Definition/Description

Syringomyelia is a rare neurogenic disease which can damage the spinal cord
due to formation of a fluid-filled area in the form of a cyst (Syrinx), usually
found in the high cervical spine. (Also found in lumbar area but this is very
rare)
The name derives from the word Syrinx, greek for a tube-formed object and
the Myelum, referring to the spinal cord.

Types of Syringomyelia:

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. Obstruction of CSF (cerebro-spinal fluid) circulation from
the basal posterior fossa to the caudal space may cause syringomyelia of this
type. The most common example is Arnold-Chiari malformation, which is also
associated with communicating syringomyelia.
 Other causes include the following:

 Basal arachnoiditis (postinfectious, inflammatory, postirradiation, blood in

subarachnoid space) [1]
 Basilar impression or invagination
 Meningeal carcinomatosis
 Pathological masses (arachnoid cysts, rheumatoid arthritis pannus, occipital
encephalocele, tumors)

Syringomyelia with fourth ventricle communication

Approximately 10% of syringomyelia cases are of this type. The

communication is visible on MRI. In some cases a blockage of CSF circulation
occurs: a shunt operation may be the best therapeutic option for these
patients.

Syringomyelia due to spinal cord injury

Fewer than 10% of syringomyelia cases are of this type. Mechanisms of injury
include:

1. spinal trauma
2. radiation necrosis
3. hemorrhage from aneurysm rupture or arteriovenous malformation or in a
tumour bed
4. infection (spinal abscess, human immunodeficiency virus, transverse myelitis)
5. cavitation following ischaemic injury or degenerative disease

Idiopathic Syringomyelia

Idiopathic syringomyelia has an unknown cause and cannot be classified

under any of the previous categories.[2] Surgical decompression can help in
some patients with remarkable neurologic deficit [2].

Clinically Relevant Anatomy

There is no exact location where the Syrinx may occur. The most common
location is the upper cervical spine. Since a picture says more than a thousand
words, here is a schematic representation of an upper chiari deformation in
both sagittal and intersectional view[3].

We can observe how the Syrinx causes stress on the spinal cord and damages
it on a long term.

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 [4].

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
The damage to the spinal cord as seen in patients suffering from
Syringomyelia is caused by a Syrinx. This is comparable to a cyst, a cavity
filling with a fluid identical of similar to cerebrospinal fluid and extracellular
fluid, which slowly expands, putting pressure on the spinal cord and thus
damaging it. Such a Syrinx may be a result of a spinal cord trauma, pressing
spinal cord tumors, inflammation or birth-related defects (e.g. chiari
deformation or Arnold Chiari Malformation)[4][5]

The symptoms identifying this particular disease aren’t always clear. There
may be no symptoms at all (depending on the location of the Syrinx and the
 patients’ anatomical and proprioceptive properties) or there can be a number
of indications such as:

 Gradual muscle atrophy (especially scapular and cervical neck muscles)

 Muscle function loss and muscle weakness
 Decreased reflexes in the arm
 Persistent headaches
 Decreased sensation and sensitivity or even numbness
 Radiating pain through the neck, shoulder, upper arms and upper trunk (also
ventral)
 Uncoordinated movements, spasms and involuntary muscle contractions
 Dizziness
 Nystagmus (Jerky eye movements)
 Facial sensory impairment on one or both sides of the face

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. Clawhand 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 joints) may affect the shoulder, elbow, or
wrist.[6] Scoliosis is seen sometimes. [7]
Acute painful enlargement of the shoulder is associated with 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 tumor [8]

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 [8]

. 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 [9][10]

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 therapy 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.
[1. Level of evidence: C]

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.' [11] '[9]

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 aboute 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 of 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. [12]

 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