Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (synonym Lou Gehrig's disease - Kozhevnikov) - a chronic progressive disease of the Central nervous system, mainly affecting motor neurons and pathways of the brain and spinal cord, accompanied by the development of muscular atrophy and paralysis of the upper and then lower extremities. Often the process applied to the cranial nerve nuclei. In the development of the disease are set to hereditary factors, as well as infections and intoxication.
The disease develops more often in the age of 40-50 years. Begins with weakness and fibrillar spasms in the muscles of the hands, passing on the muscles of the forearm, shoulder, shoulder girdle. Tendon reflexes increased, there are pathological reflexes. Gradually develop mixed paralysis of the upper extremities with a noticeable by atrophy, paresis or paralysis of the lower extremities, symptoms bulbar palsy (see), respiratory failure and cardiac activity. Out of frustration swallowing may develop aspiration pneumonia. The forecast is unfavorable: the disease progresses and ends with death.
Treatment: vitamins (B12 from 200 to 500 mcg intramuscularly, vitamin E), neostigmine 0.015 g 3 times a day, oksazil - 0.01 g 2-3 times a day. Needs special care, to feed the patient need to be careful with a spoon, in a sitting position, make sure that it is not choked.

Amyotrophic lateral sclerosis is a disease of the Central nervous system in which defeat is distributed mainly on the pyramid system and the front horns spinal cord. According to different authors, amyotrophic lateral sclerosis is about 3% of all organic diseases of the nervous system. Observed mainly at the age of 40-50 years.
Etiology. A. B. C. is considered by some authors as a degenerative disease, others - as a consequence of autointoxication by an unknown toxin.
In favor of viral origin were expressed M. S. Margulis and N. Century Konovalov (in electronic microscope on sections of the medulla oblongata N. Century Konovalov were detected particles round or elongated).
Pathology and pathogenesis. The greatest changes are detected in the bulbar, cervical and thoracic spinal cord; less and less frequently in the brain. In the membranes of the spinal cord are marked inflammatory changes. In the backs of the front and the front horns arise degenerative changes, reduced the size and number of ganglion cells. In the side columns marked diffuse degenerative process with pronounced symptoms of demyelination. Kortiko-muscularly (pyramid) path is hurt all over. Detected and vascular inflammatory reaction with okoloserdecna infiltrates (M. S. Margulis). The degenerative changes are observed in the cortex of the brain, especially in the layers III and V, as well as in the rear pillars of the spinal cord. Some authors consider one of the main pathogenetic factors of the disease congenital weakness of the pyramid system.
The symptomology. When amyotrophic lateral sclerosis are observed mainly motor and trophic disorders; sensory disorders expressed less and less frequent. The disease is sometimes preceded by pain or paresthesia in runes and legs. Typically onset - the appearance of weakness in the distal hands. Already in the early period can be found increasing tendon reflexes in the hands and feet; appear pathological reflexes: symptoms Babinsky, Rossolimo and others [see Reflexes (pathological)]. First, muscle tone gradually increases and reaches a high degree; with the rise of the atrophy tone could have been compromised. Arising in amyotrophic lateral sclerosis atrophy of muscles of the hand with a greater or lesser speed progress, hand takes the form of "bird" and "monkey" paws. Later atrophy capture the muscles of the shoulder and arm, and then and legs. Atrophy have degenerative in nature and are one of the most typical symptoms of amyotrophic lateral sclerosis (see muscular Atrophy). The initial quantitative changes of elektrovozoremontnij later replaced by the reaction of rebirth. Method of electromyography Yu. S. Usevich showed that already in the early stages of the disease can be detected oscillations of the potential and oscillations, pointing to the defeat of motoneurons. As the disease progresses, especially when it bulbar form, begin to disrupt speech and swallowing (due to the defeat language-pharyngeal, wandering and hypoglossal nerve). In the initial period bulbar disorders had little dysarthria (see) in speaking, podergivani when eating; may later come a speech disorder to the degree of anarthria (see) and the sharp swallowing disorder. At the same time there and the atrophy of muscles of tongue. Sensitive disorders are rare, are of a binding nature or distributed by type of glove and stocking".
The diagnosis. For amyotrophic lateral sclerosis is characterized by progressive disorder of motor functions with acceding degenerative atrophy hands in the absence or low intensity sensitive violations. When electric diagnostics can detect quantitative and qualitative changes (see above). Differential diagnosis have to spend with amyotrophic spinal syphilis, which atrophy less pronounced, no bulbar disorders, there are positive reaction Wasserman in the blood and cerebrospinal fluid. More often we have to differentiate amyotrophic lateral sclerosis with tick-borne encephalitis, which is characterized history (the bite of ticks), the initial acute infectious development, lower the severity of atrophy. Subacute polio adults from A. b. C. distinguish giporeflexia and hypotension.
Forecast of course unfavorable.
Treatment. Rational etiological treatment does not exist. Appoint vitamin B12 intramuscularly (500-1000 μg) up to 15 to 20 injections per course (in order to improve the exchange in diseased tissue), and vitamins B1, E, lecithin, lipocerin. For disorders of respiratory: artificial respiration (see), tracheotomy (see), followed by intratracheal injection of oxygen. For patients need constant care.