagnose syringomyelia, your healthcare provider will review your medical history and perform a physical exam focusing on neurological function. [3] Apr 17, 2023 — Physicians primarily use magnetic resonance imaging (MRI) to diagnose syringomyelia. [4] If a patient presents with symptoms associated with syringomyelia, the surgeon may order a magnetic resonance imaging (MR) scan. [5] MRIs are the most common ways to diagnose syringomyelia, as they provide a detailed view of the spine and can clearly show tumors or syrinxes. [6] Your health care provider will perform a physical exam and ask about the symptoms, focusing on the nervous system. Tests that may be done include: MRI of the spine. [7] Sep 8, 2022 — Syringomyelia Diagnosis​​ Doctors use magnetic resonance imaging (MRI) scans of the brain and spine to see if you have syringomyelia. [8] A neurological exam should rule out most of the symptoms we find in Syringomyelia Also, a well-executed anamnesis is one of the main diagnostic procedures in diagnosing this condition. [9] Diagnosis of syringomyelia · medical history · physical examination · magnetic resonance imaging (MRI) · computed tomography (CT) scans · electromyography – to test for muscle and nerve function.

Treatment Options for Syringomyelia

Syringomyelia, a chronic condition characterized by the formation of a fluid-filled cavity (syrinx) within the spinal cord, can be challenging to treat. While there is no known effective treatment that can completely cure the condition, various medications and surgical interventions can help manage its symptoms.

Medications for Pain Management

• Pain-relievers: Medications such as ibuprofen, acetylsalicylic acid, naproxen, indomethacin, mefenamic acid, and piroxicam may be prescribed to alleviate pain associated with syringomyelia [1].
• Muscle relaxants: Centrally acting antispasticity agents like baclofen (Lioresal) can help reduce muscle spasms and discomfort [4].

Other Medications

• Gabapentin: An anticonvulsant medication, gabapentin may be prescribed to manage postherpetic neuralgia and epilepsy-related symptoms [9].
• Tricyclic antidepressants: Amitriptyline (Elavil) can help alleviate pain and improve mood in some patients [4].

Surgical Interventions

• Shunting: A thin tube may be inserted to drain the syrinx and relieve pressure on surrounding nerve tissue [7].
• Restoration of spinal cord function: Surgery might involve removing a small section of bone at the base of the skull (Chiari malformation) if it's causing the condition [3].

Limitations and Goals of Treatment

While these treatments can help manage symptoms, there is no known effective treatment for syringomyelia. The primary goal of treatment is to stop the spinal cord damage from getting worse and alleviate associated pain and discomfort [8][11].

Syringomyelia, a condition characterized by the formation of a fluid-filled cavity or syrinx within the spinal cord, can be challenging to diagnose due to its varied and often subtle symptoms. When considering differential diagnoses for syringomyelia, several conditions should be taken into account.

• Persistent or dilated central canal (hydromyelia, slits): This condition involves an abnormal dilation of the central canal within the spinal cord, which can mimic the symptoms of syringomyelia.
• Myelomalacia: A condition where the spinal cord tissue becomes softened and damaged, often due to trauma or compression.
• Tumoral cysts: Benign or malignant tumors that can form within the spinal cord, potentially causing similar symptoms to syringomyelia.
• Myelitis: Inflammation of the spinal cord, which can be caused by various factors such as infections, autoimmune disorders, or trauma.
• Amyotrophic lateral sclerosis (ALS): A progressive neurodegenerative disease that affects motor neurons, leading to muscle weakness and atrophy.

These conditions should be considered in the differential diagnosis of syringomyelia, especially when evaluating patients with similar symptoms. Accurate diagnosis is crucial for effective treatment and management of these conditions.

References:

• [1] Apr 17, 2023 — Failure to recognize the symptoms of syringomyelia or attributing symptoms (pain, excess sweating, increased spasticity, numbness, or weakness) ...
• [5] The differential diagnosis includes persistent or dilated central canal (hydromyelia, slits), myelomalacia, tumoral cysts, myelitis, amyotrophic lateral sclerosis.
• [9] by LL Goetz · 2023 · Cited by 12 — Differential Diagnosis; Prognosis; Complications; Consultations; Deterrence and Patient Education; Enhancing Healthcare Team Outcomes; Review