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Related Concept Videos

Parkinson's Disease: Overview01:15

Parkinson's Disease: Overview

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 to...
Parkinson Disease ll: Pathophysiology01:24

Parkinson Disease ll: Pathophysiology

Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
Alterations in Muscle Tone lll01:11

Alterations in Muscle Tone lll

Rigidity and myotonia are distinct abnormalities of muscle tone that affect resistance and relaxation during movement. Although both involve altered muscle contraction, they arise from different neurological and muscular mechanisms.CharacteristicsRigidity is characterized by uniform resistance to passive movement across the entire range, independent of speed, affecting flexors and extensors equally. It may appear as lead-pipe rigidity (smooth, constant resistance) or cogwheel rigidity...
Parkinson Disease l: Introduction01:24

Parkinson Disease l: Introduction

Parkinson’s disease is a chronic, progressive neurodegenerative disorder that primarily affects movement. It is characterized by motor symptoms such as resting tremors, muscle rigidity, bradykinesia (slowness of movement), and postural instability. Patients may notice hand tremors at rest, stiffness during movement, or a shuffling gait. In addition to motor features, non-motor symptoms include sleep disturbances, mood and behavioral changes, constipation, and cognitive impairment, all of which...
Alterations in Muscle Tone ll01:12

Alterations in Muscle Tone ll

Alterations in muscle tone are common manifestations of neurological disorders and reflect dysfunction within different nervous system regions. Spasticity, paratonia, and dystonia represent distinct forms of hypertonia, each with unique mechanisms, clinical features, and diagnostic importance.CharacteristicsSpasticity happens from upper motor neuron lesions and is characterized by velocity-dependent resistance to passive movement. Clinical features include:Exaggerated deep tendon reflexesClonus...
Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...

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Updated: May 15, 2026

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
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Published on: March 4, 2014

Functional impairment in progressive supranuclear palsy.

Kevin Duff1, Adam Gerstenecker, Irene Litvan

  • 1Department of Neurology, University of Utah, Salt Lake City, USA. kevin.duff@hsc.utah.edu

Neurology
|January 11, 2013
PubMed
Summary
This summary is machine-generated.

Patients with early-stage progressive supranuclear palsy (PSP) experience significant functional disability, primarily linked to motor symptoms rather than cognitive or psychiatric issues.

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Area of Science:

  • Neuroscience
  • Clinical Neurology

Background:

  • Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease.
  • Understanding functional impact in early stages is crucial for patient care and research.

Purpose of the Study:

  • To characterize the functional profiles of patients with early-stage PSP.
  • To investigate the relationship between functional status and motor, cognitive, and psychiatric symptoms.

Main Methods:

  • A prospective, multisite study included 202 individuals with clinically definite or probable PSP.
  • Three functional scales were assessed and compared with motor, cognitive, and psychiatric measures.

Main Results:

  • All patients exhibited functional disability, with no perfect scores on functional scales.
  • Functional impairment was strongly correlated with motor ratings.
  • Functional scores showed little relation to cognition or psychiatric symptoms.

Conclusions:

  • High functional disability is evident even in early-stage PSP.
  • The definition of functional impairment has significant clinical and research implications.
  • Further research is needed to validate current functional scales or develop new ones for PSP.