Genetic diseases

Cerebromedullary malformation syndrome

Syndrome Arnold Chiari

Arnold Chiari Syndrome photo Syndrome Arnold - Chiari syndrome (anomalies Arnold - Chiari) - congenital abnormality of the brain, which appears inconsistent with the size of the posterior fossa, and are in the area of ​​brain structures, so thatptosis occurs cerebellar tonsils and brainstem into the foramen magnum, which causes them to infringe on this level.Often this defect combined with abnormalities of the spinal cord, and hydrocephalus.Most of the patients suffering from the syndrome Arnold - Chiari have no signs or symptoms of the disease and therefore do not need treatment.Most often, this pathology is found by chance during diagnostic procedures for suspected other diseases.The incidence anomaly Arnold - Chiari is 3-8 per 100,000 population.

causes of this syndrome to date, has not been fully established

Syndrome Arnold - Chiari symptoms

If this anomaly is usually observed several of the below given symptoms:

- The precarious and / or dizziness, which intensified when corneringhead

- noise, hum, whistle, hissing, ringing in one or both ears, which can also be aggravated by turning your head

- Due to the increased intracranial pressure, or as a result of increasing the tone of the neck muscles, appears quite sensitive headache (the most heavilymanifested in the morning)

- There is an involuntary jerking of the eyeballs (nystagmus)

in more severe anomalies Arnold - Chiari present the following symptoms:

- observed visual disturbances (expressed as double vision, coming blindness, etc.), which are oftenworse when turning the head

- There are ataxia, tremor of arms and legs

- reduces the sensitivity of the body, the face, a few, or one limb

- Urinating becomes difficult and / or involuntary

- There is muscle weakness of the torso,part of the face, a few, or one limb

- There may be loss of consciousness (most often provoked by turning the head)

- Perhaps the development of conditions that can lead to a heart attack the spinal and / or brain

Possible consequences and complicationsthe syndrome of Arnold - Chiari

- Paralysis .The paralysis may develop as a result of compression of the spinal cord and persist even conducted a field surgery

- Hydrocephalus .Caused by the accumulation in the brain of excess fluid may require placement of a shunt (flexible tube) to drain cerebrospinal fluid and exhaust in any - any other part of the body

- Syringomyelia .Sometimes patients suffering from an abnormality of Arnold - Chiari disease develops, in which the spine is formed a cyst or cavity (abnormal cavity in the spinal cord or brain).After the formation, this cavity is filled with fluid, which leads to disruption of the spinal cord

Syndrome Arnold - Chiari treatment

Treatment of anomalies Arnold - Chiari syndrome is a highly individual approach and are directly dependent on the state and severity of each patient.In the absence of severe symptoms, the doctor as a treatment prescribed observation with periodic regular inspections.If the primary symptoms are pain, recommended intake of painkillers.In some cases, patients with this syndrome brings significant relief receiving anti-inflammatory drugs that can delay or completely prevent the carrying out of surgery

Operative treatment

The most common treatment of anomalies Arnold - Chiari performed surgical method, which aims to stop the progression of changes inthe structure of the spine and brain, as well as to stabilize symptoms.In case of a successful outcome of the operation, the pressure on the spinal cord and the cerebellum is reduced, and the normal flow of cerebrospinal fluid is restored.

most common surgery for this syndrome is decompression of the posterior fossa or posterior fossa kraniektomiya.The operation involves the removal of the back of the skull of a small fragment of the bone, thereby providing more space for the brain and reduced pressure.At this time surgery it takes one to two hours and the recovery period is typically less than seven days.

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