From Big Medical Encyclopedia

KRANIOVERTEBRALNY ANOMALIES (grech, kranion skull + lat. vertebra vertebra; anomaly) — the defects of development of kraniovertebralny area which are quite often combined with a prelum of a myelencephalon, cerebellum and upper parts of a spinal cord. To. and. can be inborn or form in the course of life.

Distinguish five main versions To. and.

1. Assimilation (occipitalization) of the Atlas — the union of the first cervical vertebra with an occipital bone which is quite often combined with an underdevelopment and shift it in relation to epistrofeyu.

2. Anomaly of development of tooth of an axial vertebra (epistrofeya) or its copular device, leading to removal of the Atlas forward and to a prelum of a spinal cord. If tooth of an axial vertebra puts in a big occipital opening, then there are disorders of vital functions.

3. Basilar impression (platybasia) is formed because the backbone presses the basis of an occipital bone and a slope into a cavity of a back cranial pole. At the same time the corner between the surfaces of a wedge-shaped bone and a slope increases to 160 — 180 ° (normal it does not exceed 140 °). Conditions for a prelum of a myelencephalon and upper parts of a spinal cord are as a result created. This anomaly is quite often combined with assimilation of the Atlas.

4. Arnold's anomaly — Kiari is characterized by the shift of sites of a cerebellum or a brain trunk to the vertebral canal in connection with disturbance of development of a back cranial pole. At the same time the prelum of a spinal cord can be observed.

5. Klippel's syndrome — Feylya, or a syndrome of a short neck, arises in connection with a synostosis of two or several cervical vertebrae (see. Klippelya — Feylya a disease ). Sometimes cervical edges or not fusions of handles of vertebrae are found. Defects of development of cervical department of a spinal cord are possible.

In some cases the combined forms can be observed.

Outward of patients with is characteristic To. and.: short neck, low border of pilosis, forced position of the head, restriction of mobility of cervical department of a backbone.

The first signs of defeat of a nervous system appear in various years of life, a thicket in 20 — 30 years.

Nevrol, symptomatology is caused by damage of upper parts spinal cord (see), myelencephalon (see) and cerebellum (see). Symmetric increase in periosteal and tendon jerks on hands and legs at relative safety of force and lack of spasticity is characteristic in them, frustration of joint muscle sense in extremities, segmented or conduction frustration painful and a thermoesthesia, pain in a nape and a neck, an ataxy.

At a platybasia, in addition to pyramidal and cerebellar symptoms, there can be paresis or paralyzes of the IX—XII pairs of cranial nerves. Arnold's anomalies — Kiari are inherent vestibular and cerebellar symptoms, symptoms of defeat III, VI, VII or XII pairs of cranial nerves; at a lumbar puncture signs of blockade subarachnoid spaces come to light.

At differential diagnosis To. and. and tumors of this localization have crucial importance given to a X-ray analysis of kraniovertebralny area.

At treatment analgetics, Muscle relaxants, antispasmodics in combination with physiotherapeutic procedures are applied. Operational treatment is directed to elimination of a compression of upper parts of a spinal cord, a trunk of a brain and cerebellum.

Easy forms K. and. proceed asymptomatically. If anomaly is expressed sharply, it can lead to death of the patient. Treatment reduces expressiveness nevrol, symptoms also are improved by the general condition of the patient.

Bibliography: Dyomina V. S. To clinic of craniospinal anomalies, in book: Vopr, wedge, and Eksperim, neuropath., under the editorship of N. S. Misyuk, page 67, Minsk, 1964; Deryabina E. I. To diagnosis of craniospinal anomalies, in book: Sketches wedge, nevrol., under the editorship of S. N. Davidenkov, century 1, page 71, L., 1962; it, the Spinal syndrome at kranio-vertebralny anomalies, Zhurn, a neuropath, and psikhiat., t. 71, No. 8, page 1178, 1971; Kosinskaya N. S. Disturbances of development of the kostnosustavny device, page 180, L., 1966; Bares L. Basilar impression and the so-called associated anomalies, Europ. Neurol. (Basel), v. 13, p. 92, 1975.

H. S. Misyuk.