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The progressing nadjyaderny paralysis


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.

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