Overview: What is syringomyelia?
In syringomyelia, the spinal cord has one or more cavities that are filled with cerebrospinal fluid (CSF). These cavities are called syringes (singular: syrinx).
The fluid-filled cavities usually develop in the spinal cord of the cervical and upper thoracic spine. Sometimes a syrinx extends further up into the brain, into the lower brain stem. Experts then speak of syringobulbia.
Over the years, a syrinx can expand and grow larger. It can press on neighboring nerve tracts and damage them permanently. A wide variety of complaints are possible consequences. It often begins with a reduced perception of pain and temperature, for example in the hands. Other symptoms may occur later – including paralysis (especially of the legs) and bladder and bowel dysfunction.
Difference: syringomyelia and hydromyelia
A distinction must be made between syringomyelia and hydromyelia, which is a congenital dilation of the small central canal that naturally runs through the middle of the spinal cord and is filled with cerebrospinal fluid. It does not tend to increase in size over time – in contrast to the fluid-filled cavities in the spinal cord in syringomyelia.
Syringomyelia – frequency and age
Syringomyelia is a rare disease. It affects around eight in every 100,000 people. It is congenital, occurs after accidents, in the context of other (partly congenital diseases of the nervous system) or previous operations on the spine and affects men more frequently than women. The first symptoms of congenital forms usually appear between the ages of 20 and 40.
Syringomyelia: causes and risk factors
Syringomyelia is caused by impaired circulation of the cerebrospinal fluid (CSF). In the course of a day, around 500 milliliters of cerebrospinal fluid are produced in the brain, mostly in the ventricles. From there, the cerebrospinal fluid flows through all the interconnected cavities within the brain (inner cerebrospinal fluid space) and then enters the outer cerebrospinal fluid space via small openings. This is the gap-shaped space between the inner and middle meninges that surrounds both the brain and the spinal cord (subarachnoid space).
The cerebrospinal fluid therefore washes around the brain and spinal cord before it is absorbed into the tissue and transported away via the lymph channels.
If this constant flow is partially blocked at one point, the cerebrospinal fluid builds up in the narrow spinal canal – a cavity filled with cerebrospinal fluid (syrinx) is formed. The reason for the circulatory disorder is often congenital. In other cases, syringomyelia is acquired in the course of life.
Congenital syringomyelia
In 50 to 80 percent of all cases, syringomyelia is congenital. It is usually caused by a congenital malformation. This is very often a so-called Chiari malformation: as a result of a disorder in embryonic development, parts of the cerebellum and the hindbrain (medulla oblongata) are displaced downwards towards the spinal canal. This displacement of parts of the brain means that the cerebrospinal fluid can no longer flow freely. Those affected often also have a “hydrocephalus”.
Acquired syringomyelia
Possible causes of the acquired form of syringomyelia are
- severe spinal cord injuries: Syringomyelia can occur years or decades after the injury as a late complication.
- Inflammation: Inflammation of the meninges (e.g. the middle meninges) in the area of the spinal cord or (more rarely) the posterior fossa can lead to scarring or adhesions that impair the circulation of the cerebrospinal fluid.
- Tumors: Benign or malignant tumors of the spinal cord, the posterior cranial fossa or the meninges in the spinal cord area can also disrupt the flow of cerebrospinal fluid, for example because they constrict the cerebrospinal fluid space.
Symptoms: Syringomyelia
A fluid-filled cavity in the spinal cord can cause a wide range of symptoms due to pressure on the neighboring nerve tissue.
Possible symptoms:
- Diffuse shoulder-arm pain
- migraine-like headaches
- Sensory disorders, e.g. reduced perception of pain and temperature in the hands, sensitivity to touch in the limbs, impaired depth sensitivity (perception of the position and movement of the body in space)
- Sensations in the arms or legs (e.g. tingling)
- Muscle cramps, muscle twitching, loss of muscle mass (muscle atrophy), paralysis
- Disturbed sweat secretion
- Paw-like swelling of the hand (“paw hand”)
- Circulatory disorders, poor wound healing
- Decalcification of the bones with joint diseases, bone fractures, deformation of the bony ribcage or kyphoscoliosis (increased curvature of the spine backwards in combination with lateral curvature)
- Horner’s syndrome (combination of constricted pupil, drooping of the upper eyelid and the eyeball sinking into the eye socket)
- Bladder and bowel emptying disorders
- Sexual dysfunction, impotence (erectile dysfunction)
- Fatigue, rapid exhaustion, general weakness
- Sleep problems, depressive mood
The symptoms are variable. Which symptoms occur in individual cases may – but need not – be related to the location of the syrinx or syringes. There is also no correlation between the number and size of syringes on the one hand and the severity of the disease on the other.
Symptoms of syringobulbaria
Typical symptoms of syringobulbia – a fluid-filled cavity that extends from the spinal cord to the lower sections of the brain – include:
- Eye tremor (nystagmus)
- Dizziness
- Sensory disturbances in the face
- Speech disorders
- Atrophy of the tongue muscles (tongue atrophy), weakness of the tongue
- Speech disorder, swallowing disorder
- Hoarseness
Syringomyelia – diagnosis by us
First, we will take your medical history (anamnesis). To do this, we will ask you to describe all your symptoms in detail. We can also ask you about any pre-existing conditions and previous accident injuries.
This is followed by a physical and neurological examination. It includes, for example, testing the reflexes and strength of various muscles (such as those in the arms and legs).
If the medical history and physical-neurological examination lead to a suspicion of syringomyelia, a magnetic resonance imaging (MRI) scan of the spinal cord and brain can provide certainty: Syringes are easily recognizable on the detailed MRI images. The images also show the exact location and extent of the syringes.
Syringomyelia examination: magnetic resonance imaging (MRI)
Patients are often injected with a contrast agent containing gadolinium before the MRI examination. This usually makes it easier to detect a possible spinal cord tumor as the cause of syringomyelia on the images. In addition, the circulation of the cerebrospinal fluid can be recorded using a special MRI examination. This makes it possible to identify areas where the flow of cerebrospinal fluid is impaired.
X-ray examination for syringomyelia
An X-ray examination can be useful, for example, to visualize deformations of the spine (kyphoscoliosis) as a result of syringomyelia.
Syringomyelia: prevention, early detection, prognosis
To date, there are no known ways of preventing syringomyelia.
Syringomyelia – course and prognosis
Syringomyelia can take a very different individual course. The symptoms usually appear gradually and the disease progresses slowly (over years or decades). This can happen continuously or the disease may come to a temporary standstill. However, it can also be the case that the symptoms hardly change or do not change at all over a long period of time – or that the condition of those affected deteriorates very quickly.
It is not possible to predict what the prognosis will be in individual cases. If left untreated, chronically progressive syringomyelia can lead to severe disabilities. However, there are also people who are only slightly or not at all affected by the disease. In many cases, early treatment can counteract a worsening of the condition. To date, however, there is no cure for syringomyelia.
Syringomyelia: treatment is planned individually
The therapy for syringomyelia is adapted to the needs of the person affected. It consists primarily of conservative treatment to alleviate the symptoms. In some cases, surgical interventions may also be useful.