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Related Experiment Video

Updated: Feb 2, 2026

A Quick Phenotypic Neurological Scoring System for Evaluating Disease Progression in the SOD1-G93A Mouse Model of ALS
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Inflammation in ALS/FTD pathogenesis.

Madelyn E McCauley1, Robert H Baloh2,3

  • 1Board of Governors Regenerative Medicine Institute, Los Angeles, USA.

Acta Neuropathologica
|November 23, 2018
PubMed
Summary
This summary is machine-generated.

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) share neuroinflammation, impacting glial cells and immune responses. Despite this, anti-inflammatory therapies have not improved ALS/FTD patient outcomes.

Keywords:
Amyotrophic lateral sclerosisFrontotemporal dementiaNeurodegenerationNeuroinflammation

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Area of Science:

  • Neuroimmunology
  • Neurodegenerative Diseases
  • Genetics

Background:

  • Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are linked neurodegenerative disorders with overlapping clinical, pathological, and genetic features.
  • Neuroinflammation, involving glial cells and innate immune responses, is a hallmark of end-stage ALS/FTD pathology and emerges early in disease progression.
  • Systemic immune alterations, including changes in circulating immune cells and cytokines, suggest an autoinflammatory state in ALS/FTD patients.

Purpose of the Study:

  • To review the characteristics of neuroinflammation and systemic immune changes in ALS/FTD.
  • To discuss the influence of ALS/FTD-associated gene mutations on immune cell function.
  • To explore the potential mechanisms linking genetic factors to the altered immune tone in ALS/FTD.

Main Methods:

  • Literature review of neuroinflammation in ALS/FTD.
  • Analysis of human imaging and rodent model studies on neuroinflammation.
  • Examination of genetic studies linking ALS/FTD genes to immune function.

Main Results:

  • Neuroinflammation is a consistent feature in ALS/FTD, present from early pathogenesis through end-stage pathology.
  • ALS/FTD patients exhibit altered peripheral immune cell populations and cytokine profiles, indicating systemic inflammation.
  • Mutations in genes like C9orf72, TBK1, and OPTN directly impact immune cell function, contributing to the observed immune dysregulation.

Conclusions:

  • Neuroinflammation and systemic immune alterations are integral to the ALS/FTD disease spectrum.
  • Genetic factors play a direct role in modulating immune responses in ALS/FTD.
  • Understanding these immune mechanisms is crucial for developing effective therapeutic strategies, despite past failures of anti-inflammatory approaches.