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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...
Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin01:26

Directly Acting Muscle Relaxants: Dantrolene and Botulinum Toxin

Directly acting muscle relaxants like dantrolene and botulinum toxin (BoNT) have distinct mechanisms and applications. Dantrolene, a hydantoin derivative, acts on the ryanodine receptor (RYR1) in skeletal muscle cells. RYR1 are calcium channels present at the sarcoplasmic reticulum membrane. In response to excitation, they release calcium ions from the sarcoplasmic reticulum to the cytosol. Calcium promotes actin-myosin-mediated contraction of muscles.
The binding of dantrolene to the RYR1...
Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
Parkinson's Disease: Treatment01:24

Parkinson's Disease: Treatment

Neurodegenerative disorders, such as Parkinson's Disease (PD), involve the gradual and irreversible destruction of neurons in particular brain areas. These disorders exhibit standard features like proteinopathies, selective vulnerability of some neurons, and an interaction of intrinsic properties, genetics, and environmental influences in neural injury.
Parkinson's Disease is primarily a result of the loss of dopaminergic neurons in the substantia nigra pars compacta. The cornerstone of its...
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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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Related Experiment Video

Updated: Jun 2, 2026

Measurement & Analysis of the Temporal Discrimination Threshold Applied to Cervical Dystonia
10:05

Measurement & Analysis of the Temporal Discrimination Threshold Applied to Cervical Dystonia

Published on: January 27, 2018

Early-onset primary dystonia.

Bradley J Robottom1, William J Weiner, Cynthia L Comella

  • 1Department of Neurology, University of Maryland School of Medicine, Baltimore, MD, USA.

Handbook of Clinical Neurology
|April 19, 2011
PubMed
Summary
This summary is machine-generated.

This chapter details early-onset primary dystonia, focusing on its diagnosis and treatment. It highlights DYT1 dystonia and proposes a diagnostic algorithm for gene-negative cases.

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Last Updated: Jun 2, 2026

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10:05

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Published on: January 27, 2018

Implantation of Osmotic Pumps and Induction of Stress to Establish a Symptomatic, Pharmacological Mouse Model for DYT/PARK-ATP1A3 Dystonia
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Area of Science:

  • Neurology
  • Genetics
  • Movement Disorders

Background:

  • Dystonia involves sustained muscle contractions causing abnormal postures and movements.
  • Classification by age of onset aids diagnosis and treatment planning for dystonia.
  • Early-onset primary dystonia affects individuals under 26 years old.

Observation:

  • This chapter reviews the history, clinical features, genetics, pathophysiology, diagnosis, and treatment of early-onset primary dystonia.
  • DYT1 dystonia, an autosomal-dominant form, is the most common type of early-onset primary dystonia.
  • A diagnostic algorithm is proposed for cases without identified genetic markers.

Findings:

  • Early-onset primary dystonia requires a comprehensive diagnostic approach.
  • Genetic factors play a significant role in the etiology of dystonia.
  • Specific treatment recommendations are provided for generalized early-onset dystonia.

Implications:

  • Understanding early-onset dystonia aids in timely diagnosis and management.
  • Genetic insights into dystonia can inform future therapeutic strategies.
  • The proposed diagnostic algorithm can streamline the evaluation of affected individuals.