Jaime Kulisevsky, Spain

Sant Pau Hospital - Universitat Autònoma de Barcelona Neurology
Prof. Dr. Jaime Kulisevsky is neurologist and Director of the Movement Disorders Unit at the Neurology Department of Sant Pau Hospital in Barcelona. He is Principal Investigator of the Parkinson’s Disease (PD) Area at the Spanish Biomedical Network Research Centre for Neurodegenerative Diseases (CIBERNED-Instituto de Salud Carlos III) and Full Professor of Neurology and Vice-Dean of the Faculty of Medicine at the Autonomous University of Barcelona. Among other national and international societies, he is an active member of the International Parkinson’s and Movement Disorders Society (MDS) being the present Treasurer Elect of the European Section of the MDS (ES-MDS), member of the Executive Committee of the European Huntington’s Disease Network (EHDN) and coordinator of the Cognitive Phenotype Working Group of the EHDN. The Movement Disorders Unit leaded by prof. Kulisevsky offers a multidisciplinary outpatient consultation for PD and other movement disorders and the research group runs several different lines on PD, parkinsonisms, tremor and HD, from molecular, to clinical and imaging studies and including functional neurosurgery. His main research and clinical interest focus on movement and basal ganglia disorders, on which he published more than 250 papers in peer reviewed journals and acts as a reviewer on various scientific journals. He also served as advisor for several non-profit organizations like the Michael J. Fox Foundation, the FDA or the EHDN, among others. He has been recipient of several public and private research grants and participated and coordinated numerous clinical trials in movement disorders. A particular research interest of Prof. Kulisevsky is on the cognitive and behavioral consequences of basal ganglia dysfunction, and on pharmacological management of movement disorders.

Moderator of 1 Session

LIVE SYMPOSIUM DISCUSSION

LIVE DISCUSSION - COGNITIVE DYSFUNCTIONS, PATHOLOGY AND TREATMENT APPROACHES IN PD, HD

Date
12.03.2021, Friday
Session Time
17:30 - 18:00
Session Icon
Live

Presenter of 2 Presentations

COGNITIVE ASPECTS OF HUNTINGTON'S DISEASE

Session Type
SYMPOSIUM
Date
12.03.2021, Friday
Session Time
12:00 - 14:00
Room
On Demand Symposia B
Lecture Time
13:00 - 13:15
Session Icon
On-Demand

Abstract

Abstract Body

Huntington’s disease (HD) has historically been conceptualized as eminently a subcortical dementia due to basal ganglia degeneration. Accordingly, the cognitive domains assumed to be prototypically affected in HD are frontal-executive functions, attention and psychomotor speed. However, compelling evidence proves that, from the early stages of the disease, the pattern of brain atrophy characterising HD extends beyond the basal ganglia and also involves posterior-cortical and temporal regions. Thus, in HD, accurate neuropsychological examination reveals a complex cognitive phenotype not limited to frontal-striatal functions.

In addition to frontal-executive, attention and psychomotor speed disturbances, deficits of different severity at level of visuomotor integration, episodic and autobiographical memory, visual perception, mental rotation, arithmetic reasoning, social cognition and language can be also detected decades before the diagnosis of the disease.

The time of presentation and the pattern of progression of cognitive changes in HD show a relation with the disease burden. However, a marked heterogeneity is also frequently observed between patients sharing equivalent CAG repeat length, education and age. It suggest, that additional mechanisms rather than those driven by disease burden may also contribute to the complex cognitive picture of HD. Ultimately; the progression of cognitive deterioration along HD always associates a significant impact on functional independence to the point of dementia.

Here we review the cognitive aspects of HD, emphasising on the neural substrates of general-to-specific cognitive changes, the affected cognitive domains and processes, the neuropsychological assessment approaches, and the possible mechanisms participating in the differential expression of cognitive deterioration in the HD population.

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