Paget ‘s disease is a chronic systemic bone disease, but it is often more limited in nature. The origin of the disease remains unclear. Paget ‘s disease is considered to be senile, because its clinical symptoms in noticeable form usually appear at the age of 50-70 years, however, with a targeted x-ray examination, it is possible to identify its initial symptoms in middle age. Diseases occur very slowly over many years or decades. Having begun, it progresses to death. With this disease, the long bones of the limbs, especially the lower ones, are also affected, and flat, but from a neurological point of view, the damage to the spine and skull attracts attention. In the spine most often affects a number of neighboring vertebrae or a whole section of it, less often there is an isolated process in one or more disparate vertebrae. Morphologically,
Paget’s disease is a typical fibrous osteodystrophy, with complex processes of destruction and creation of bone material, in which bone tissue is rearranged by lacunar resorption followed by osteoblastic (and partly metablastic ) neoplasm of an inferior bone substance rich in osteoid elements. Radiologically, when focal points of sharp darkening and enlightenment and other characteristics of changes are revealed in the bone, it is not difficult to establish a diagnosis of Paget’s disease . The vertebral body is flattened from top to bottom and the height of the body decreases very significantly. A normal serrated network gives way to a roughly trabecular binding. Angular deformations of the spine sometimes reach such a sharp degree that the corresponding neurological symptoms of partial compression of the spinal cord, or less often its roots, reappear. Pathological spinal fractures are frequent, but neurological complications are relatively rare with these fractures. It is believed that even with clear symptoms of spinal cord compression as a result of
Paget’s disease , there are almost no indications for decompression, moreover, the expressed symptoms of this compression usually appear already in old age and it is difficult to exclude that spinal pathology is not a consequence of arteriosclerosis. It should be noted that many patients with moderate manifestations of symptomatic compression of the spinal cord over the years in a home stay feel relatively satisfactory. The defeat of the skull manifests itself either against the background of systemic Paget’s disease , or is the only localization of this disease.
Characteristic is an increase in head volume, patients are forced to increase the size of headgear, the head hangs over the facial skull and hangs the chin on the chest . In this case, headaches of a burning or boring nature are sometimes noted; with compression of the hemispheres of the brain, behavioral disorders and epileptic seizures can be observed, with narrowing of the cranial nerve exit openings on the base of the skull, these are symptoms of damage to these nerves. A significant thickening of the planar (integumentary) bones of the skull is revealed x-ray, while the normal differentiation of the pattern into the outer and inner compact plates with a more transparent cHp1 between them disappears. The surface of the skull takes on a peculiar appearance of a smeared wide ribbon, against which more dense islands of irregular shape stand out. There is no cure. For bone pain, analgesics are prescribed, radiation therapy is sometimes used for the same purpose.
Achondroplasia
Achondroplasia ( achondroplasia ), or chondrodystrophy, is a systemic congenital disorder of the skeletal enchondral development, which is manifested by slow and perverse growth of cartilage cells along the ossification of developing bones. From a neurological point of view, cases of spinal injury deserve special attention, in which there is a narrowing of the lumen of the spinal canal with a shortening of the legs of the vertebral arches; Moreover, the presence of dorsal kyphosis with a tendency to develop a thickening of the border plates of the vertebral bodies, the formation of osteoarthritic osteophytes and protrusion of the intervertebral discs at one or several levels leads to an even greater narrowing of the spinal canal.
The consequence of this is the onset of symptoms of compression of the intravertebral masses. With the localization of the main pathological process in the cervical region, spastic tetraparesis appears , with a lower level of damage, lower spastic paraparesis with pelvic organ disorders. Most often, this narrowing appears, starting with a Thu level of the vertebra and lower, with paresthesia and pain in the lower extremities (especially at the time of standing) and weakness in them, causing patients to lower. Myelography clarifies the level of greatest narrowing of the lumen of the spinal canal. With the development of paresis or other serious neurological disorders, a wide laminectomy is indicated, after which the progression of these disorders is suspended or partial recovery is observed.