Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Secondary Spinal Cord Injury llI: Pathophysiology01:25

Secondary Spinal Cord Injury llI: Pathophysiology

Early Ischemia and Ionic ImbalanceWithin minutes of spinal cord injury, a secondary cascade begins, progressing over hours to weeks. Vascular damage reduces blood flow, causing ischemia and mitochondrial dysfunction. ATP depletion leads to ion pump failure, membrane depolarization, sodium influx, potassium efflux, and water accumulation, resulting in cellular swelling. Increased intracellular calcium further disrupts mitochondria and accelerates cellular injury.Excitotoxicity and Neuronal...
Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
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...
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 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...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Prospective Validation of the New PLS Diagnostic Criteria From PLS Natural History Study: EMG and Neurofilament Analyses.

Muscle & nerve·2026
Same author

Primary Lateral Sclerosis Natural History Study: Primary Lateral Sclerosis Functional Rating Scale and Other Outcomes Assessment.

Annals of neurology·2025
Same author

Prognostic relationship of neurofilaments, CHIT1, YKL-40 and MCP-1 in amyotrophic lateral sclerosis.

Journal of neurology, neurosurgery, and psychiatry·2021
Same author

Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis.

JAMA neurology·2021
Same author

Measuring disease progression in primary lateral sclerosis.

Amyotrophic lateral sclerosis & frontotemporal degeneration·2021
Same author

Clinical care and therapeutic trials in PLS.

Amyotrophic lateral sclerosis & frontotemporal degeneration·2021

Related Experiment Video

Updated: Jun 18, 2026

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
08:16

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis

Published on: March 4, 2014

Progression in primary lateral sclerosis: a prospective analysis.

Mary Kay Floeter1, Reversa Mills

  • 1Electromyography Section, National Institutes of Neurological Disorders and Stroke, 10 Center Drive, Bethesda, Maryland 20892, USA. floeterm@ninds.nih.gov

Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
|November 20, 2009
PubMed
Summary

Primary lateral sclerosis (PLS) progression varies by subtype, with faster declines when the disease spreads to new areas. Subtype classification aids in predicting disease spread but not severity progression.

More Related Videos

A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS
06:49

A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS

Published on: October 6, 2015

A Protocol for Comprehensive Assessment of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis (ALS)
12:43

A Protocol for Comprehensive Assessment of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis (ALS)

Published on: February 21, 2011

Related Experiment Videos

Last Updated: Jun 18, 2026

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
08:16

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis

Published on: March 4, 2014

A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS
06:49

A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS

Published on: October 6, 2015

A Protocol for Comprehensive Assessment of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis (ALS)
12:43

A Protocol for Comprehensive Assessment of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis (ALS)

Published on: February 21, 2011

Area of Science:

  • Neurology
  • Neurodegenerative Diseases

Background:

  • Primary Lateral Sclerosis (PLS) is a rare, adult-onset motor neuron disease.
  • Understanding PLS progression patterns is crucial for patient management and therapeutic development.

Purpose of the Study:

  • To investigate differences in progression rates and patterns among primary lateral sclerosis (PLS) subtypes.
  • To assess the predictive value of PLS subtypes for disease spread and severity.

Main Methods:

  • Fifty patients meeting PLS criteria were classified into ascending (PLS-A), multifocal (PLS-M), or sporadic paraparesis (PLS-SP) subtypes.
  • Annual surveys and clinical assessments, including movement speed, rating scales, and transcranial magnetic stimulation, were conducted over 1-5 years.
  • Evaluated spread to new body regions and severity progression within affected areas.

Main Results:

  • Forty-seven patients completed follow-up (mean 6.6 years), with a mean disease duration exceeding 14 years.
  • PLS-A patients exhibited more predictable progression to additional body regions.
  • Severity increased rapidly in newly affected regions, followed by stabilization in PLS-A and PLS-M subtypes.

Conclusions:

  • PLS progression is characterized by periods of accelerated decline upon spreading to new regions, rather than steady decline.
  • PLS subtype classification is valuable for predicting disease spread but not the rate of severity progression.