scholarly journals Cerebellar contributions to cognition in corticobasal syndrome and progressive supranuclear palsy

2020 ◽  
Vol 2 (2) ◽  
Author(s):  
Nga Yan Tse ◽  
Yu Chen ◽  
Muireann Irish ◽  
Nicholas J Cordato ◽  
Ramon Landin-Romero ◽  
...  

Abstract Mounting evidence suggests an association between cerebellar atrophy and cognitive impairment in the main frontotemporal dementia syndromes. In contrast, whether cerebellar atrophy is present in the motor syndromes associated with frontotemporal lobar degeneration (corticobasal syndrome and progressive supranuclear palsy) and the extent of its contribution to their cognitive profile remain poorly understood. The current study aimed to comprehensively chart profiles of cognitive impairment in relation to cerebellar atrophy in 49 dementia patients (corticobasal syndrome = 33; progressive supranuclear palsy = 16) compared to 33 age-, sex- and education-matched healthy controls. Relative to controls, corticobasal syndrome and progressive supranuclear palsy patients demonstrated characteristic cognitive impairment, spanning the majority of cognitive domains including attention and processing speed, language, working memory, and executive function with relative preservation of verbal and nonverbal memory. Voxel-based morphometry analysis revealed largely overlapping patterns of cerebellar atrophy in corticobasal syndrome and progressive supranuclear palsy relative to controls, primarily involving bilateral Crus II extending into adjacent lobules VIIb and VIIIa. After controlling for overall cerebral atrophy and disease duration, exploratory voxel-wise general linear model analysis revealed distinct cerebellar subregions differentially implicated across cognitive domains in each patient group. In corticobasal syndrome, reduction in grey matter intensity in the left Crus I was significantly correlated with executive dysfunction. In progressive supranuclear palsy, integrity of the vermis and adjacent right lobules I–IV was significantly associated with language performance. These results are consistent with the well-established role of Crus I in executive functions and provide further supporting evidence for vermal involvement in cognitive processing. The current study presents the first detailed exploration of the role of cerebellar atrophy in cognitive deficits in corticobasal syndrome and progressive supranuclear palsy, offering insights into the cerebellum’s contribution to cognitive processing even in neurodegenerative syndromes characterized by motor impairment.

1996 ◽  
Vol 2 (4) ◽  
pp. 359-367 ◽  
Author(s):  
K.J. Meador ◽  
D.W. Loring ◽  
K.D. Sethi ◽  
F. Yaghmai ◽  
S.D. Styren ◽  
...  

AbstractAlthough the dorsal midbrain has been implicated in cognitive processes in animals, its role in humans is unclear. We report the neuropsychological and postmortem neuropathological findings of a 52-yr-old university professor who developed a profound dementia in association with a focal dorsal midbrain lesion. The patient's disorder appeared to result from a tuberculous granuloma based on the clinical course and autopsy results. Neuropsychologically, he exhibited a generalized impairment across most of the cognitive domains assessed. His deficits were not explained by impaired arousal, specific sensory or motor defects, depression, or hydrocephalus. Although there are inherent limitations to a single-case investigation, our observations are consistent with animal studies that have demonstrated that focal dorsal midbrain lesions may result in cognitive impairment. We propose that the dorsal midbrain is involved in cognitive processing via modulation of thalamocortical networks. (JINS, 1996, 2, 359–367.)


2020 ◽  
Vol 11 (5-6) ◽  
pp. 309-314
Author(s):  
Veronica Arora ◽  
Sunita Bijarnia-Mahay ◽  
Sudhisha Dubey ◽  
Renu Saxena

Peroxisomal disorders are a heterogeneous group of inborn errors of metabolism that result in impaired function of the peroxisome. Within this, single enzyme deficiencies are known to cause a constellation of symptoms not very different from the peroxisome biogenesis defects. Thus, there is a need to identify features that differentiate the two. We present 3 molecularly confirmed families: 1 with Acyl CoA oxidase deficiency and 2 with D-bifunctional protein deficiency. The clinical, biochemical, and radiological features of these patients have been discussed. We attempt to highlight the overlap in facial features as well as strikingly similar MRI findings of cerebellar atrophy and white matter hyperintensities. This unique clinical profile will not only help in reaching a quick diagnosis, but in this era of variants of uncertain significance, it will prove as supporting evidence. Finally, we expand the genotypic spectrum with a description of 3 homozygous novel mutations (<i>HSD17B4</i>: c.670C&#x3e;T, c.1807T&#x3e;C; <i>ACOX1</i>: 1.03-kb exonic deletion) and discuss the role of protein modeling its establishing pathogenicity.


2009 ◽  
Vol 24 (S1) ◽  
pp. 1-1 ◽  
Author(s):  
F. Moretti ◽  
A.R. Atti ◽  
S. Cesano ◽  
V. Morini ◽  
C. Forlani ◽  
...  

Aims:To evaluate the prevalence of MCI and its subtypes in the Faenza Project, using the Mini-Mental State Examination (MMSE).Methods:Subjects living in the municipality of Faenza were clinically assessed. The diagnosis of MCI was achieved according to international criteria using a double-step procedure: first, we excluded subjects with dementia, functional impairment and MMSE< 24, leaving 6,745 persons. Then, the performance in different cognitive domains was considered and scores less than 1.5 standard deviations below the age- and education-adjusted norms on MMSE were settled as cut-offs. Four subtypes were identified: amnestic MCI, single non-memory MCI, multidomains non-amnestic, multidomains amnestic MCI.Results:2,720 (40.3%) participants met MCI criteria. Prevalence were 4% (amnestic MCI), 26.5% (single non-memory MCI), 4.4% (multidomains amnestic MCI), and 5.4% (multidomains non-amnestic MCI).Conclusions:MCI is common among Italian elderly. In our study prevalence are higher than reported in other population-based studies. The use of MMSE with different operational criteria, diverse sampling and assessment procedure might explain such discrepancy. The role of MMSE to identify persons with mild cognitive deficits deserves concerns. However, MMSE is largely used in both clinical practice and research and has been suggested as a useful screening tool for assessing the degree of cognitive impairment in the guidelines for the Report of the Quality Standards Subcommittee of the American Academy of Neurology.


2015 ◽  
Vol 40 (3-4) ◽  
pp. 130-136 ◽  
Author(s):  
Zhaolu Wang ◽  
Adrian Wong ◽  
Wenyan Liu ◽  
Jie Yang ◽  
Winnie C.W. Chu ◽  
...  

Background: We explored the association between cerebral microbleeds (CMBs) and cognitive impairment in patients with ischemic stroke/transient ischemic attack (TIA). Methods: A total of 488 ischemic stroke/TIA patients received magnetic resonance imaging. Montreal Cognitive Assessment (MoCA) was used to evaluate global cognitive function and cognitive domains. The association of CMB quantity with cognitive function and the impact of CMB locations (strictly lobar, strictly deep, and mixed regions) on cognitive impairment were examined in regression models with adjustments for confounders. Results: A total of 113 subjects (23.2%) had ≥1 CMB. Strictly lobar, strictly deep, and mixed CMBs were identified in 36, 40, and 37 patients, respectively. The presence of ≥5 CMBs or strictly deep CMBs was associated with the MoCA total score (p = 0.007 and 0.020, respectively). Of all MoCA domains tested, a lower score in the attention domain was related to the presence of ≥5 CMBs (p = 0.014) and strictly deep CMBs (p = 0.028). Conclusion: CMBs were associated with cognitive dysfunction in stroke/TIA patients, especially in the attention domain. This association was mainly driven by CMBs in the deep region, underlining the role of hypertensive microangiopathy in stroke-related cognitive impairment.


2017 ◽  
Vol 3 (1) ◽  
Author(s):  
Christopher R. Madan

A growing body of literature has demonstrated that motivation influences cognitive processing. The breadth of these effects is extensive and span influences of reward, emotion, and other motivational processes across all cognitive domains. As examples, this scope includes studies of emotional memory, value-based attentional capture, emotion effects on semantic processing, reward-related biases in decision making, and the role of approach/avoidance motivation on cognitive scope. Additionally, other less common forms of motivation–cognition interactions, such as self-referential and motoric processing can also be considered instances of motivated cognition. Here I outline some of the evidence indicating the generality and pervasiveness of these motivation influences on cognition, and introduce the associated ‘research nexus’ at Collabra: Psychology.


2007 ◽  
Author(s):  
Elizabeth Ranslow ◽  
Kim Lyon-Pratt ◽  
Amanda Ferrier ◽  
Katharine Elliott ◽  
Alexandra Macdonald ◽  
...  

Author(s):  
Mariia Matveeva ◽  
Julia Samoilova ◽  
Natalie Zhukova

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