Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

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,...
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
Graves Disease II: Pathophysiology01:24

Graves Disease II: Pathophysiology

Graves’ disease is an autoimmune disorder characterized by the production of thyroid-stimulating immunoglobulins (TSI) that activate TSH receptors, leading to excessive synthesis and release of thyroid hormones (T3 and T4) and resulting in hyperthyroidism.Among all causes of hyperthyroidism, Graves’ disease is the most common and can happen at any age, though it is more frequent in women. It produces a hypermetabolic state with features such as weight loss, tachycardia, tremor, and heat...
Lysosomal Hydrolases01:22

Lysosomal Hydrolases

Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...
Graves' Disease I: Introduction01:28

Graves' Disease I: Introduction

Graves' disease is an autoimmune disorder that causes hyperthyroidism, or overactivity of the thyroid gland. It results from autoantibodies called thyroid-stimulating immunoglobulins (TSIs), which bind to thyroid-stimulating hormone (TSH) receptors, leading to overstimulation of hormone production and a hypermetabolic state.EtiologyAlthough considered idiopathic, Graves’ disease has well-established contributing factors. There is a strong genetic component, with increased prevalence in...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Reconfigurable Logic-in-Memory Oxide Transistors Enabled by Transferable Ferroelectric HZO.

ACS nano·2026
Same author

Aristolochic acid and the risk of female lung cancer: Population-based case-control study in Taiwan.

Cancer epidemiology·2026
Same author

Subject: Author response to "Comment on 'Identification of the molecular characterization and tumor microenvironment of thoracic inflammatory myofibroblastic tumors"' (JFMA-D-26-00819).

Journal of the Formosan Medical Association = Taiwan yi zhi·2026
Same author

Response to letter to the editor "Revisiting outcome interpretation and tumor microenvironment characterization in thoracic inflammatory myofibroblastic tumors".

Journal of the Formosan Medical Association = Taiwan yi zhi·2026
Same author

Cabozantinib and atezolizumab for recurrent or metastatic esophageal squamous cell carcinoma after platinum-based chemotherapy failure: a single-arm phase II study and biomarker analysis.

Cancer immunology, immunotherapy : CII·2026
Same author

CASK promotes non-small cell lung cancer growth through coordinated regulation of EGFR expression, trafficking, and p21 expression.

Journal of biomedical science·2026
Same journal

State-of-the-Art Epilepsy Imaging: Improving Tools for Epileptogenic Lesion Detection and Treatment.

Neuroimaging clinics of North America·2026
Same journal

Imaging of Epilepsy.

Neuroimaging clinics of North America·2026
Same journal

Implanted Devices for Management of Drug-Resistant Epilepsy: Background and MR Imaging Considerations.

Neuroimaging clinics of North America·2026
Same journal

Imaging of Epilepsy Surgery, Minimally Invasive Techniques, and Neuromodulation.

Neuroimaging clinics of North America·2026
Same journal

Neuroimaging and the Epilepsy Journey.

Neuroimaging clinics of North America·2026
Same journal

Clinical Functional Magnetic Resonance Imaging in Epilepsy.

Neuroimaging clinics of North America·2026
See all related articles

Related Experiment Video

Updated: May 28, 2026

Functional Characterization of Endogenously Expressed Human RYR1 Variants
07:59

Functional Characterization of Endogenously Expressed Human RYR1 Variants

Published on: June 9, 2021

Hirayama disease.

Yen-Lin Huang1, Chi-Jen Chen

  • 1Department of Diagnostic Radiology, Shuang-Ho Hospital, Taipei Medical University, New Taipei City, Taiwan, Republic of China.

Neuroimaging Clinics of North America
|October 29, 2011
PubMed
Summary
This summary is machine-generated.

Hirayama disease, a cervical myelopathy affecting adolescent males, causes progressive upper limb weakness. It may stem from dural sac displacement during neck flexion, compressing the spinal cord.

More Related Videos

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
03:45

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Published on: August 8, 2022

Related Experiment Videos

Last Updated: May 28, 2026

Functional Characterization of Endogenously Expressed Human RYR1 Variants
07:59

Functional Characterization of Endogenously Expressed Human RYR1 Variants

Published on: June 9, 2021

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
03:45

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Published on: August 8, 2022

Area of Science:

  • Neurology
  • Orthopedics

Background:

  • Hirayama disease, also known as juvenile muscular atrophy of the distal upper extremity, is a cervical myelopathy.
  • It predominantly affects adolescent males, presenting with progressive muscular weakness and atrophy in the distal upper limbs.
  • The condition typically arrests spontaneously within several years.

Purpose of the Study:

  • To elucidate the underlying mechanisms of Hirayama disease.
  • To emphasize the importance of early recognition and management for halting disease progression.

Main Methods:

  • Neuropathologic and neuroradiologic investigations were utilized.
  • Analysis focused on cervical cord changes during neck flexion.

Main Results:

  • Findings suggest a forward displacement of the posterior cervical dural sac during neck flexion.
  • This displacement leads to cervical cord compression.
  • The compression results in atrophic and ischemic changes in the anterior horn cells.

Conclusions:

  • Understanding Hirayama disease is crucial for effective intervention.
  • Early diagnosis and management can prevent further deterioration.