Degree of Cerebellar Ataxia Correlates with Three-Dimensional MRI-Based Cerebellar Volume in Pure Cerebellar Degeneration

Richter, Stefanie; Dimitrova, Albena; Maschke, Matthias; Gizewski, Elke R.; Beck, Andreas; Aurich, Volker; Timmann, Dagmar

doi:10.1159/000087241

Cited by 54 publications

(29 citation statements)

References 18 publications

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“…Richter et al 27 found significant correlations between lower limb ataxia and cerebellar volume in a heterogeneous group of patients with pure cerebellar degeneration, also including 7 patients with SCA6. In our present study, we were not able to replicate this finding in a homogeneous SCA6 cohort.…”

Section: Discussionmentioning

confidence: 99%

Quantitative Assessment of Brain Stem and Cerebellar Atrophy in Spinocerebellar Ataxia Types 3 and 6: Impact on Clinical Status

Eichler¹,

Bellenberg²,

Hahn³

et al. 2011

AJNR Am J Neuroradiol

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Section: Discussionmentioning

confidence: 99%

Quantitative Assessment of Brain Stem and Cerebellar Atrophy in Spinocerebellar Ataxia Types 3 and 6: Impact on Clinical Status

Eichler¹,

Bellenberg²,

Hahn³

et al. 2011

AJNR Am J Neuroradiol

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“…We used conventional MRI-based volumetry to quantify the degree of atrophy of the cerebellum and its three longitudinal subdivisions in patients with cerebellar cortical degeneration. Total clinical ataxia rating scores showed a significant negative correlation with the volume of the entire cerebellum (normalised to the total intracranial volume) [12,13]. Oculomotor disorders were highly correlated with atrophy of the medial cerebellum.…”

Section: Somatotopy Motor Learningmentioning

confidence: 93%

Lesion-Symptom Mapping of the Human Cerebellum

et al. 2008

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High-resolution structural magnetic resonance imaging (MRI) has become a powerful tool in human cerebellar lesion studies. Structural MRI is helpful to analyse the localisation and extent of cerebellar lesions and to determine possible extracerebellar involvement. Functionally meaningful correlations between a cerebellar lesion site and behavioural data can be obtained both in subjects with degenerative as well as focal cerebellar disorders. In this review, examples are presented which demonstrate that MRI-based lesion-symptom mapping is helpful to study the function of cerebellar cortex and cerebellar nuclei. Behavioural measures were used which represent two main areas of cerebellar function, that is, motor coordination and motor learning. One example are correlations with clinical data which are in good accordance with the known functional compartmentalisation of the cerebellum in three sagittal zones: In patients with cerebellar cortical degeneration ataxia of stance and gait was correlated with atrophy of the medial (and intermediate) cerebellum, oculomotor disorders with the medial, dysarthria with the intermediate and limb ataxia with atrophy of the intermediate and lateral cerebellum. Similar findings were obtained in patients with focal lesions. In addition, in patients with acute focal lesions, a somatotopy in the superior cerebellar cortex was found which is in close relationship to animal data and functional MRI data in healthy control subjects. Finally, comparison of data in patients with acute and chronic focal lesions revealed that lesion site appears to be critical for motor recovery. Recovery after lesions to the nuclei of the cerebellum was less complete. Another example which extended knowledge about functional localisation within the cerebellum is classical conditioning of the eyeblink response, a simple form of motor learning. In healthy subjects, learning rate was related to the volume of the cortex of the posterior cerebellar lobe. In patients with focal cerebellar lesions, acquisition of eyeblink conditioning was significantly reduced in lesions including the cortex of the superior posterior lobe, but not the inferior posterior lobe. Disordered timing of conditioned eyeblink responses correlated with lesions of the anterior lobe. Findings are in good agreement with the animal literature. Different parts of the cerebellar cortex may be involved in acquisition and timing of conditioned eyeblink responses in humans. These examples demonstrate that MRI-based lesion-symptom mapping is helpful to study the contribution of functionally relevant cerebellar compartments in motor control and recovery in patients with cerebellar disease. In addition, information about the function of cerebellar cortex and nuclei can be gained.

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“…Pons, V2, total brain stem, hemispheres and total cerebellum correlated with ICARS. ICARS seems to be a valuable clinical parameter since it has been demonstrated a relevant inverse correlation between this scale and cerebellar atrophy 24 . An inverse correlation was found between ICARS and spinal cord area in MJD/SCA3 patients although with no statistical significance 25 .…”

Section: Icarsmentioning

confidence: 99%

Brain stem and cerebellum volumetric analysis of Machado Joseph disease patients

Camargos

Marques-Jr

Santos

2011

Arq. Neuro-Psiquiatr.

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Machado-Joseph disease, or spinocerebellar ataxia type 3(MJD/SCA3), is the most frequent late onset spinocerebellar ataxia and results from a CAG repeat expansion in the ataxin-3 gene. Previous studies have found correlation between atrophy of cerebellum and brainstem with age and CAG repeats, although no such correlation has been found with disease duration and clinical manifestations. In this study we test the hypothesis that atrophy of cerebellum and brainstem in MJD/SCA3 is related to clinical severity, disease duration and CAG repeat length as well as to other variables such as age and ICARS (International Cooperative Ataxia Rating Scale). Whole brain high resolution MRI and volumetric measurement with cranial volume normalization were obtained from 15 MJD/ SCA3 patients and 15 normal, age and sex-matched controls. We applied ICARS and compared the score with volumes and CAG number, disease duration and age. We found significant correlation of both brain stem and cerebellar atrophy with CAG repeat length, age, disease duration and degree of disability. The Spearman rank correlation was stronger with volumetric reduction of the cerebellum than with brain stem. Our data allow us to conclude that volumetric analysis might reveal progressive degeneration after disease onset, which in turn is linked to both age and number of CAG repeat expansions in SCA 3. Key words: CAG expansion, Machado-Joseph disease, MJD/SCA3, spinocerebellar ataxia, volumetry.Análise volumétrica de cerebelo e tronco cerebral de pacientes com doença de Machado Joseph RESUMO Doença de Machado-Joseph, ou ataxia espinocerebelar tipo 3 (MJD/SCA3) é ataxia espinocerebelar de início tardio mais frequente e resulta de uma expansão da repetição CAG no gene da ataxina-3. Estudos precedentes encontraram correlação entre a atrofia do cerebelo e do tronco cerebral com a idade e número de expansões CAG. Tais correlações não foram encontradas em relação ao tempo de doença ou manifestações clínicas. Neste estudo testamos a hipótese de que a atrofia do cerebelo e do tronco encefálico em MJD/SCA3 está relacionada à gravidade clínica, duração da doença e número de repetições CAG, bem como com outras variáveis como a idade e a ICARS (escala cooperativa internacional de avaliação de ataxias). Foram realizados estudos de imagem pela ressonância magnética de alta resolução e volumetria com normalização de volume craniano de 15 pacientes portadores de MJD/SCA3 e 15 controles pareados por idade e sexo. Nós aplicamos a ICARS e correlacionamos com o escore de volumes e número de CAG, duração da doença e idade. Encontramos correlação significativa entre atrofia de tronco cerebral e cerebelo com duração da doença, repetição CAG, idade e grau de acometimento da doença. O índice de correlação de Spearman foi maior em relação à atrofia de cerebelo do que à atrofia de tronco. Nossos dados permitem concluir que a análise volumétrica pode revelar degeneração progressiva após o início da doença que, por sua vez, está ligada à idade e número de expansões CAG em SCA 3...

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Degree of Cerebellar Ataxia Correlates with Three-Dimensional MRI-Based Cerebellar Volume in Pure Cerebellar Degeneration

Cited by 54 publications

References 18 publications

Quantitative Assessment of Brain Stem and Cerebellar Atrophy in Spinocerebellar Ataxia Types 3 and 6: Impact on Clinical Status

Quantitative Assessment of Brain Stem and Cerebellar Atrophy in Spinocerebellar Ataxia Types 3 and 6: Impact on Clinical Status

Lesion-Symptom Mapping of the Human Cerebellum

Brain stem and cerebellum volumetric analysis of Machado Joseph disease patients

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