Welcome to the WCN 2021 Interactive Program
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Please note that all sessions will run at their scheduled time and be followed by a LIVE Q&A/Discussion at the end
The viewing of sessions, cannot be accessed from this conference calendar. All sessions are accessible via the Virtual Platform
- Masud Husain (United Kingdom)
THE MOTOR SIDE OF THE FRONTOTEMPORAL DEMENTIA SPECTRUM
- James B. Rowe (United Kingdom)
Abstract
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Frontotemporal dementias (FTD) represent a family of disorders, affecting primarily behaviour and language. However, movement disorders are common in behavioural variant (bvFTD) and primary progressive aphasia (PPA) variants. In this talk, I will show that movement disorders are not restricted to those with the historic "Frontotemporal dementia with parkinsonism-17", but occur in up to half of patients with FTD during the course of their illness, whether the FTD's underlying pathology is Tau or TDP43 related (except TDP43-typeC associated with semantic dementia/svPPA). Movement disorders occur in sporadic and genetic forms. Mutations of GRN, C9orf72 and MAPT may present with a movement disorder before, concurrent, or after presentation of dementia. The movement disorder may include akinetic-rigidity (axial, appendicular, or facial), gait disorder, poor postural reflexes, dystonia, tremor and oculomotor impairments. Myoclonus is less common. They are poorly responsive to levodopa, despite the striatal dopamine depletion. The behavioural variant of FTD may also overlap clinically with motor neuron disease (MND, amyotrophic lateral sclerosis ALS). FTD is usually the result of one of the frontotemporal lobar degeneration pathologies (FTLD), which also include progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). Indeed, movement disorders associated with FTD have clinical features that resemble PSP and corticobasal syndrome rather than Parkinson's disease. This is reflected in diagnostic inter-states like "PSP-frontal" and "CBS-frontobehavioural". With myriad overlap phenotypes, evolving symptoms over time, and inherently poor clinico-pathological correlations, a dimensional approach to understand the motor side of FTD is most useful. Recognition of the movement disorder helps proactive clinical management, and risk reduction, as well as new disease modifying strategies.
THE COGNITIVE SIDE OF ALS
- Stefano F. Cappa (Italy)
Abstract
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The presence of cognitive and/or behavioral impairment, ranging from frontotemporal dementia (FTD) to selective neuropsychological dysfunction, in now established, and has been shown to have prognostic impact.The diagnosis is now defined on the basis of the revised diagnostic criteria proposed by Strong et al (Amyotrophic lateral sclerosis and frontotemporal degeneration 18,153-174, 2017), which emphasize the impact of disorders of executive function, but acknowledge the possible presence of "mixed" patters of cognitive impairment. This is confirmed by the results of studies using data-driven approaches, which have indicated the presence of three main test clusters, two compatible with the behavioral (ALSbi) and cognitive (ALSci) profiles of Strong et al. criteria, and a third loading on social cognition, language and memory tests, representing a distinct pattern of non-motor manifestations in a subsample of patients. The latter pattern of cognitive dysfunction is of particular interest, as disorders of social cognition and language are the typical markers of, respectively, the behavioral and language variants of FTD, and are associated to the neurodegenerative involvement of the large scale networks specifically associated with these function. Magnetic resonance studies have confirmed the correlation of performance on social cognition test wth atrophy measures in the anterior cingulate cortex and right inferior frontal gyrus, and of language impairment with left temporal pole and insular involvement in ALS. Further studies on large patient populations are ongoing to confim the existence of consistent cognitive phenotype differences and their assoiciation with additional genetic, clinical and prognostic factors.
COGNITION IN HUNTINGTON’S DISEASE
- Emre Bora (Turkey)
Abstract
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Cognitive impairment is a well-known feature of Huntington’s disease (HD). Generalized cognitive impairment is evident in various stages of HD including prodromal period of the disease. Cognitive and behavioral symptoms emerge many years before the onset of motor symptoms and are associated with functional decline in HD. Cognitive deficits in HD are widespread including abnormalities in processing speed, memory and executive functions. A substantial subset of patients with HD met criteria for mild cognitive impairment (MCI) in pre-HD stage. Nonamnestic type of MCI is more common than amnestic type MCI in HD. In recent years, studies investigating social cognitive deficits and empathy in HD have increased. Emotion recognition and theory of mind (ToM) abilities are impaired in both manifest and presymptomatic phase of the disease. Other aspects of social cognition have been investigated less often. Current talk discuss the evidence regarding specificity and predominance of particular aspects of neurocognitive and social cognitive deficits in HD. Neuroanatomic and biological correlates of cognitive and social cognitive impairment in HD is also discussed.