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Related Concept Videos

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...
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
Tetanus01:29

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Tetanus is a life-threatening neurological disorder characterized by persistent muscle contractions and spastic paralysis. It is caused by Clostridium tetani, a motile, Gram-positive, rod-shaped, obligate anaerobe. These bacteria produce terminal endospores, giving them a distinctive “lollipop” or “tennis-racket” appearance. They thrive in anaerobic environments, such as those found in deep puncture wounds.Once introduced into the body, the spores germinate into vegetative cells. These cells...
Disorders of the Autonomic Nervous System01:18

Disorders of the Autonomic Nervous System

The autonomic nervous system (ANS) is an intricate network of nerves that controls functions such as the regulation of heart rate, digestion, and blood pressure regulation. When this system malfunctions, it can lead to various disorders that affect multiple bodily functions. One common feature of many autonomic disorders is the involvement of smooth blood vessels, which play a crucial role in regulating blood flow throughout the body.
Raynaud's disease, also known as Raynaud's phenomenon, is a...
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...
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REM Sleep Behavior Disorder

REM Sleep Behavior Disorder (RBD) is a sleep disorder characterized by the absence of muscle paralysis that normally occurs during the REM phase of sleep. This absence allows individuals to physically act out their dreams, which are often vivid and disturbing. Common behaviors exhibited during episodes include kicking, punching, and yelling. These actions can be dangerous, potentially leading to injuries for the person with RBD or their bed partner.
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Related Experiment Video

Updated: Jun 24, 2026

Quantifying Arms and Legs Contributions during Repetitive Electrically-Assisted Sit-To-Stand Exercise in Paraplegics: A Pilot Study
08:40

Quantifying Arms and Legs Contributions during Repetitive Electrically-Assisted Sit-To-Stand Exercise in Paraplegics: A Pilot Study

Published on: November 11, 2022

Stiff person syndrome.

Martin E Duddy, Mark R Baker

    Frontiers of Neurology and Neuroscience
    |April 8, 2009
    PubMed
    Summary
    This summary is machine-generated.

    Stiff person syndrome (SPS) and related disorders are rare autoimmune central nervous system diseases characterized by rigidity and spasms. Intravenous immunoglobulin is a key treatment, though prognosis varies by subtype.

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    Robotic Mirror Therapy System for Functional Recovery of Hemiplegic Arms
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    Published on: August 15, 2016

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    Last Updated: Jun 24, 2026

    Quantifying Arms and Legs Contributions during Repetitive Electrically-Assisted Sit-To-Stand Exercise in Paraplegics: A Pilot Study
    08:40

    Quantifying Arms and Legs Contributions during Repetitive Electrically-Assisted Sit-To-Stand Exercise in Paraplegics: A Pilot Study

    Published on: November 11, 2022

    Robotic Mirror Therapy System for Functional Recovery of Hemiplegic Arms
    10:32

    Robotic Mirror Therapy System for Functional Recovery of Hemiplegic Arms

    Published on: August 15, 2016

    Area of Science:

    • Neurology
    • Immunology
    • Rare Diseases

    Background:

    • Stiff person syndrome (SPS) and its variants, including stiff limb syndrome, jerking SPS, and progressive encephalomyelitis with rigidity and myoclonus (PERM), are rare, progressive central nervous system disorders.
    • These conditions share core clinical features of continuous involuntary motor unit activity causing appendicular and axial rigidity, along with stimulus-sensitive spasms.
    • A primary autoimmune etiology is strongly supported for these disorders.

    Purpose of the Study:

    • To provide a comprehensive overview of Stiff Person Syndrome (SPS) and its related spectrum of rare neurological disorders.
    • To discuss the autoimmune basis, diagnostic markers, therapeutic strategies, and prognostic factors associated with SPS and its variants.

    Main Methods:

    • Review of existing literature on Stiff Person Syndrome (SPS) and related conditions.
    • Analysis of clinical features, autoimmune markers (specifically anti-GAD65 antibodies), and treatment outcomes.
    • Examination of prognostic indicators and mortality rates in different subtypes of SPS.

    Main Results:

    • Anti-glutamic acid decarboxylase (anti-GAD) antibodies, particularly against the GAD65 isoform, are detected in 60-80% of patients with SPS and its variants.
    • A paraneoplastic form of SPS occurs in approximately 5% of cases, often associated with distinct auto-antibody profiles.
    • Intravenous immunoglobulin (IVIg) is the primary disease-modifying therapy, while symptomatic treatments include benzodiazepines, baclofen, tiagabine, and levetiracetam.

    Conclusions:

    • SPS and its variants are autoimmune neurological disorders with characteristic rigidity and spasms.
    • While many patients stabilize, a significant minority face severe complications, including intensive care needs and mortality.
    • Prognosis is generally poorer for paraneoplastic SPS, jerking SPS, and PERM compared to primary SPS.