Syringomyelia

Original Editor - Nick Libotton

Top Contributors - Nick Libotton, Wendy Walker, Nikhil Benhur Abburi, Kim Jackson, Lucinda hampton, Naomi O'Reilly, WikiSysop, Aminat Abolade, 127.0.0.1, Admin, Rachael Lowe, Evan Thomas and Vidya Acharya

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.
• Without treatment, syringomyelia may result in paraplegia or quadriplegia. Treatment options include surgery.
• 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.
  

Types

• 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.
• Syringomyelia with fourth ventricle communication: Approximately 10% of syringomyelia cases are of this type.
• Syringomyelia due to spinal cord injury: Fewer than 10% of syringomyelia cases are of this type.
• Idiopathic Syringomyelia has an unknown cause and cannot be classified under any of the previous categories
  

Epidemiology /Etiology   

Syringomyelia has a prevalence of approximately 8.4 out of 100.000 individuals. This is a rough worldwide estimation. Lately, the number of patients that remain stable grow, although an older study suggested that 20% of patients suffering from Syringomyelia died at an average age of 47.

The reason for this lowering is a result of surgical interventions, better physical therapy and better treatment of complications associated with significant paresis.

The disease usually appears in the third or fourth decade of life, with a mean age of onset of 30 years.
Rarely, syringomyelia may develop in childhood or late adulthood.

Syringomyelia occurs more frequently in men than in women.

Characteristics/Clinical Presentation

Patients with this condition demonstrate a wide variety of neurological symptoms depending on where exactly the syrinx is located, but classically patients present with:

• Cape-like loss of pain and temperature sensation along the back and arms, known as a ‘suspended sensory level’
• Disproportionately greater bilateral motor impairment in upper compared to lower extremities

This motor and sensory impairment can eventually cause atrophy, which begins in the hands and progresses proximally, resulting in deformities such as 'claw hands' and Charcot’s arthropathy

.

• 
  

Sensory

Dissociated sensory loss: Syrinx interrupts the decussating spinothalamic fibers that mediate pain and temperature sensibility, resulting in loss of these sensations, while light touch, vibration, and position senses are preserved.
When the cavity enlarges to involve the posterior columns, position and vibration senses in the feet are lost; astereognosis may be noted in the hands.
Pain and temperature sensation may be impaired in either or both arms, or in a shawl-like distribution across the shoulders and upper torso anteriorly and posteriorly.
Dysesthetic pain, a common complaint in syringomyelia, usually involves the neck and shoulders, but may follow a radicular distribution in the arms or trunk. The discomfort, which is sometimes experienced early in the course of the disease, generally is deep and aching and can be severe.

Motor

Syrinx extension into the anterior horns of the spinal cord damages motor neurons (lower motor neuron) and causes diffuse muscle atrophy that begins in the hands and progresses proximally to include the forearms and shoulder girdles. Claw hand may develop.
Respiratory insufficiency, which usually is related to changes in position, may occur.

Autonomic

Impaired bowel and bladder functions usually occur as a late manifestation.
Sexual dysfunction may develop in long-standing cases.
Horner syndrome may appear, reflecting damage to the sympathetic neurons in the intermediolateral cell column.

Other symptoms

• Painless ulcers of the hands are frequent.
• Oedema and hyperhidrosis can occur due to interruption of central autonomic pathways.
• Neurogenic arthropathies (Charcot's joints) may affect the shoulder, elbow, or wrist. Scoliosis is seen sometimes.
• Acute painful enlargement of the shoulder is associated with the destruction of the head of the humerus.

Differential Diagnosis

We have to differentiate between subtypes of cystic processes in the spinal cord; not all cystic lesions can be defined as Syringomyelia. Other similar cystic processes can be Hydromyelia, Myelomalacia, Cystic Neoplasms or Glioependymal Cysts. Syringomyelia can be easily differentiated because of its association with an underlying disorder that is characterized by obstruction of cerebrospinal fluid flow, tethering of the spinal cord, or a spinal tumour

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.

Outcome Measures   

At the moment, no specific Chiari or Syringomyelia scale has been made, but for now, we most often use the KPI: Karnofsky Performance Index

Although it is quite a crude measure, it relies on doctor assessment and some research indicates that the scale is not very reliable, we use it until a better-suited scale is created and has been tested for validity and reliability. 

Medical Management

There is no specific medical treatment for syringomyelia, but the origin of the disease can be met. For example, the pressure caused by the Syrinx can be relieved by surgical intervention and thus, reduce pain and stop further progression of neurological symptoms. Congenital syringomyelia may require that pieces of the cervical vertebrae in the area of the cavity be removed.

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.

Note: the following physical therapy techniques take into account the most frequent manifestations of this disease:

1. ROM
2. Muscle strength
3. Neck Stability
4. Balance
5. Coping
6. Occupational Therapy
   

We try to focus the therapy on the following subjects:

Maintaining range of motion using stretching and mobilizations of the neck and upper limb. Do note: there is no evidence to support the use of manipulations, and since the spinal cord has been damaged, most authorities advise avoiding this.'

1. Increasing their muscle strength (Usually upper extremity and paravertebral muscles, using training schemes individualized to the patients’ tolerance)
2. Training neck stability (best guided by physiotherapist at first)
3. Sitting and standing balance can be physical and occupational therapy (referring to daily activities as in getting dressed and grooming)
4. 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
5. 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’)
6. 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