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NATIONAL CENTER FOR NEUROSURGERY

Anomalies of the spine and spinal cord development

Anomalies of the spine and spinal cord development (Arnold-Chiari syndrome, syringomyelia, diastematomyelia, etc.)

Arnold-Chiari syndrome -is an abnormality of the brain, in which section of the skull containing the cerebellum is too small or distorted, in the result the compression of the brain occurs. The lowest part of the cerebellar tonsils are displaced or in the upper part of the spinal canal. Pediatric shape - Arnold-Chiari syndrome type III - always associated with myelomeningocele (herniated spinal cord and meninges). The adult form - Chiari syndrome type I - develops due to insufficiently large back of the skull.

When the cerebellum is pushed to the top of the spinal canal, it can interfere with the normal outflow of cerebrospinal fluid that protects the brain and spinal cord. Abnormal CSF circulation can lead to blockage of signals transmitted from the brain to the underlying organs or cerebrospinal fluid accumulation in the brain and spinal cord. The pressure of the cerebellum to the spinal cord or the lower part of the brain stem can cause syringomyelia.

Symptoms

  •  Headache provoked by coughing, sneezing or physical overexertion
  •  Numbness and tingling in hands
  •  Violation of temperature sensation in the hands, frequent burns
  •  Pain in the back of the head and neck (sometimes going down to the shoulders)
  •  An uneven gait (difficulty in holding the balance)
  •  Poor coordination of hands (fine motor skills)

Syringomyelia (from the Greek. Syrinx - reed pipe + Greek. Myllos - brain) - a disease characterized by the formation of cystic cavities in the interior of the spinal cord. There are two main forms of syringomyelia - reported and unreported. Reported syringomyelia is an extension of the central canal of the spinal cord, which in normal conditions OBLITERATING. The main cause of this form of syringomyelia is a violation of the flow of cerebrospinal fluid at the level of the craniovertebral junction, which is especially common in the development of such deformities as Chiari malformation, Dandy-Walker syndrome, and others. Cystic expansion of the central channel in the medulla oblongata leads to siringobulbii. Unreported Syringomyelia is characterized by the formation of cysts in the thickness of the spinal cord as a result of trauma or inflammation. These cysts are not connected to the central spinal canal and does not communicate with the subarachnoid space.

Symptoms

Syringomyelia can develop without any symptoms for a long time, symptoms grow slowly. Typically, the earliest symptom is loss of pain and temperature sensitivity of one or both hands. A person can possibly cause substantial injury without experiencing pain. Then there is muscle weakness, muscle atrophy - hands " lose weight ". Symptoms gradually spread to the rest of the body, usually in the form of separate zones. In areas of impaired sensitivity sweating is impaired too, which is often not available, but in some areas can be strengthened.

Later you feel pain, often stubborn headache, and muscle pain of neuralgic character. Sometimes the pain can be an early symptom. When syringomyelia bones lose calcium, resulting in becoming brittle, however, even when severe fractures syringomyelia painless. Musculo-skeletal apparatus atrophy. Gradually, the disease takes generalized character.

Diastematomyelia - is a longitudinal splitting of the spinal cord into separate parts due to growing into bone and cartilage partitions. Each half or part of the spinal cord is covered with its own envelope.

Symptoms

Clinical sings of diastematomyelia are of several symptoms. The main complaint of patients - spinal deformity. In 90% of cases pathology is accompanied by the presence of neurological deficit (muscle weakness, gait and function of the pelvic organs, sometimes - minimal neurological symptoms). Diastematomyelia can be combined with myelomeningocele. In 75% of cases over the area of diastematomyelia one reveals skin lesions - areas of hypertrichosis, lipomas, nevi, hemangiomas.

Methods of treatment:

  • Decompression of the craniovertebral junction
  • Opening and drainage of syringomyelia cysts