tailieunhanh - PARKINSON’S DISEASE AND RELATED DISORDERS - PART 2

Bất kỳ cuộc thảo luận về các đặc điểm lâm sàng của bệnh Parkinson phải đưa vào tài khoản không chính xác của chẩn đoán lâm sàng. Trong một loạt kế tiếp của 100 bệnh nhân với chẩn đoán lâm sàng của bệnh Parkinson, | Parkinson s disease Any discussion of the clinical characteristics of Parkinson s disease must take into account the inaccuracies of clinical diagnosis. In a successive series of 100 patients with a clinical diagnosis of Parkinson s disease only 76 fulfilled the criteria for diagnosis at post-mortem examination Table 1 . Attempts to tighten the diagnostic criteria lead to increased specificity but with reduced sensitivity. Neuropathology Typically there is loss of at least 50 of the melanincontaining nerve cells of the substantia nigra the changes concentrating in the central part of the zona compacta Figure 3 . Accompanying these changes is depletion of tyrosine hydroxylase the Table 1 Pathological findings in 100 successive Parkinsonian patients Idiopathic Parkinson s disease 76 Progressive supranuclear palsy 6 Multiple system atrophy 5 Alzheimer s disease 3 Alzheimer-type pathology with striatal involvement 3 Lacunar state 3 Nigral atrophy 2 Postencephalitic Parkinsonism 1 Normal essential tremor 1 from Hughes et al. 1992 rate-limiting enzyme in the biosynthetic pathway for catecholamines. Figures 4 and 5 . A characteristic indeed inevitable finding is the presence of Lewy bodies in some of the remaining nerve cells Figure 6 . Together with Lewy body formation degenerative changes occur at other sites including the locus ceruleus the dorsal motor nucleus of the vagus the hypothalamus the nucleus basalis of Meynert and the sympathetic ganglia. Cortical Lewy bodies are probably present in all patients with idiopathic Parkinson s disease although not with the frequency that would permit a diagnosis of cortical Lewy body disease vide infra . In Parkinsonian patients with cortical dementia the pathological changes are either those of cortical Lewy body disease or those associated with Alzheimer s disease including senile plaques neurofibrillary tangles granulovacuolar degeneration and nerve cell loss in the neocortex and hippocampus. Epidemiology The prevalence of .

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