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Related Experiment Video

Updated: Mar 31, 2026

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies

Published on: July 29, 2007

Primary Lateral Sclerosis.

Jeffrey M Statland¹, Richard J Barohn¹, Mazen M Dimachkie¹

¹Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Mailstop 2012, Kansas City, KS 66160, USA.

Neurologic Clinics

|October 31, 2015

Summary

This summary is machine-generated.

Primary lateral sclerosis (PLS) involves progressive upper motor neuron issues without lower motor neuron signs. Diagnosis relies on clinical findings and ruling out other causes, with supportive care being the main treatment.

Keywords:

Motor neuron disease Neuroimaging Primary lateral sclerosis Pseudobulbar affect Spastic quadriparesis Upper motor neuron disease

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

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

Published on: February 21, 2011

Area of Science:

Neurology
Neuroscience

Background:

Primary lateral sclerosis (PLS) is a rare, progressive neurological disorder.
It specifically affects upper motor neurons, leading to characteristic motor dysfunction.

Observation:

Patients present with insidious onset of stiffness, impaired balance, and mild weakness.
Bulbar involvement can cause dysphagia, dysarthria, and emotional lability.
Absence of lower motor neuron signs is a key diagnostic feature.

Findings:

Diagnosis is primarily clinical, supported by exclusion of other conditions.
Electromyography (EMG) typically shows normal or non-specific mild neurogenic findings.
Findings do not meet the El Escorial criteria for amyotrophic lateral sclerosis.

Implications:

Understanding PLS aids in accurate diagnosis and patient management.
Highlights the importance of differentiating PLS from other motor neuron diseases.
Emphasizes the current lack of disease-modifying treatments, necessitating supportive care strategies.