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Related Concept Videos

Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

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...
Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...
Myasthenia Gravis: Diagnostic Tests01:15

Myasthenia Gravis: Diagnostic Tests

Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
Autoimmune Disorders01:29

Autoimmune Disorders

Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune system...
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...

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Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP): Estimation of pathological lesion stage from brain images.

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Pathologic basis of the preferential thinning of thecorpus callosum in adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP).

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Related Experiment Video

Updated: Jun 4, 2026

Du-Moxibustion in a Mouse Model of Ankylosing Spondylitis
04:47

Du-Moxibustion in a Mouse Model of Ankylosing Spondylitis

Published on: October 27, 2023

[Yuasa-Mitsuyama disease].

Yoshio Mitsuyama1

  • 1Daigo Hospital, Miyazaki, Japan.

Brain and Nerve = Shinkei Kenkyu No Shinpo
|February 9, 2011
PubMed
Summary

Frontotemporal dementia with motor neuron disease (FTD-MND) is a unique clinicopathological entity. Its diagnosis relies on identifying ubiquitin-positive, tau-negative inclusions, distinct from Pick's disease.

Area of Science:

  • Neurology and neuropathology
  • Neurodegenerative diseases

Context:

  • Frontotemporal dementia (FTD) encompasses Pick's disease and frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP-43).
  • FTLD-TDP-43 is frequently associated with motor neuron disease (MND), forming FTD-MND.
  • Over 200 cases of FTD-MND have been reported in Japan.

Purpose:

  • To define FTD-MND as a unique clinicopathological entity.
  • To outline the distinct clinical and pathological characteristics of FTD-MND.
  • To highlight the diagnostic significance of TDP-43 protein inclusions.

Summary:

  • FTD-MND presents with insidious frontotemporal dementia, progressive neurogenic muscular wasting (ALS/SPMA-like symptoms), and a 2-5 year survival.
  • Key pathological findings include ubiquitin-immunoreactive, tau-negative, TDP-43 positive intraneuronal inclusions in specific brain regions.

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  • Screening tests like CSF and EEG show no characteristic abnormalities; familial occurrence is rare.

    • Impact:

    • Establishes FTD-MND as a distinct disease entity, aiding in accurate diagnosis and classification.
    • Identifies TDP-43 inclusions as a crucial diagnostic marker for FTD-MND.
    • Calls for further research to differentiate FTD-MND from ALS-dementia (ALS-D).