RESUMO
BACKGROUND AND PURPOSE: Essential Tremor (ET) is among the most prevalent neurologic disorders. Growing clinical and neuro-imaging evidence implicates cerebellar dysfunction in the pathogenesis of ET and emerging postmortem studies have identified structural changes in the cerebellum, particularly in Purkinje cells. In this study we systematically quantified focal Purkinje cell dendritic swellings (DS) in 20 ET vs. 19 control brains. METHODS: In each brain, a standard parasagittal neocerebellar tissue block was harvested. DS were quantified in one 7-µm thick section stained with Luxol Fast Blue/Hematoxylin and Eosin (LH&E) and one section stained with Bielschowsky method. RESULTS: The number of DS were higher in cases than controls by LH&E (1.50 ± 1.79 vs. 0.05 ± 0.23, P = 0.002) and Bielschowsky methods (2.70 ± 3.10 vs. 0.37 ± 0.50, P = 0.002). The number of DS was correlated with the number of torpedoes and marginally inversely correlated with the number of Purkinje cells. CONCLUSION: The current study documents and quantifies an additional structural abnormality in the ET cerebellum, adding to the growing list of such changes in this disease. The mechanisms that underlie this and other structural changes observed in ET are currently unknown, and they deserve additional exploration.
Assuntos
Edema Encefálico/etiologia , Edema Encefálico/patologia , Córtex Cerebelar/patologia , Dendritos/patologia , Tremor Essencial/complicações , Células de Purkinje/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Contagem de Células , Criança , Pré-Escolar , Tremor Essencial/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Coloração e Rotulagem , Estatísticas não Paramétricas , Adulto JovemAssuntos
Doença de Alzheimer/genética , Doença de Alzheimer/patologia , Variação Genética/genética , Proteínas Relacionadas a Receptor de LDL/genética , Proteínas de Membrana Transportadoras/genética , Idoso , Idoso de 80 Anos ou mais , Autopsia , Estudos de Coortes , Feminino , Humanos , Masculino , Polimorfismo de Nucleotídeo Único/genética , Estudos ProspectivosRESUMO
Pathologic findings, including cerebellar changes and brainstem Lewy bodies, distinguished 10 essential tremor (ET) cases from 12 controls. Numbers of torpedoes (p = 0.009) and Bergmann glia (p = 0.046) were increased in cases. Six cases (60%) had Lewy bodies vs 2 controls (16.7%) (odds ratio 7.5, 95% CI 1.04 to 54.1; p = 0.035). Four of these six had an atypical distribution of brainstem Lewy bodies. ET may be pathologically heterogeneous.
