Syringomyelia is a rare disease of the spinal cord characterized by the presence of a fluid-filled cavity, with resulting neurologic defects.
The symptoms of syringomyelia usually present slowly and may eventually lead to permanent disabilities. Signs and symptoms of significant syrinx formation are mostly observed in ages 25 to 40 years old. It is imperative to identify the early signs of the disease to merit prompt medical or surgical attention.
In the head and neck areas, signs are often times unilateral. The tongue may present with weakness and hemiatrophy. Difficulty in deglutition may be experienced early. Facial paresis or diminished sensation of the face may be experienced up to the nape area. The eyes will noticeably have slow reaction to tactile stimulus or altered reflexes. Skin areas where supplying nerves are affected by the lesion may experience diminished pain and temperature sensation. Pain may be felt in certain dermatomes supplied by the affected nerves. The lower extremities are more prone to weakness or spasms during caudal compressions although arm fasciculation may be experienced in cervical lesions. Stiffness of back muscles, shoulders and arms are common. Spine deformities may be observable in large spinal tumors while scoliosis of the spine is not infrequent. The lower abdomen may be affected in syringes within the thoracic spinal cavity causing disruption in bowel activity and urinary bladder functions. Chronic constipation and bladder atony may be observable in these cases.
A detailed medical history and clinical examination is paramount in the diagnosis of syringomyelia. The following imaging techniques offer the most reliable diagnostic choice :
Asymptomatic syringomyelia may be treated with constant monitoring and conservative treatment, in severe, symptomatic cases, surgery is necessary. Identification of the underlying etiology is paramount to identify the appropriate treatment. The conservative treatment includes analgesics and physical therapy. The following surgical approaches are available:
Successful surgery reduces the size of the syrinx. Regular follow-up with MRI is important to detect recurrence early.
The intensity and extent of the symptoms are not directly dependent on the size of the syrinx. In mild non-progressive asymptomatic cases of syringomyelia, an uneventful clinical course may be expected. In progressive and enlarging syrinx however, progressive loss of motor and sensory functions of the extremities may be observed. Unabated compression may cause severe disability to the patient in the long run. Surgical therapy and the underlying etiology may significantly affect prognosis and outcome of the disease .
A syringomyelia may be congenital or acquired . Congenital forms are common and in about 50% associated with malformations such as Arnold Chiari malformation, platybasia and Klippel-Feil syndrome. There are a number of diseases that can lead to the development of syringomyelia, including:
In about 20 to 30% of cases, no cause can be found, this is referred to as idiopathic syringomyelia.
The prevalence of syringomyelia is relatively low and is estimated at about 84 cases per 1,000,000 inhabitants. In Japan only 19 cases per million population were described. In most cases the disease occurs in the third and fourth decade of life, childhood cases have been reported.
The exact pathophysiology in syringomyelia remains unknown. However there are leading theories to explain it:
Patients diagnosed early with syringomyelia should avoid all occasion of straining and lifting to prevent the enlargement of the syrinx. For those with neurologic complications, physical therapy may significantly reduce pain symptoms. Chronic pain may be submitted for pain management to prevent aggravation of symptoms .
Syringomyelia is an acquired or congenital disease that involves the development of a fluid-filled cavity or “syrinx” within the spinal cord. Small, slowly progressive lesions may be asymptomatic. However, typical symptoms of the disease are dissociated sensory loss at the level of the lesion, pain, trophic and vegetative disorders. Since obstruction of CSF pulsation is the cause of the syringomyelia, a potential therapy is surgical removal of this cause. Although the syringomyelia is not operated directly, it will be smaller when the cause has been removed successfully. The risk of the surgical procedure has to be considered when discussing possible surgical treatment options. In some patients, in spite of all diagnostic possibilities, no cause of the disease can be found. Here, an operation is rarely indicated and conservative treatment options, such as physical therapy or antiepileptic medicatio ncan be considered.
Syringomyelia is the development of a fluid-filled cyst or cavity, called syrinx, within the spinal cord. The most common cause of syringomyelia is an abnormality of the brain (Arnold-Chiari malformation), other causes include tumors, injuries and inflammation. Common symptoms are pain, abnormal sensations, loss of sensation, weakness, stiffness, among others. If the condition is not causing severe symptoms, monitoring and conservative treatment (painkillers and physical therapy) may be sufficient. In other cases, surgery will be needed.