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Type: Capítulo de livro
Title: Neuroimaging In Spinocerebellar Ataxia Type 3: Clinical And Anatomical Correlates
Author: D'Abreu A.
Cendes F.
Lopes-Cendes I.
Abstract: Spinocerebellar ataxia 3 (SCA3) is a clinically heterogeneous neurodegenerative disorder characterized by ataxia, ophthalmoplegia, peripheral neuropathy, pyramidal dysfunction and movement disorders. It has an autosomal dominant inheritance and it results from a CAG repeat expansion mutation in the protein coding region of the ATXN3 gene located at chromosome 14q32. Early neuropathological and neuroimaging studies mostly concentrated on the cerebellum, brainstem, spinal cord and basal ganglia; however, recent observations have demonstrated a more widespread cerebral involvement in SCA3. Visual analysis usually displays atrophy of the pons, cerebellar peduncles, frontal and temporal lobes, globus pallidus, as well as decreased anteroposterior and transverse diameters of the midbrain and decrease anteroposterior diameter of the medulla oblongata. Brain SPECT showed perfusion abnormalities in the parietal lobes, inferior portion of the frontal lobes, mesial and lateral portions of the temporal lobes, basal ganglia, and cerebellar hemispheres and vermis, while 18F-Dopa uptake (PET) was significantly decreased in the cerebellum, brainstem and nigro-striatal dopaminergic system, cerebral cortex and the striatum. Magnetic resonance spectroscopy (MRS) of the deep white matter demonstrated changes suggestive of axonal dysfunction in normal appearing white matter. Voxel-based morphometry (VBM) studies have conflicting results probably reflecting differences in sample sizes and characteristics. Different studies using texture analysis, manual volumetry and VBM demonstrated thalamic involvement in SCA3. The objective of this review is to discuss neuroimaging findings in SCA3 and how they contribute to a better understanding of the disease pathophysiology and future research directions. © 2012 by Nova Science Publishers, Inc. All rights reserved.
Editor: Nova Science Publishers, Inc.
Rights: fechado
Identifier DOI: 
Date Issue: 2012
Appears in Collections:Unicamp - Artigos e Outros Documentos

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