Syringomyelia

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Introduction

T2-weighted MRI of a syrinx located at C6–C7 in the cervical spine

Syringomyelia refers to a cystic collection, or syrinx, that occurs within the spinal cord around the central canal. It is a rare neurogenic disease. As the cyst gets larger, it presses on the spinal cord and interferes with the transmission of nerve impulses. Syringomyelia predominantly presents with sensory symptoms such as pain and temperature insensitivity.

• Causes include congenital brain defects, spinal cord trauma and infection. The incidence of syringomyelia following trauma which could result in paraplegia is higher than 1%–4%.
• Syringomyelia accounts for up to 5% of paraplegia. The quality of life in syringomyelia patients is comparable with that of patients with heart failure or malignant neoplasms.

Classification

Classification of Syringomyelia

Communicating: This is characterized by central canal dilatation that communicates with the fourth ventricle and is invariably associated with hydrocephalus. The cavity of the syrinx features an ependymal lining.

Non-communicating (isolated): This involves dilations of the central canal resulting from altered CSF flow dynamics within the spinal subarachnoid space resulting from CM-1, basilar impression, and arachnoiditis. These dilations tend to dissect paracentrally into the posterolateral quadrant of the spinal cord. Non-communicating syringomyelia is the most common type of syringomyelia.

Extracanalicular: This is observed in the watershed area of the spinal cord following trauma or strokes and is characteristically associated with myelomalacia. These syrinx cavities are lined with glial or fibroglial tissue.

Types

Types include:

• Type I: Syringomyelia due to blockage of CSF circulation (without fourth ventricular communication): Representing at least 50% of all cases, this is the most common type of syringomyelia. There is obstruction at the foramen magnum and subsequent dilation of the central spinal canal caused by conditions such as CM-1, basilar invagination.
• Type II: Syringomyelia with fourth ventricle communication: Approximately 10% of syringomyelia cases are of this type. These are often considered Idiopathic.
• Type III: Syringomyelia due to spinal cord injury or lesions directly affecting the spinal cord, including tumors, traumatic myelopathy, spinal arachnoiditis, pachymeningitis, and myelomalacia. Fewer than 10% of syringomyelia cases are of this type.
• Type IV: Pure hydromyelia
• Idiopathic Syringomyelia has an unknown cause and cannot be classified under any of the previous categories

Epidemiology

Epidemiological data on syringomyelia is limited. Some studies have found the prevalence of syringomyelia to be from 8.4/100,000 to 0.9/10,000 with ethnic and geographic variation. The majority of the patients present between the ages of 20 to 50.

Characteristics/Clinical Presentation

A syrinx may be clinically silent and is often identified incidentally during spinal cord imaging. In symptomatic cases of syringomyelia, patients typically present with bilateral motor and sensory deficits, sparing the head and face, consistent with the presentation of other spinal cord lesions.

Symptoms often fluctuate in severity and duration. The combination of symptoms varies between patients and is influenced by the size and location of the syrinx within the spinal cord.

• Patients often experience pain, weakness, and stiffness in the back, shoulders, and extremities.
• Loss of the ability to sense extreme hot or cold, particularly in the hands, is commonly seen.
• A cape-like pattern of reduced pain and temperature sensation along the back and arms is frequently observed, caused by damage to centrally crossing spinothalamic tract fibers.
• Autonomic bladder and bowel dysfunction, which only appears in the advanced stages, is a rare clinical finding.

Syringomyelia involving the cervical spinal cord can at times hand weakness and sensory abnormalities. These symptoms can closely resemble those of symptomatic Chiari-II malformation, a condition invariably present in these patients.

Watch this informative 5 minute video.

Diagnostic Procedures

The procedure for identifying Syringomyelia varies depending on the patients’ complaints. 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 identifying this disease. Furthermore, we look for symptoms as we have seen in the ‘differential diagnosis’ topic.

Treatment

The goal of treatment is to correct the underlying causative pathophysiology. All current treatment strategies are directed toward improving CSF flow dynamics.

Physical Therapy Management 

The physical therapy for patients suffering from Syringomyelia differs depending on the location and impact of the disease unless presented with an MRI, a neurological examination should clear out at which level the syrinx occurs. The goals of the treatment are to stop the spinal cord damage from getting worse using the techniques explained in the following paragraph and to maximize functioning. They may require active physical therapy, passive mobilizations, occupational therapy or even speech therapy.

We try to focus the therapy on the following subjects:

1. Increasing their muscle strength (Usually upper extremity and paravertebral muscles, using training schemes individualized to the patients’ tolerance)
2. Maintaining range of motion using stretching and mobilizations of the neck and upper limb.
3. Training neck stability (best guided by physiotherapist at first)
4. Sitting and standing balance can be physical and occupational therapy (referring to daily activities as in getting dressed and grooming)
5. Educating the patients about their disease and it’s process over time. It is important to maintain an active lifestyle but there are several risks in high impact activities which should not be overlooked. (See topic “patient advice during follow up after surgery) They should also learn about adequate pain management and coping techniques
6. Improving and/or maintaining communication using speech therapy (when the syrinx is present in the lower brain stem, although that should be referred to as ‘syringobulbia’)
7. Maximizing functional capabilities by testing and asking the patient about daily activities and then acting upon the answers. This includes activities such as getting in and out of bed, walking, using a cane or crutches and such. Physical therapists may also refer a patient to the use of leg braces if they are deemed appropriate.

Patient advice during follow up after surgery:

Hospital Course: pain relief will be the most significant factor. Therefore it is important the patient receives a lot of bed rest and moves carefully.

• During the first month after surgery: when the operation is successful, the pain and neurological symptoms should improve rapidly. The patient should try to maintain a modestly active lifestyle. It is important that these patients try to avoid tasks involving heavy lifting! Walking and performing daily activities at ease is highly recommended.
• On the long term: an adequate follow-up is very important for these patients. If they do not deteriorate, they should try to maintain a relatively active lifestyle.
• Avoid:
  • Activities that cause excessive strain on the nervous system and spinal cord.
  • Any activity in which high impact can be expected (e.g. football, rugby…)
  • Avoid straining due to heavy bowel  movement
  • Avoid extensive coughing 
  • Most important: Avoid heavy lifting and straining