Syringomyelia in Arnold-Chiari Syndrome

Syringomyelia is a condition characterised by formation of fluid-filled cavities within the spinal cord. This can result in pain and loss of basic functions of the body, leading to weakness or numbness. Patients suffering from Chiari I malformation generally develop a syrinx; these two conditions are believed to be related. Syrinx occurs after meningitis, a spinal cord injury, surgery, or due to the presence of a tumour.

Syringomyelia seems to occur when there is an obstruction blocking normal flow of the cerebrospinal fluid (CSF). CSF is a protective fluid-covering that surrounds the brain and the spinal canal like a cushion, and protects the human body's central nervous system, along with transporting the nutrients and the waste products. When this flow of CSF is hindered, it gets diverted to the spinal cord region and the central canal. This, in turn, accumulates within the tissues of the spinal cord, resulting in a fluid-filled sac called syrinx. Syringomyelia manifests in two forms.

Syringomyelia mostly occurs as a result of Arnold-Chiari syndrome, wherein the cerebellum region protrudes by bulging into the spinal canal and blocks the flow of cerebrospinal fluid between the brain and the spine region. This condition is called 'communicating syringomyelia,' in which the syrinx formation is seen higher towards the cervical spine. If this blockage is not addressed quickly enough, it would expand to cause further damage. The second type of syringomyelia is called 'non-communicating syringomyelia,' which is usually seen after meningitis, trauma, haemorrhage, or the formation of tumor, among other reasons. Symptoms will be seen years after the initial injury; pain and weakness towards the lower extremities is usually experienced during the initial stages.

Diagnosis of Syringomyelia and/or Chiari Malformation

The diagnostic process starts with an assessment of the patient's medical history, followed by physical as well as neurological examinations. There are other specific tests carried out for revealing the abnormality and also the extent or degree of damage.

Imaging studies include:

This also includes electrodiagnostic studies such as Brainstem Auditory Evoked Response (BAER), Somasensory Evoke Response or Potential (SER, SSEP), and also Electromyography (EMG). These tests help determine if the spinal cord and the brainstem are functioning normally. An electromyography reveals more information regarding the functions of the spinal cord, as well as the sensory and the motor nerves.

Treatment

If syringomyelia does not give away any obvious signals, then a treatment would probably not be required. A surgical treatment can relieve and stop further progression of the symptoms. To get things started, one needs to seek an appointment with the general practitioner; a neurologist should be consulted only if recommended by the physician. You can request for an MRI scan, since it is considered the best diagnostic tool for confirming, or ruling out syringomyelia. A full-fledged spinal MRI will be ideal, as it can cover the entire spine. Also check whether a head MRI would be needed to help figure out the causes of syringomyelia. Chiari malformations and syringomyelia are difficult to be treated, as they involve operating at the hind portion of the head to enlarge the bony space, performing endoscopic third ventriculostomy, and inserting drainage tubes in the fluid spaces of the head or the syrinx.