Related Experiment Video

Updated: May 24, 2026

Methods to Quantify Pharmacologically Induced Alterations in Motor Function in Human Incomplete SCI
14:55

Methods to Quantify Pharmacologically Induced Alterations in Motor Function in Human Incomplete SCI

Published on: April 18, 2011

A systematic review of behavioural changes in motor neuron disease

Joost Raaphorst1, Emma Beeldman, Marianne De Visser

  • 1Department of Neurology, Academic Medical Centre, University of Amsterdam, The Netherlands. j.raaphorst@amc.uva.nl

Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
|March 20, 2012
PubMed

Insights

Motor neuron disease (MND) and behavioral variant frontotemporal dementia (bvFTD) share a spectrum. Approximately 8% of MND patients exhibit bvFTD, often with memory issues and bulbar onset.

Area of Science:

  • Neurology
  • Neuroscience
  • Clinical Medicine

Background:

  • Motor neuron disease (MND) and behavioral variant frontotemporal dementia (bvFTD) are increasingly recognized as part of a shared disease spectrum.
  • Uncertainty exists regarding the prevalence and specific characteristics of behavioral changes in MND patients, as well as the association between bvFTD and MND onset location.

Purpose of the Study:

  • To systematically review existing literature on behavioral changes in MND.
  • To determine the prevalence of bvFTD and mild behavioral changes in MND cohorts.
  • To analyze the characteristics, pooled prevalences, and demographic variables of MND-bvFTD patients, including onset site and survival.

Main Methods:

  • Systematic literature search for articles detailing behavioral changes in MND patients.
  • Review of cohort studies to summarize the prevalence of bvFTD and mild behavioral changes.
  • Analysis of individual patient data (primarily from case reports) for MND-bvFTD patients to characterize symptoms and pooled prevalences.

Main Results:

  • In cohort studies, 8.1% of MND patients were diagnosed with bvFTD (95% CI 5.6 - 11.5%).
  • Among 170 individual MND-bvFTD patients, the most common behavioral changes were perseveration (40%), apathy (29%), and disinhibition (26%).
  • Memory disturbances were reported in 43% of MND-bvFTD patients, and bulbar onset was observed in 48%.

Conclusions:

  • Approximately 8% of individuals with MND meet criteria for bvFTD.
  • Perseveration is the most frequently observed behavioral change in MND-bvFTD.
  • MND-bvFTD is frequently associated with memory disturbances and a bulbar site of onset.

More Related Videos

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies15:48

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

Published on: July 29, 2007

Electrophysiological Motor Unit Number Estimation (MUNE) Measuring Compound Muscle Action Potential (CMAP) in Mouse Hindlimb Muscles09:07

Electrophysiological Motor Unit Number Estimation (MUNE) Measuring Compound Muscle Action Potential (CMAP) in Mouse Hindlimb Muscles

Published on: September 25, 2015

Related Concept Videos

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...
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'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...
Neural Regulation01:37

Neural Regulation

Digestion begins with a cephalic phase that prepares the digestive system to receive food. When our brain processes visual or olfactory information about food, it triggers impulses in the cranial nerves innervating the salivary glands and stomach to prepare for food.