Enfermedad con cuerpos de Levy difusos: primeros datos de frecuencia en España

Lewy body disease (LBD) as a separate nosologic entity causing dementia in the elderly is being firmly established. To know its prevalence and characterization, we reviewed 549 consecutive autopsied brains in our Department. The age of death was 60 years or older in 391 subjects. Immunohistochemical staining with ubiquitin antibodies facilitated the identification of LB. Their specific density was measured (number per 100xfield) following a protocol in the predilection neocortical sites, entorhinal cortex, hippocampal gyrus, diencephalon and brainstem. We assessed the clinical features according to LB findings. Twelve brains (2.1%) had neocortical LB. Nine of them where diagnosed as diffuse Lewy body disease (DLBD). One more brain had nigral and neocortical LB leading to a pathological diagnosis of PD. In the remaining 2 cases, the finding of neocortical LB seems to be either incidental or asymptomatic or preclinical. Cognitive decline was mild to moderate in all subjects wich had neocortical LB in 4 or more areas. However, the density of these LB does not correlate with the severity of dementia. Dementia was associated with minor parkinsonian symptoms and psichiatric features in the most of patients with DLBD. Thirty eight cases of the 391 (9.7%) older than 60 years in these series had been clinically diagnosed as senile dementia. Ussing accepted neuropathologic criteria, diagnoses were AD (63.1%), DLBD (21.05%) and vascular dementia (13.1%). These observation suggest that on consultant diagnosis of senile dementia, DLBD must be taken in account.