Arnold-Kiari's anomaly – inborn defect in development of a rhombencephalon. This anomaly by discrepancy of the sizes of back cranial area and the brain components located in it is shown. As a result it leads to the fact that a part of almonds of a cerebellum and brain falls to a big occipital opening where they are restrained.
Statistically, this pathology is observed at 3-8 people from each 100 thousand.
Today the exact reason of development of anomaly of Kiari is not established. Most likely, display of this disease is accompanied by three following factors:
At this pathology the cerebellum is in a back cranial pole.
Normal the lower part of a cerebellum (almond) has to be located above a big occipital opening. At Arnold-Kiari's anomaly of an almond are located in the vertebral channel, that is under a big occipital opening.
The big occipital opening serves as a peculiar border between a backbone and a skull, and also between back and a brain. Over this opening the back cranial pole, and under it – the vertebral channel is located.
The lower part of a brainstem passes (myelencephalon) into a spinal cord at the level of a big occipital opening. Normal liquor (cerebrospinal fluid) has to circulate freely in subarachnoid spaces of a spinal and brain cord. These subarachnoid spaces connect among themselves at the level of a big occipital opening that provides free outflow of cerebrospinal fluid from a brain.
At Arnold-Kiari's anomaly of an almond are located under a big occipital opening that complicates free current of liquor between a head and spinal cord. Almonds of a cerebellum block a big occipital opening like a stopper that significantly breaks outflow of cerebrospinal fluid and leads to development of hydrocephaly.
In 1891 Kiari defined four main types of pathology and in detail described everyone. Doctors use this classification also till today.
The third and fourth types of anomaly of Arnold-Kiari will not respond to treatment and, as a rule, lead to a lethal outcome.
At preferential most of patients (about 80%) Arnold-Kiari's anomaly is combined with a myelosyringosis – pathology of a spinal cord of which formation of the cysts in it promoting development of the progressing myelipathy is characteristic. Similar cysts at omission of structures of a back cranial pole are formed and as a result of a prelum of cervical department of a spinal cord.
The following clinical signs are characteristic of this pathology:
At more started disease stages weakening of a gag reflex, episodes of an apnoea (temporary cessation of breathing) and the involuntary bystry movements of eyes join symptoms of anomaly of Arnold-Kiari.
This disease is fraught with development of the following complications:
Sometimes Arnold-Kiari's anomaly passes asymptomatically and it is found only when carrying out the general research of the patient.
The main diagnostic method of this disease is MRT of chest and cervical departments of a spinal cord and MRT of a brain today. MRT of a spinal cord do preferential for the purpose of detection of a myelosyringosis.
If the disease has only one symptom – pain in a neck, then treatment of anomaly of Arnold-Kiari generally conservative. Therapy includes various schemes with use of muscle relaxants and nonsteroid antiinflammatory medicines.
If the effect of conservative treatment insufficient or is absent at all for 2-3 months of treatment, and also in the presence at the patient of symptoms of neurologic insufficiency (weakness and numbness of extremities, etc.), then the doctor, as a rule, offers carrying out operation at Arnold-Kiari's anomaly.
Main goal of carrying out operation at Arnold-Kiari's anomaly is minimizing of infringement of nerve terminations and fabrics and normalization of outflow of cerebrospinal fluid for what the size of a back cranial pole is a little increased in volume. As a result of operation at Arnold-Kiari's anomaly the headache completely disappears or decreases, motive functions and sensitivity of extremities are partially recovered.
The educated person is less subject to brain diseases. Intellectual activity promotes formation of the additional fabric compensating sick.
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