Brain Single-Photon Emission Computed Tomography and Magnetic Resonance Imaging in Machado-Joseph Disease

Etchebehere, Elba; Cendes, Fernando; Lopes‐Cendes, Íscia; Pereira, Juliana A.; Lima, Mariana; Sansana, Carla R.; Silva, Cleide A. M.; Camargo, Maria F. A. G.; Santos, Allan O.; Ramos, Celso Darío; Camargo, Edwaldo E.

doi:10.1001/archneur.58.8.1257

Cited by 54 publications

(36 citation statements)

References 22 publications

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“…As in SCD patients, however, anti-TPO Abs were not detected in the CSF of the patients in the present study. Moreover, 5 of the 10 patients in the present series showed reduced cerebellar blood perfusion on SPECT imaging, which is also consistent with findings in SCD patients [23,24]. Accordingly, care should be taken not to overlook treatable HE, even in patients with clinical histories and neurological signs mimicking SCD.…”

Section: Discussionsupporting

confidence: 90%

Hashimoto’s Encephalopathy as a Treatable Adult-Onset Cerebellar Ataxia Mimicking Spinocerebellar Degeneration

et al. 2012

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Background: Hashimoto’s encephalopathy (HE) presents with a variety of neurologic and neuropsychiatric features. In this study, we investigated the clinical and immunological profiles of the cerebellar ataxic form of HE. Methods: The clinical features, treatments, laboratory features, brain imaging, and serum anti-NH₂-terminal of α-enolase autoantibodies (anti-NAE Abs), a useful diagnostic marker for HE, were investigated in 13 patients who presented with sporadic adult-onset cerebellar ataxia and fulfilled the HE diagnostic criteria (antithyroid Abs and responsiveness to immunotherapy). Results: All of the patients presented with truncal ataxia, but nystagmus was uncommon (17%). Eight patients had an insidious onset that mimicked spinocerebellar degeneration (SCD), but brain imaging showed little or no cerebellar atrophy in all of the patients. Those patients with serum anti-NAE Abs (n = 8) did not have nystagmus and tended to respond better to immunotherapy than the anti-NAE Ab-negative patients. Conclusion: The present study suggests that insidious adult-onset and truncal ataxia are common in the cerebellar ataxic form of HE, which mimics SCD, but that nystagmus and severe cerebellar atrophy are uncommon. Antithyroid and anti-NAE Abs may be useful for diagnosing cerebellar ataxic HE.

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Section: Discussionsupporting

confidence: 90%

Hashimoto’s Encephalopathy as a Treatable Adult-Onset Cerebellar Ataxia Mimicking Spinocerebellar Degeneration

et al. 2012

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“…These lesions, newly recognized by polyglutamine immunohistochemistry, may be responsible for the cerebral cortical dysfunctions in SCA3 patients. Consistent with the results of this pathological study, the regional cerebral glucose metabolism or perfusion in SCA3, studied with PET or SPECT, was found to be significantly decreased in the entire cerebral cortex [41], occipital cortex [42], frontal, temporal and parietal lobes [43] aswell as in the cerebellar hemispheres, vermis, and brain stem. Murata et al [44] found similar results using MRI; not only were the widths of the superior cerebellar peduncles reduced and the transverse diameters of the globus pallidus and pons diminished, but the frontal and temporal lobes were also atrophied.…”

Section: Spinocerebellar Ataxia Type 3 (Machado-joseph Disease)supporting

confidence: 86%

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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“…and spinocerebellar ataxia type 3 (SCA3), MRI generally shows brainstem and cerebellar atrophy while SPECT shows reduced blood perfusion in the same area (1,2). On the other hand, atrophy and reduced perfusion of the brainstem are usually not apparent in cases of cortical cerebellar atrophy (CCA) and spinocerebellar ataxia type 6 (SCA6), although the cerebellum is atrophic and exhibits reduced perfusion (3).…”

Section: The Diagnosis and Classification Of Cerebellar Atrophy Requimentioning

confidence: 99%

Classification of Cerebellar Atrophy Using Voxel-based Morphometry and SPECT with an Easy Z-score Imaging System

et al. 2010

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Brain Single-Photon Emission Computed Tomography and Magnetic Resonance Imaging in Machado-Joseph Disease

Abstract: Brain SPECT imaging and MRI were capable of identifying subclinical abnormalities in individuals with MJD. These findings may be helpful for a better understanding of the pathophysiology of this disease.

Cited by 54 publications

References 22 publications

Hashimoto’s Encephalopathy as a Treatable Adult-Onset Cerebellar Ataxia Mimicking Spinocerebellar Degeneration

Hashimoto’s Encephalopathy as a Treatable Adult-Onset Cerebellar Ataxia Mimicking Spinocerebellar Degeneration

Cognitive Impairment in Spinocerebellar Degeneration

Classification of Cerebellar Atrophy Using Voxel-based Morphometry and SPECT with an Easy Z-score Imaging System

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