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Updated: Dec 9, 2025

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

The NEALS primary lateral sclerosis registry.

Sabrina Paganoni¹, Fabiola De Marchi¹, James Chan²

¹Department of Neurology, Sean M. Healey & AMG Center for ALS at Mass General, Massachusetts General Hospital, Boston, MA, USA.

Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration

|September 11, 2020

Summary

This summary is machine-generated.

Primary lateral sclerosis (PLS) is a rare neurodegenerative disease. This study established the largest registry of PLS patients, detailing disease progression and functional decline for future research.

Keywords:

PLS disability outcome measures survival upper motor neurons

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Last Updated: Dec 9, 2025

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

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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

Area of Science:

Neurology
Neurodegenerative Diseases

Background:

Primary lateral sclerosis (PLS) is a rare neurodegenerative disorder affecting upper motor neurons.
Limited data exists on the natural history of PLS due to its low prevalence (1-4% of adult motor neuron diseases).

Purpose of the Study:

To establish a multicenter retrospective longitudinal registry of PLS patients.
To characterize the natural progression of primary lateral sclerosis.

Main Methods:

Collected clinical data, electrophysiological findings, lab values, symptoms, and medications from PLS patients (2000-2015).
Utilized data from 250 PLS patients across Northeast ALS Consortium (NEALS) sites.

Main Results:

Mean decline in ALSFRS-R score was -1.6 points/year.
Mean annual decline in vital capacity was -3%.
Observed mortality, feeding tube placement, and need for ventilation in a subset of patients.

Conclusions:

The NEALS PLS Registry is the largest collection of longitudinal PLS data.
Provides insights into expected disease progression for this rare condition.
Registry data will support future clinical trial planning for PLS.