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

Nanri, Kazunori; Koizumi, Kiyoshi; Mitoma, Hiroshi; Taguchi, Takeshi; Takeguchi, Masafumi; Ishiko, Tomoko; Otsuka, Takao; Nishioka, Hiroshi; Mizusawa, Hidehiro

doi:10.2169/internalmedicine.49.2785

Cited by 15 publications

(7 citation statements)

References 24 publications

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“…It has been reported that while the frequency of gait ataxia in patients with cerebellar ataxia with GAD-Ab was 95%, the frequency of dysarthria in those was 66% ( 4 ). In addition, this disease did not necessarily show a reduced perfusion in the cerebellar region by SPECT, such as was seen in the present case ( 17 ). These findings suggested the possibility that cases of cerebellar ataxia with GAD-Ab manifested more advanced dysfunction in cerebellar vermis than cerebellar hemisphere.…”

Section: Discussionsupporting

confidence: 56%

Acute Cerebellar Ataxia Associated with Anti-glutamic Acid Decarboxylase Antibodies Mimicking Miller Fisher Syndrome

et al. 2018

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We herein report the case of a 53-year-old man with cerebellar ataxia with anti-glutamic acid decarboxylase antibody (GAD-Ab) who mimicked Miller Fisher syndrome (MFS). He developed ophthalmoplegia, diplopia, and gait ataxia for one week. The serum and cerebrospinal fluid GAD-Ab titers were greatly increased, and the GAD-Ab index suggesting intrathecal antibody synthesis was elevated, while GQ1b-Ab was negative. After steroid pulse therapy and following prednisolone, his symptoms dramatically improved over the course of 11 months with the simultaneous decline of GAD-Ab titers. This case indicates that cerebellar ataxia with GAD-Ab can present with acute neurological findings mimicking MFS, and that steroid therapy has an excellent therapeutic effect.

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Section: Discussionsupporting

confidence: 56%

Acute Cerebellar Ataxia Associated with Anti-glutamic Acid Decarboxylase Antibodies Mimicking Miller Fisher Syndrome

et al. 2018

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“…To address whether these changes were localized across the vermis, we compared the cell density in anterior and posterior lobules in SCA6 84Q/84Q mice, and found no significant differences at 2 years (anterior, 3.7 ± 0.19 cells/100 μm; posterior, 4.2 ± 0.25 cells/100 μm; p = 0.51). Rather, we observed a reduced Purkinje cell density in both anterior and posterior lobules of SCA6 84Q/84Q mice at 2 years compared with age- and litter-matched WT mice (anterior: F (3,92) = 10.16; p < 0.0001; posterior: F (3,106) = 3.37; p = 0.02; data not shown), in contrast to the predominantly anterior Purkinje cell degeneration observed in some human SCA6 patients ( Gierga et al, 2009 ; Nanri et al, 2010 ).…”

Section: Resultsmentioning

confidence: 84%

“…7 A , B ), which is consistent with previous reports ( Watase et al, 2008 ). We wondered whether subtle changes in Purkinje cell numbers or morphology might be restricted to only part of the cerebellum, since Purkinje cell degeneration has been reported to be more prevalent in anterior lobules of cerebellar vermis in some human patients ( Gierga et al, 2009 ; Nanri et al, 2010 ). To address whether changes might be localized to subregions of the cerebellar vermis, we measured Purkinje cell density in anterior and posterior lobules, but observed no significant differences in both WT and SCA6 84Q/84Q mice (data not shown).…”

Section: Resultsmentioning

confidence: 99%

Rapid Onset of Motor Deficits in a Mouse Model of Spinocerebellar Ataxia Type 6 Precedes Late Cerebellar Degeneration

Jayabal

Ljungberg

Erwes

et al. 2015

eneuro

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Spinocerebellar ataxia type 6 (SCA6) is an autosomal-dominant cerebellar ataxia that has been associated with loss of cerebellar Purkinje cells. Disease onset is typically at midlife, although it can vary widely from late teens to old age in SCA6 patients. Our study focused on an SCA6 knock-in mouse model with a hyper-expanded (84X) CAG repeat expansion that displays midlife-onset motor deficits at ∼7 months old, reminiscent of midlife-onset symptoms in SCA6 patients, although a detailed phenotypic analysis of these mice has not yet been reported. Here, we characterize the onset of motor deficits in SCA684Q mice using a battery of behavioral assays to test for impairments in motor coordination, balance, and gait. We found that these mice performed normally on these assays up to and including at 6 months, but motor impairment was detected at 7 months with all motor coordination assays used, suggesting that motor deficits emerge rapidly during a narrow age window in SCA684Q mice. In contrast to what is seen in SCA6 patients, the decrease in motor coordination was observed without alterations in gait. No loss of cerebellar Purkinje cells or striatal neurons were observed at 7 months, the age at which motor deficits were first detected, but significant Purkinje cell loss was observed in 2-year-old SCA684Q mice, arguing that Purkinje cell death does not significantly contribute to the early stages of SCA6.

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“…SCA-6 is a slowly progressive, genetic neurodegenerative condition where damage is restricted to the Purkinje cells of the cerebellum (Zhuchenko et al, 1997). The degeneration is characterised by a stereotyped, dorso-ventral progression in the cerebellar cortex (Butteriss, Chinnery, & Burchall, 2005), with usually no significant white matter loss or degeneration in extracerebellar areas including cortical areas (Ishikawa et al, 1999; Lukas et al, 2006; Nanri et al, 2010; Schlos et al, 1998; Schulz et al, 2010; Zhuchenko et al, 1997). Interpretation of previous studies has been limited by the use of heterogeneous patient groups with lesions (especially strokes) in which the cerebellum is not affected in the same way across patients, and where extracerebellar damage may also occur.…”

Section: Introductionmentioning

confidence: 99%

Distinct critical cerebellar subregions for components of verbal working memory

et al. 2012

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A role for the cerebellum in cognition has been proposed based on studies suggesting a profile of cognitive deficits due to cerebellar stroke. Such studies are limited in the determination of the detailed organisation of cerebellar subregions that are critical for different aspects of cognition. In this study we examined the correlation between cognitive performance and cerebellar integrity in a specific degeneration of the cerebellar cortex: Spinocerebellar Ataxia type 6 (SCA6). The results demonstrate a critical relationship between verbal working memory and grey matter density in superior (bilateral lobules VI and crus I of lobule VII) and inferior (bilateral lobules VIIIa and VIIIb, and right lobule IX) parts of the cerebellum. We demonstrate that distinct cerebellar regions subserve different components of the prevalent psychological model for verbal working memory based on a phonological loop. The work confirms the involvement of the cerebellum in verbal working memory and defines specific subsystems for this within the cerebellum.

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

Abstract: Objective With conventional MRI and single-photon emission computed tomography (SPECT),

Cited by 15 publications

References 24 publications

Acute Cerebellar Ataxia Associated with Anti-glutamic Acid Decarboxylase Antibodies Mimicking Miller Fisher Syndrome

Acute Cerebellar Ataxia Associated with Anti-glutamic Acid Decarboxylase Antibodies Mimicking Miller Fisher Syndrome

Rapid Onset of Motor Deficits in a Mouse Model of Spinocerebellar Ataxia Type 6 Precedes Late Cerebellar Degeneration

Distinct critical cerebellar subregions for components of verbal working memory

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