The progressing nadjyaderny paralysis
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Description:
The progressing nadjyaderny paralysis (Stila-Richardsona-Olszewski's syndrome) - the rare sporadic degenerative disease of TsNS which is characterized by a gliosis, a neurofibrillar degeneration and death of neurons of a mesencephalon, a varoliyev of the bridge, basal kernels and a gear kernel of a cerebellum. Neurofibrillar inclusions at the progressing nadjyaderny paralysis are presented by direct neurofilaments whereas at Alzheimer's disease they are spiralno bent. In a kernel having a tail and a shell concentration of dopamine and gomovanilinovy acid is reduced. Neurofibrillar inclusions are found both in a brainstem, and in bark of big hemispheres.
The reasons of the progressing nadjyaderny paralysis:
Disturbance of bonds of the centers of a look in a trunk and bark of big cerebral hemispheres is the reason of a nadjyaderny ophthalmoplegia. The Nadjyaderny ophthalmoplegia is characterized by look paralysis, i.e. lack of any consensual movements of eyes in the horizontal or vertical plane.
Symptoms of the progressing nadjyaderny paralysis:
Usually the disease begins at the age of 45-75 years. Men are ill twice more often. A characteristic sign - a nadjyaderny ophthalmoplegia. Sakkada (and also the bystry component of a railroad nystagmus is broken) in the vertical plane, preferential down are roughly broken. Later horizontal paresis of a look joins. Eventually also the smooth watching movements of eyes are broken. Test of doll eyes and cold test are not changed. Often dystonia, preferential razgibately neck, muscle tension of extremities and a hypokinesia joins that forces to carry out the differential diagnosis with Parkinson's disease. The tremor, however, is not characteristic. The combination of a nadjyaderny ophthalmoplegia to muscle tension of a trunk and neck leads to frequent falling. Defeat of a facial nerve, a dysarthtia, a dysphagy is possible, the mandibular reflex, an emetic reflex (pseudobulbar paralysis) raises. Violent laughter and crying is possible. Strengthening of tendon jerks, pathological extensive reflexes and cerebellar frustration are sometimes observed. Often the intelligence decreases, however focal disturbances of cortical functions are not characteristic.
The progressing nadjyaderny paralysis can be suspected when the patient of middle or advanced age complains of frequent falling, and at survey extrapyramidal frustration, dystonia of a razgibately neck and paresis of a look come to light down. The expressed look paresis down, horizontal paresis of a look, dominance of a tone of razgibatel of a trunk, lack of a tremor and a low performance of antiparkinsonichesky means distinguish this disease from Parkinson's disease.
Treatment of the progressing nadjyaderny paralysis:
The disease progresses, aspiration of food joins, exhaustion develops. Life expectancy usually does not exceed 10 years. Drug treatment is ineffective. Dofaminergichesky means can reduce rigidity and a hypokinesia, M-holinoblokatory (trigeksifenidit, 6-15 mg/days) and tricyclic antidepressants (amitriptyline, 50-75 mg before going to bed) sometimes improve the speech, gait and eliminate violent laughter and crying.
Efficiency of a levodofa is small; other means do not work at all.
