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Cross-bridge Cycle01:26

Cross-bridge Cycle

As muscle contracts, the overlap between the thin and thick filaments increases, decreasing the length of the sarcomere—the contractile unit of the muscle—using energy in the form of ATP. At the molecular level, this is a cyclic, multistep process that involves binding and hydrolysis of ATP, and movement of actin by myosin.
Myasthenia Gravis ll: Pathophysiology01:22

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The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...
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...
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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...
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Updated: Jun 25, 2026

ALS - Motor Neuron Disease: Mechanism and Development of New Therapies
15:48

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

Published on: July 29, 2007

Amyotrophic lateral sclerosis.

Lokesh C Wijesekera1, P Nigel Leigh

  • 1MRC centre for Neurodegeneration Research, Department of Clinical Neuroscience, Institute of Psychiatry, Kings College London, London, UK. lokesh.wijesekera@iop.kcl.ac.uk

Orphanet Journal of Rare Diseases
|February 5, 2009
PubMed
Summary
This summary is machine-generated.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease causing muscle paralysis. Diagnosis involves clinical evaluation and excluding mimics, with supportive care and Riluzole offering the only survival benefit.

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Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis (ALS)
12:35

Clinical Testing and Spinal Cord Removal in a Mouse Model for Amyotrophic Lateral Sclerosis (ALS)

Published on: March 17, 2012

Area of Science:

  • Neurology
  • Neuroscience
  • Genetics

Background:

  • Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by motor neuron degeneration.
  • It leads to muscular paralysis, with incidence rates around 1.89 per 100,000 person-years and a prevalence of 5.2 per 100,000.
  • Onset typically occurs around age 60, with a slight male predominance.

Purpose of the Study:

  • To provide a comprehensive overview of Amyotrophic Lateral Sclerosis (ALS).
  • To detail the clinical presentation, diagnosis, pathology, and management strategies for ALS.
  • To highlight genetic factors and epidemiological data related to ALS.

Main Methods:

  • Clinical history and examination are primary diagnostic tools.
  • Electromyography (EMG) aids in assessing motor neuron function.
  • Exclusion of other neurological conditions mimicking ALS is crucial.

Main Results:

  • ALS presents with either spinal (limb onset) or bulbar onset symptoms.
  • Pathological hallmarks include motor neuron loss and specific protein inclusions (TDP-43).
  • Familial cases involve SOD1 and TARDBP gene mutations, also found in sporadic forms.

Conclusions:

  • ALS management is multidisciplinary and supportive, focusing on palliative care.
  • Non-invasive ventilation can improve survival and quality of life.
  • Riluzole remains the sole approved medication demonstrated to extend survival in ALS patients.