Artículos de revistas
Nonmotor And Extracerebellar Features In Machado-joseph Disease: A Review
Registro en:
Movement Disorders. , v. 28, n. 9, p. 1200 - 1208, 2013.
8853185
10.1002/mds.25513
2-s2.0-84883559735
Autor
Pedroso J.L.
Franca M.C.
Braga-Neto P.
D'Abreu A.
Saraiva-Pereira M.L.
Saute J.A.
Teive H.A.
Caramelli P.
Jardim L.B.
Lopes-Cendes I.
Barsottini O.G.P.
Institución
Resumen
Spinocerebellar ataxia type 3 or Machado-Joseph disease is the most common spinocerebellar ataxia worldwide, and the high frequency of nonmotor manifestations in Machado-Joseph disease demonstrates how variable is the clinical expression of this single genetic entity. Anatomical, physiological, clinical, and functional neuroimaging data reinforce the idea of a degenerative process involving extracerebellar regions of the nervous system in Machado-Joseph disease. Brain imaging and neuropathologic studies have revealed atrophy of the pons, basal ganglia, midbrain, medulla oblongata, multiple cranial nerve nuclei, and thalamus and of the frontal, parietal, temporal, occipital, and limbic lobes. This review provides relevant information about nonmotor manifestations and extracerebellar symptoms in Machado-Joseph disease. 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