Syringomyelia is a rare neurological disease, characterized by the presence of fluid-filled cystic cavities within the spinal cord.
It is a chronic progressive disease, whose incidence is 1 case per 10,000 people. Besides, it can affect both sexes and all races equally.
What is the cause?
The syrinx usually begins to form in the medulla at the level of the neck. It expands slowly, initially dilating the centre of the spinal cord, injuring the fibres that collect information on the sensations of pain and temperature.
The syrinxes can often be located within the cord, in all its extension. Most of the cases are of unknown origin.
What are the symptoms?
The initial symptoms can appear at any age, but most tend to debut between 25 and 30 years, being very rare in those over 60 years.
The symptoms usually begin in young adulthood. It starts with the alteration of sensation in the upper limbs. It is characterized by a lack of thermal sensation versus preservation of the touch.
The neurological deterioration is progressive with the appearance of muscular atrophy and areflexia (absence of reflex).
The occurrence of spontaneous pain is frequent, usually of gradual onset, but exceptionally of sudden onset, which can be intense and affect the trunk and upper and/or lower limbs.
Headaches and scoliosis (abnormal oblique curvature of the thoracic spine) are also common.
The diagnosis depends on the symptoms, and it is confirmed by the complementary tests. All patients must undergo a thorough neurological examination.
Currently, magnetic resonance imaging is useful in both for diagnosis and for the follow-up and investigation of the disease. This method allows clearly delimiting the intramedullary cavity, showing the cystic cavity and its extension. Besides, it allows detecting anomalies associated with the disease.
The treatment is based in neurosurgery. It looks for cavity drainage and water decompression, but will vary depending on the extension of syringomyelia.