Progressive cerebral atrophy in multiple system atrophy

Konagaya, Masaaki; Konagaya, Yoko; Sakai, Motoko; Matsuoka, Yukihiko; Hashizume, Yoshio

doi:10.1016/s0022-510x(01)00692-x

Cited by 53 publications

(36 citation statements)

References 21 publications

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“…In PD, involvement of the cerebral hemisphere in general (and the temporal and frontal lobes in particular) has been reported. 6 Temporal lobe atrophy was also previously reported by using VBM analysis in non-demented 39,40 and demented 39,41,42 PD patients and in non-demented subjects with mild cognitive impairment. 42 Moreover, another VBM study described occipital lobe atrophy in demented PD patients.…”

Section: Discussionmentioning

confidence: 57%

“…4,5 In conventional magnetic resonance (MR)-based morphometric studies, macrostructural abnormalities in the cerebral cortex have been observed in the frontal and temporal lobes. 6 Although pyramidal dysfunction has been frequently observed in patients with MSA, 1,7 signs of pyramidal tract degeneration were only seen indistinctly on T 2 -weighted MR images. 8 Primary motor cortex (PMC) involvement in patients with clinically probable MSA was also reported by using magnetization transfer imaging.…”

Section: Introductionmentioning

confidence: 99%

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Motor‐related circuit dysfunction in MSA‐P: Usefulness of combined whole‐brain imaging analysis

et al. 2009

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The aim of this study was to evaluate in vivo changes in the brain's macro- and microstructure (notably in the motor system) in the parkinsonian variant of multiple system atrophy (MSA-P) and in Parkinson's disease (PD) and to characterize the cerebral anatomical differences between the two conditions. We used a combination of voxel-based morphometry (VBM) and whole-brain, voxel-based diffusion tensor imaging analysis (VB-DTI). Forty-seven right-handed subjects (14 MSA-P patients, 19 PD patients, and 14 controls) were evaluated using VBM and VB-DTI in an analysis of covariance (ANCOVA) with a significance threshold set to P < 0.005. In MSA-P patients, VBM analysis revealed a lower density of grey matter (GM) in a motor-related circuit (especially in the left primary motor cortex, PMC), relative to PD patients, and in the left supplementary motor area (SMA), relative to controls). Diffusion tensor imaging analysis revealed lower fractional anisotropy (FA) values in the left PMC and the right cerebellum in MSA-P patients, compared with controls. Using a volumetric diffusion technique, our study revealed selective tissue degeneration in motor circuits, regardless of the volume loss detected in VBM and in agreement with pathology reports and clinical motor characteristics. Our findings suggest that MSA-P is characterized by both macro- and microstructural changes in the sensorimotor circuit.

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Section: Discussionmentioning

confidence: 57%

Section: Introductionmentioning

confidence: 99%

Motor‐related circuit dysfunction in MSA‐P: Usefulness of combined whole‐brain imaging analysis

et al. 2009

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“…Pathological studies evaluating neuronal morphology in detail and distribution of GCIs have provided evidence of cortical involvement especially in the frontal cortex and its associated white matter as well as in the striatum of patients with MSA, suggesting that GCIs in these areas may contribute to cognitive deficits [15,24]. Recent longitudinal and voxel-based MRI studies additionally supported the notion of cortical involvement in patients with MSA, demonstrating widespread cortical atrophy of the frontal, parietal, and insular areas [3,14,21]. We also reported that in a large series of patients with MSA-C, there were widespread areas of decreased fluorodeoxyglucose in the cerebral cortex, including the dorsolateral frontal, orbitofrontal, medial frontal, and parietal regions.…”

Section: Discussionmentioning

confidence: 95%

White matter hyperintensities in patients with multiple system atrophy

et al. 2009

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Recent studies have reported that the majority of patients with multiple system atrophy (MSA) had hypertensive heart disease. However, the effect of autonomic failure on the brain in MSA has not been studied. We consecutively enrolled 63 patients with MSA and selected 63 age-and sex-matched healthy subjects. We performed a comparative analysis of cerebrovascular lesions between the patients with MSA and the control subjects and analyzed predisposing factors for cerebrovascular lesions in the patients with MSA. There was no significant difference in lacune and territorial infarcts between the patients with MSA and the control subjects. The median grading score of white matter hyperintensity (WMH) was significantly higher in the patients with MSA (1.0, interquartile range 0.5-2.0) than the control subjects (0.0, interquartile range 0.0-1.0; P \ 0.01). In the patients with MSA, there was strong correlation between the grading score of WMH and supine systolic blood pressure (r = 0.529, P \ 0.001) after adjusting for age. Multiple linear regression analysis showed that age and supine systolic blood pressure was significantly and independently correlated with the grading score of WMH. The present study demonstrates that patients with MSA had more severe WMH and that supine systolic pressure is a major contributing factor for the severity of WMH, suggesting that patients with MSA have target-organ damage of the brain.

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“…In recent years, some cases of MSA were reported to have cerebral cortical atrophy with white matter involvement [100,101,102,103,104]. The main pathological change comprised cell loss and gliosis in the putamen, substantia nigra, locus ceruleus, inferior olives, pontine nuclei, cerebellar Purkinje cells, and intermediolateral cell columns of the spinal cord [105].…”

Section: Multiple System Atrophymentioning

confidence: 99%

Cognitive Impairment in Spinocerebellar Degeneration

et al. 2009

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It has been reported that patients with spinocerebellar degenerations (SCDs) have cognitive dysfunction as well as limb and truncal ataxia, dysarthria and dysphagia. We review cognitive dysfunction in common types of SCD, including spinocerebellar ataxia types 1, 2, 3, 6, and 17, dentatorubral-pallidoluysian atrophy, Friedreich’s ataxia, and multiple system atrophy. There are few studies that address cognitive function in SCD. Although there are few comparison studies among the various SCDs, cognitive dysfunction may be more common and severe in spinocerebellar ataxia type 17 and dentatorubral-pallidoluysian atrophy. While cognitive dysfunction in SCD appears to represent frontal dysfunction, the mechanisms of cognitive dysfunction have not been directly clarified. Nevertheless, various lesions, including those in the cerebrocerebellar circuitry, cortico-striatal-thalamocortical circuitry, and the frontal lobe, may influence cognitive function to various degrees for each disease.

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Progressive cerebral atrophy in multiple system atrophy

Cited by 53 publications

References 21 publications

Motor‐related circuit dysfunction in MSA‐P: Usefulness of combined whole‐brain imaging analysis

Motor‐related circuit dysfunction in MSA‐P: Usefulness of combined whole‐brain imaging analysis

White matter hyperintensities in patients with multiple system atrophy

Cognitive Impairment in Spinocerebellar Degeneration

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