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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,...
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...
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 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...
Disorders of the Skeletal Muscle01:28

Disorders of the Skeletal Muscle

The clinical conditions affecting the skeletal muscle tissue are broadly categorized as musculoskeletal and neuromuscular disorders.
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
Parkinson Disease ll: Pathophysiology01:24

Parkinson Disease ll: Pathophysiology

Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...

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Related Experiment Video

Updated: May 14, 2026

Handwriting Analysis Indicates Spontaneous Dyskinesias in Neuroleptic Naïve Adolescents at High Risk for Psychosis
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Handwriting Analysis Indicates Spontaneous Dyskinesias in Neuroleptic Naïve Adolescents at High Risk for Psychosis

Published on: November 21, 2013

Autoimmune chorea in adults.

Orna O'Toole1, Vanda A Lennon, J Eric Ahlskog

  • 1Department of Neurology, Mayo Clinic, College of Medicine, Rochester, MN, USA.

Neurology
|February 22, 2013
PubMed
Summary

Autoimmune chorea is a rare, rapidly progressing neurological disorder. Older age, male sex, weight loss, and peripheral neuropathy may indicate an underlying cancer.

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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis

Published on: July 4, 2007

Area of Science:

  • Neurology
  • Immunology
  • Oncology

Background:

  • Autoimmune chorea is a rare neurological disorder characterized by involuntary, irregular, and purposeless movements.
  • Distinguishing between paraneoplastic and idiopathic causes is crucial for appropriate management and prognosis.

Purpose of the Study:

  • To characterize adult-onset autoimmune chorea.
  • To compare the clinical and immunological features of paraneoplastic and idiopathic subgroups.

Main Methods:

  • A retrospective review of 36 adult patients with autoimmune chorea diagnosed between 1997 and 2012.
  • Comparison of clinical data and autoantibody profiles between nonparaneoplastic (n=22) and paraneoplastic (n=14) cases.

Main Results:

  • The median age of onset was 67 years, with subacute symptom progression in all patients.
  • Paraneoplastic cases were older, more frequently male, and exhibited weight loss and peripheral neuropathy compared to idiopathic cases.
  • While NMDA receptor antibodies were absent, novel synaptic IgG antibodies (GAD65, CASPR2) were identified in some patients. Common cancers included small-cell carcinoma and adenocarcinoma, with CRMP-5-IgG and ANNA-1 autoantibodies frequently detected. Idiopathic cases often coexisted with other autoimmune disorders like systemic lupus erythematosus and antiphospholipid syndrome.

Conclusions:

  • Autoimmune chorea typically presents with rapid onset and is more common in women.
  • Factors such as male sex, older age, severe chorea, peripheral neuropathy, and weight loss are associated with an increased likelihood of an underlying malignancy.