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Related Concept Videos

Dementia01:30

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Dementia is a collective term for cognitive disorders primarily affecting memory, thinking, and reasoning. It is not a specific disease but a syndrome, with Alzheimer's disease being the most common cause, accounting for approximately 60-80% of cases. Other types include vascular dementia, Lewy body dementia, and frontotemporal dementia. Dementia affects millions worldwide, particularly older adults, though it is not a normal part of aging.
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Alzheimer's Disease (AD), a neurodegenerative disorder, is pathologically identified by amyloid plaques and neurofibrillary tangles composed of tau protein. AD pharmacotherapy aims to manage cognitive symptoms, delay disease progression, and treat behavioral symptoms. The treatment is primarily symptomatic and palliative, with no definitive disease-modifying therapy available. Cholinesterase inhibitors, including donepezil (Aricept), rivastigmine (Exelon), and galantamine (Razadyne), are...
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Neurodegenerative disorders are progressive diseases that cause irreversible damage and loss to neurons in specific brain areas. Examples of these disorders include Parkinson's disease, Alzheimer's disease, Multiple Sclerosis (MS), and Amyotrophic Lateral Sclerosis (ALS). These disorders share characteristics such as proteinopathies, selective neuronal vulnerability, and a complex interplay between genetic and environmental factors. The primary therapeutic goal for these conditions is...
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Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
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Association areas are regions of the cerebral cortex that do not have a specific sensory or motor function. Instead, they integrate and interpret information from various sources to enable higher cognitive processes such as memory, learning, and decision-making. Some key association areas include the following:
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Updated: Mar 31, 2026

Eye-Tracking Control to Assess Cognitive Functions in Patients with Amyotrophic Lateral Sclerosis
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Frontotemporal Dysfunction and Dementia in Amyotrophic Lateral Sclerosis.

Susan C Woolley1, Michael J Strong2

  • 1Forbes Norris MDA/ALS Research Center, California Pacific Medical Center, 2324 Sacramento Street, Suite 111, San Francisco, CA 94115, USA.

Neurologic Clinics
|October 31, 2015
PubMed
Summary
This summary is machine-generated.

Amyotrophic lateral sclerosis (ALS) often involves frontotemporal dysfunction, impacting cognition and behavior in over half of patients. Stratifying ALS clinical trials by frontotemporal dysfunction presence is crucial for understanding disease progression.

Keywords:
Cognitive impairmentFrontotemporal dementiaFrontotemporal lobar degenerationNeural networkTDP-43TauTheory of mind

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Area of Science:

  • Neuroscience
  • Neurology
  • Clinical Medicine

Background:

  • Amyotrophic lateral sclerosis (ALS) is traditionally viewed as a motor neuron disease.
  • Emerging evidence suggests a significant overlap with frontotemporal dysfunction syndromes.

Observation:

  • Over 50% of ALS patients exhibit clinical, neuroimaging, or neuropathologic signs of frontotemporal dysfunction.
  • These dysfunctions manifest as frontotemporal dementia, behavioral changes, or cognitive impairments.

Findings:

  • Neuroimaging and neuropathology reveal frontotemporal lobar degeneration (FTLD) in affected ALS patients.
  • FTLD is associated with altered network connectivity within the brain.

Implications:

  • Frontotemporal dysfunction is a common comorbidity in ALS, influencing disease course.
  • Future ALS clinical trials should stratify participants based on the presence or absence of frontotemporal dysfunction for more accurate results.