Neuro-ophthalmic Manifestations of Cerebellar Disease

Beh, Shin C.; Frohman, Teresa C.; Frohman, Elliot M.

doi:10.1016/j.ncl.2014.07.002

Cited by 19 publications

(10 citation statements)

References 691 publications

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“…In addition, decreased GM volume in bilateral cerebellar lobules IX-X might explain some clinical symptoms in SAOA. The impairments of lobule IX and X are frequently reported as being associated with abnormal eye-movements (33,34). Previous lesions studies in lobule IX-X showed lobule IX lesions to cause nystagmus and impaired vestibular-ocular reflex suppression, while lobule X lesions typically result in downbeat fixation nystagmus (32).…”

Section: Discussionmentioning

confidence: 99%

Characterization of Cerebellar Atrophy and Resting State Functional Connectivity Patterns in Sporadic Adult-Onset Ataxia of Unknown Etiology (SAOA)

et al. 2019

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Background: Sporadic adult onset ataxia of unknown etiology (SAOA) is a non-genetic neurodegenerative disorder of the cerebellum of unknown cause which manifests with progressive ataxia without severe autonomic failure. Although SAOA is associated with cerebellar degeneration, little is known about the specific cerebellar atrophy pattern in SAOA.Methods: 37 SAOA patients and 49 healthy controls (HCs) were included at two centers. We investigated the structural and functional characteristics of SAOA brains using voxel based morphometry (VBM) and resting state functional imaging (rs-fMRI). In order to examine the functional consequence of structural cerebellar alterations, the amplitude of low frequency fluctuation (ALFF) and degree centrality (DC) were analyzed, and then assessed their relation with disease severity, disease duration, and age of onset within these regions. Group differences were investigated using two-sample t-tests, controlling for age, gender, site, and the total intracranial volume. Results:The VBM analysis revealed a significant, mostly bilateral reduction of local gray matter (GM) volume in lobules I-V, V, VI, IX, X and vermis VIIIa/b in SAOA patients, compared to HCs. The GM volume loss in these regions was significantly associated with disease severity, disease duration and age of onset. The disease-related atrophy regions did not show any functional alternations compared with HCs, but were functionally characterized by high ALFF and poor DC compared with intact cerebellar regions.Conclusions: Our data revealed volume reduction in SAOA in cerebellar regions that are known to be involved in motor and somatosensory processing, corresponding with the clinical phenotype of SAOA. Our data suggest that the atrophy occurs in those cerebellar regions which are characterized by high ALFF and poor DC. Further studies have to show if these findings are specific for SAOA, and if they can be used to predict disease progression.

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Section: Discussionmentioning

confidence: 99%

Characterization of Cerebellar Atrophy and Resting State Functional Connectivity Patterns in Sporadic Adult-Onset Ataxia of Unknown Etiology (SAOA)

et al. 2019

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“…Cerebellar control of eye movements ensures appropriate speed, precision, and reliability of vision rather than the actual movements [20]. Specifically, lesions of lobule IX cause nystagmus, impaired smooth pursuits, and impaired vestibular-ocular reflex suppression, gain, and direction.…”

Section: General Anatomy and Function Of The Cerebellummentioning

confidence: 99%

Neurodegeneration and the Cerebellum

Samson

Claassen²

2017

Neurodegener Dis

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Background: The cerebellum modulates diverse neuronal networks, regulating motor, cognitive, behavioral, and limbic circuits. Structural and functional changes to the cerebellum that occur with neurodegenerative conditions have not been systematically reviewed. Objective: We synthesize the current understanding of cerebellar somatotopy and function with previously described cerebellar changes in neurodegenerative diseases not associated with primary cerebellar pathology. Methods: A thorough literature review defines the role of the cerebellum in normal function and neurodegeneration, and we additionally provide exemplar cases of cerebellar dysfunction in neurodegenerative disorders. Results: Comparisons between normal function and neurodegenerative disease states emphasize how normal organization of the cerebellum is altered in neurodegenerative disease states. We illustrate key anatomic structures using novel cerebellar segmentation tools and images. Conclusions: Altered cerebellum in neurodegeneration can provide important diagnostic and pathologic insights into both predicting disease progression and network dysfunction.

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“…Cerebellar projections (shown in blue) fine-tune the saccades, given that cerebellar lesions can lead to saccadic dysmetria. The nucleus reticularis tegmenti pontis receives projections from the FEF and the superior colliculus (projection not shown) and in turn projects to the cerebellar ocular V. The Vinhibits the ipsilateral caudal fastigial nucleus, which then projects to the BGC to enhance saccades moving to the contralateral side and tamp down saccades moving to the ipsilateral side, likely via both inhibitory and excitatory connections [ 33 , 70 , 71 ].…”

mentioning

confidence: 99%

The King-Devick test of rapid number naming for concussion detection: meta-analysis and systematic review of the literature

et al. 2016

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Background:Vision encompasses a large component of the brain's pathways, yet is not represented in current sideline testing.Objectives:We performed a meta-analysis of published data for a vision-based test of rapid number naming (King-Devick [K-D] test).Studies & methods:Pooled and meta-analysis of 15 studies estimated preseason baseline K-D scores and sensitivity/specificity for identifying concussed versus nonconcussed control athletes.Result:Baseline K-D (n = 1419) showed a weighted estimate of 43.8 s (95% CI: 40.2, 47.5; I2 = 0.0%; p=0.85 – indicating very little heterogeneity). Sensitivity was 86% (96/112 concussed athletes had K-D worsening; 95% CI: 78%, 92%); specificity was 90% (181/202 controls had no worsening; 95% CI: 85%, 93%).Conclusion:Rapid number naming adds to sideline assessment and contributes a critical dimension of vision to sports-related concussion testing.

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Neuro-ophthalmic Manifestations of Cerebellar Disease

Cited by 19 publications

References 691 publications

Characterization of Cerebellar Atrophy and Resting State Functional Connectivity Patterns in Sporadic Adult-Onset Ataxia of Unknown Etiology (SAOA)

Characterization of Cerebellar Atrophy and Resting State Functional Connectivity Patterns in Sporadic Adult-Onset Ataxia of Unknown Etiology (SAOA)

Neurodegeneration and the Cerebellum

The King-Devick test of rapid number naming for concussion detection: meta-analysis and systematic review of the literature

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