Malformations of spine and spinal cord

Arnold-Chiari malformation is a malformation of the brain in which the portion of the skull containing the cerebellum is too small or malformed, causing compression of the brain. The lowest part of the cerebellum, or tonsils, is pushed into the upper part of the spinal canal. The pediatric form, Arnold-Chiari type III, is always associated with a myelomeningocele (a herniation of the spinal cord and meninges). The adult form, Chiari type I, occurs because the back of the skull is not large enough. When the cerebellum is pushed into the upper part of the spinal canal, it can block the normal flow of cerebrospinal fluid, which protects the brain and spinal cord. The impaired circulation of cerebrospinal fluid can lead to a blockage of signals from the brain to the organs below or to a buildup of cerebrospinal fluid in the brain and spinal cord. Pressure from the cerebellum on the spinal cord or lower part of the brainstem can cause syringomyelia.

Symptoms

• Headaches triggered by coughing, sneezing or physical overexertion;
• Numbness and tingling in the hands;
• Impaired temperature sensitivity in the hands, frequent burns;
• Pain in the back of the head and neck (periodically descending to the shoulders);
• Uneven gait (problems with maintaining balance);
• Weak coordination of the hands (fine motor skills).

Syringomyelia (from the Greek syrinx - reed, tube + myllos - brain) is a disease characterized by the formation of cystic cavities in the thickness of the spinal cord. There are two main forms of syringomyelia - communicating and non-communicating. Communicating syringomyelia is a widening of the central canal of the spinal cord, which is normally obliterated. The main cause of this form of syringomyelia is a disturbance of cerebrospinal fluid circulation at the level of the craniovertebral junction, which is especially often observed in such malformations as Chiari malformation, Dandy-Walker syndrome and some others. Cystic widening of the central canal in the medulla oblongata leads to the development of syringobulbia. Non-communicating 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 canal of the spinal cord and do not communicate with the subarachnoid space.

Symptoms

Syringomyelia can develop asymptomatically for a long time, with symptoms increasing slowly. Usually, the earliest symptom is the loss of pain and temperature sensitivity in one or both hands. A person can cause significant injury to themselves without experiencing pain. Then muscle weakness appears, the muscles atrophy - the arms "lose weight". Gradually, the symptoms spread to the rest of the body, usually in the form of separate zones. In the zones of impaired sensitivity, sweating is also impaired, which is most often absent, but in some areas it can be increased. Later, pain joins in, most often persistent headache, then muscle pain of a neuralgic nature. Sometimes pain can be an early symptom. With syringomyelia, bones lose calcium, as a result of which they become fragile and brittle, however, even severe bone fractures with syringomyelia are painless. The muscular-articular apparatus atrophies. Gradually, the disease becomes generalized.

Diastematomyelia is a longitudinal splitting of the spinal cord into separate parts due to the ingrowth of bone and cartilaginous septa into it. Each half or part of the spinal cord is covered with its own membrane.

Symptoms

Clinical manifestations of diastematomyelia consist of several symptoms. The main complaint of patients is spinal deformity. In 90% of cases, the pathology is accompanied by the presence of neurological deficit (muscle weakness, gait disturbances and pelvic organ dysfunction, sometimes - minimal neurological symptoms). Diastematomyelia can be combined with myelomeningocele. In 75% of cases, skin disorders are detected above the diastematomyelia zone - areas of hypertrichosis, lipomas, nevi, hemangiomas.