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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,...
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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

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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...
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Parkinson’s disease is a chronic, progressive neurodegenerative disorder that primarily affects movement. It is characterized by motor symptoms such as resting tremors, muscle rigidity, bradykinesia (slowness of movement), and postural instability. Patients may notice hand tremors at rest, stiffness during movement, or a shuffling gait. In addition to motor features, non-motor symptoms include sleep disturbances, mood and behavioral changes, constipation, and cognitive impairment, all of which...
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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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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.
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Handwriting Analysis Indicates Spontaneous Dyskinesias in Neuroleptic Naïve Adolescents at High Risk for Psychosis
05:52

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Sydenham's chorea.

Francisco Cardoso1

  • 1Movement Disorders Clinic, Neurology Service, Department of Internal Medicine, Federal University of Minas Gerais, Belo Horizonte, MG, Brazil. cardosofe@terra.com.br

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

Sydenham's chorea (SC), a neurological manifestation of acute rheumatic fever (ARF), is the leading cause of acute chorea in children. While most cases resolve spontaneously, some children experience persistent motor and non-motor symptoms.

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Area of Science:

  • Pediatric Neurology
  • Rheumatology
  • Infectious Diseases

Background:

  • Sydenham's chorea (SC) is the primary neurological symptom of acute rheumatic fever (ARF).
  • SC remains the most frequent cause of acute chorea in pediatric populations globally.
  • It presents with diverse motor and non-motor symptoms.

Purpose of the Study:

  • To review the clinical presentation, pathogenesis, diagnosis, and management of Sydenham's chorea.
  • To highlight the significance of SC within the context of ARF in children.

Main Methods:

  • Literature review focusing on clinical characteristics, diagnostic criteria, and therapeutic strategies for SC.
  • Analysis of the proposed autoimmune pathogenesis involving streptococcal antigens and basal ganglia.

Main Results:

  • SC is characterized by chorea, hypotonia, tics, and non-motor symptoms like executive dysfunction and emotional lability.
  • Diagnosis is clinical, as no specific biomarkers exist.
  • Management includes antichoreic medications and antibiotic prophylaxis against streptococcal infections.

Conclusions:

  • While SC often remits spontaneously, a notable percentage of patients endure persistent chorea.
  • Understanding SC is crucial for managing ARF sequelae in children.