The ad hoc Committee defined chorea as excessive, spontaneous, irregular, non-repetitive, randomly distributed, and abrupt movements.
Movements range from mild restlessness and fidgeting to severe, continuous, disabling, and violent movements resembling uncontrollable dancing.
Patients with chorea show motor impersistence and exhibit a “milkmaid’s grip” when gripping objects.
Patients involuntarily drop objects and may try to mask chorea with purposeful movements. Chorea affects proximal and distal muscles typically with normal tone or hypotonia present.
Levodopa-induced chorea is the most common disorder in movement centers. It describes slow chorea with writhing movements due to its sluggish nature.
Some neurologists find the term athetosis unnecessary as it only differs from chorea by movement speed.
Ballism involves continuous, violent, involuntary movements characterized by flinging of limbs due to coordinated activity of axial and proximal muscles.
Epidemiology
Huntington disease is an autosomal dominant neurodegenerative disorder linked to chromosome 4.
The estimated prevalence in the United States is 5 to 10 cases per 100,000 people. Gene prevalence may reach 1%, but disease prevalence is only 30 cases per million.
Benign hereditary chorea is a rare genetic disorder with a prevalence of about 1 in 500,000 individuals.
Huntington disease (HD) usually appears in the 40s or 50s, with under 10% of cases starting before age 20, and rare instances before 5 years old.
Onset age inversely correlates with CAG repeat size to predict motor symptom onset in patients.
Anatomy
Pathophysiology
A model of basal ganglia function describes dopaminergic and GABAergic impulses traveling from the substantia nigra to motor cortex.
Striatum modulates impulses via parallel direct and indirect loops through two pallidum pathways.
It Subthalamic nucleus activity induces parkinsonism via pallidum inhibition. Absent subthalamic nucleus inhibition increases motor activity and involuntary movements
Huntington disease results from expanded CAG repeats in the huntingtin gene causes neuronal degeneration via transcription dysregulation and mitochondrial impairment.
A critical balance of acetylcholine and dopamine is vital for normal striatal function in Parkinson’s disease.
Etiology
The causes of chorea are:
Genetic Causes
Autoimmune and Post-Infectious Causes
Metabolic and Endocrine Disorders
Vascular Causes
Infectious Causes
Genetics
Prognostic Factors
Sydenham’s chorea resolves within weeks to months but may recur.
Huntington’s disease progresses relentlessly results in disability and early death.
Poor prognosis in Huntington’s disease and dementia. Patients with multiple comorbidities face greater complication risk.
Larger CAG repeats lead to earlier onset, faster progression, and poorer outcomes.
Clinical History
Clinical History:
Collect details including the chief complaint, onset of progression, and associated symptoms, medical and family history to understand clinical history of patients.
Deep brain stimulation may benefit certain patients in specific cases.
While cell transplantation is controversial as early-stage research shows varied HD outcomes.
use-of-phases-in-managing-chorea
In the immediate assessment and stabilization phase, the goal is to identify and treat life-threatening causes to prevent complications.
Pharmacologic therapy is effective in the treatment phase as it includes the use of antipsychotic agents, monoamine depleting agents, benzodiazepines, and anticonvulsants.
In supportive care and management phase, patients should receive required attention such as lifestyle modification and interventional therapies.
The regular follow-up visits with the neurologist are scheduled to check the improvement of patients along with treatment response.
The ad hoc Committee defined chorea as excessive, spontaneous, irregular, non-repetitive, randomly distributed, and abrupt movements.
Movements range from mild restlessness and fidgeting to severe, continuous, disabling, and violent movements resembling uncontrollable dancing.
Patients with chorea show motor impersistence and exhibit a “milkmaid’s grip” when gripping objects.
Patients involuntarily drop objects and may try to mask chorea with purposeful movements. Chorea affects proximal and distal muscles typically with normal tone or hypotonia present.
Levodopa-induced chorea is the most common disorder in movement centers. It describes slow chorea with writhing movements due to its sluggish nature.
Some neurologists find the term athetosis unnecessary as it only differs from chorea by movement speed.
Ballism involves continuous, violent, involuntary movements characterized by flinging of limbs due to coordinated activity of axial and proximal muscles.
Huntington disease is an autosomal dominant neurodegenerative disorder linked to chromosome 4.
The estimated prevalence in the United States is 5 to 10 cases per 100,000 people. Gene prevalence may reach 1%, but disease prevalence is only 30 cases per million.
Benign hereditary chorea is a rare genetic disorder with a prevalence of about 1 in 500,000 individuals.
Huntington disease (HD) usually appears in the 40s or 50s, with under 10% of cases starting before age 20, and rare instances before 5 years old.
Onset age inversely correlates with CAG repeat size to predict motor symptom onset in patients.
A model of basal ganglia function describes dopaminergic and GABAergic impulses traveling from the substantia nigra to motor cortex.
Striatum modulates impulses via parallel direct and indirect loops through two pallidum pathways.
It Subthalamic nucleus activity induces parkinsonism via pallidum inhibition. Absent subthalamic nucleus inhibition increases motor activity and involuntary movements
Huntington disease results from expanded CAG repeats in the huntingtin gene causes neuronal degeneration via transcription dysregulation and mitochondrial impairment.
A critical balance of acetylcholine and dopamine is vital for normal striatal function in Parkinson’s disease.
The causes of chorea are:
Genetic Causes
Autoimmune and Post-Infectious Causes
Metabolic and Endocrine Disorders
Vascular Causes
Infectious Causes
Sydenham’s chorea resolves within weeks to months but may recur.
Huntington’s disease progresses relentlessly results in disability and early death.
Poor prognosis in Huntington’s disease and dementia. Patients with multiple comorbidities face greater complication risk.
Larger CAG repeats lead to earlier onset, faster progression, and poorer outcomes.
Clinical History:
Collect details including the chief complaint, onset of progression, and associated symptoms, medical and family history to understand clinical history of patients.
Chorea can cause bruises, fractures, falls, and impair patients’ self-feeding abilities.
Neuroleptics are the most common agents for chorea treatment, primarily working by blocking dopamine receptors.
Dopamine-depleting agents treat chorea in Huntington disease.
Coenzyme Q10 and minocycline show potential as therapies in HD rodent models.
Intravenous immunoglobulin and plasmapheresis can reduce illness duration and symptoms severity.
GABAergic drugs effectively serve as adjunctive therapy options.
Rarity and severity of disorder prevent placebo-controlled drug trials.
Neurology
Remove tripping hazards to eliminate rugs, clutter, and electrical cords from walkways.
Patient should use padded furniture to reduce injury from involuntary movements.
Use chairs with armrests and high backs to prevent falls.
Use cushioned wheelchairs with seat belts to prevent sudden movements from causing falls.
Proper awareness about chorea should be provided and its related causes with management strategies.
Appointments with neurologist and preventing recurrence of disorder is an ongoing life-long effort.
Neurology
Haloperidol:
It is indicated in the treatment of irregular spasmodic movements of limbs or facial muscles.
Fluphenazine:
It blocks postsynaptic mesolimbic dopaminergic D1 and D2 receptors in brain.
Clozapine:
It blocks norepinephrine, cholinergic, histamine, and dopaminergic receptors.
Neurology
Reserpine:
It blocks the vesicular monoamine transporters- VMAT 1 and 2 that reduce the stores of the monoamines
Tetrabenazine:
It depletes neurotransmitter stores of dopamine and noradrenaline within nerve cells in the brain.
Valbenazine:
It is a VMAT2 inhibitor used for chorea associated with Huntington disease.
Neurology
Clonazepam:
It developed as antiepileptic and anxiolytic used as adjunct for treatment.
Neurology
Valproic acid:
It increases levels of the inhibitory neurotransmitter gamma-aminobutyric acid in brain.
Carbamazepine:
It depresses activity in nucleus ventralis anterior of the thalamus in a reduction of polysynaptic responses.
Neurology
Deep brain stimulation may benefit certain patients in specific cases.
While cell transplantation is controversial as early-stage research shows varied HD outcomes.
Neurology
In the immediate assessment and stabilization phase, the goal is to identify and treat life-threatening causes to prevent complications.
Pharmacologic therapy is effective in the treatment phase as it includes the use of antipsychotic agents, monoamine depleting agents, benzodiazepines, and anticonvulsants.
In supportive care and management phase, patients should receive required attention such as lifestyle modification and interventional therapies.
The regular follow-up visits with the neurologist are scheduled to check the improvement of patients along with treatment response.
The ad hoc Committee defined chorea as excessive, spontaneous, irregular, non-repetitive, randomly distributed, and abrupt movements.
Movements range from mild restlessness and fidgeting to severe, continuous, disabling, and violent movements resembling uncontrollable dancing.
Patients with chorea show motor impersistence and exhibit a “milkmaid’s grip” when gripping objects.
Patients involuntarily drop objects and may try to mask chorea with purposeful movements. Chorea affects proximal and distal muscles typically with normal tone or hypotonia present.
Levodopa-induced chorea is the most common disorder in movement centers. It describes slow chorea with writhing movements due to its sluggish nature.
Some neurologists find the term athetosis unnecessary as it only differs from chorea by movement speed.
Ballism involves continuous, violent, involuntary movements characterized by flinging of limbs due to coordinated activity of axial and proximal muscles.
Huntington disease is an autosomal dominant neurodegenerative disorder linked to chromosome 4.
The estimated prevalence in the United States is 5 to 10 cases per 100,000 people. Gene prevalence may reach 1%, but disease prevalence is only 30 cases per million.
Benign hereditary chorea is a rare genetic disorder with a prevalence of about 1 in 500,000 individuals.
Huntington disease (HD) usually appears in the 40s or 50s, with under 10% of cases starting before age 20, and rare instances before 5 years old.
Onset age inversely correlates with CAG repeat size to predict motor symptom onset in patients.
A model of basal ganglia function describes dopaminergic and GABAergic impulses traveling from the substantia nigra to motor cortex.
Striatum modulates impulses via parallel direct and indirect loops through two pallidum pathways.
It Subthalamic nucleus activity induces parkinsonism via pallidum inhibition. Absent subthalamic nucleus inhibition increases motor activity and involuntary movements
Huntington disease results from expanded CAG repeats in the huntingtin gene causes neuronal degeneration via transcription dysregulation and mitochondrial impairment.
A critical balance of acetylcholine and dopamine is vital for normal striatal function in Parkinson’s disease.
The causes of chorea are:
Genetic Causes
Autoimmune and Post-Infectious Causes
Metabolic and Endocrine Disorders
Vascular Causes
Infectious Causes
Sydenham’s chorea resolves within weeks to months but may recur.
Huntington’s disease progresses relentlessly results in disability and early death.
Poor prognosis in Huntington’s disease and dementia. Patients with multiple comorbidities face greater complication risk.
Larger CAG repeats lead to earlier onset, faster progression, and poorer outcomes.
Clinical History:
Collect details including the chief complaint, onset of progression, and associated symptoms, medical and family history to understand clinical history of patients.
Chorea can cause bruises, fractures, falls, and impair patients’ self-feeding abilities.
Neuroleptics are the most common agents for chorea treatment, primarily working by blocking dopamine receptors.
Dopamine-depleting agents treat chorea in Huntington disease.
Coenzyme Q10 and minocycline show potential as therapies in HD rodent models.
Intravenous immunoglobulin and plasmapheresis can reduce illness duration and symptoms severity.
GABAergic drugs effectively serve as adjunctive therapy options.
Rarity and severity of disorder prevent placebo-controlled drug trials.
Neurology
Remove tripping hazards to eliminate rugs, clutter, and electrical cords from walkways.
Patient should use padded furniture to reduce injury from involuntary movements.
Use chairs with armrests and high backs to prevent falls.
Use cushioned wheelchairs with seat belts to prevent sudden movements from causing falls.
Proper awareness about chorea should be provided and its related causes with management strategies.
Appointments with neurologist and preventing recurrence of disorder is an ongoing life-long effort.
Neurology
Haloperidol:
It is indicated in the treatment of irregular spasmodic movements of limbs or facial muscles.
Fluphenazine:
It blocks postsynaptic mesolimbic dopaminergic D1 and D2 receptors in brain.
Clozapine:
It blocks norepinephrine, cholinergic, histamine, and dopaminergic receptors.
Neurology
Reserpine:
It blocks the vesicular monoamine transporters- VMAT 1 and 2 that reduce the stores of the monoamines
Tetrabenazine:
It depletes neurotransmitter stores of dopamine and noradrenaline within nerve cells in the brain.
Valbenazine:
It is a VMAT2 inhibitor used for chorea associated with Huntington disease.
Neurology
Clonazepam:
It developed as antiepileptic and anxiolytic used as adjunct for treatment.
Neurology
Valproic acid:
It increases levels of the inhibitory neurotransmitter gamma-aminobutyric acid in brain.
Carbamazepine:
It depresses activity in nucleus ventralis anterior of the thalamus in a reduction of polysynaptic responses.
Neurology
Deep brain stimulation may benefit certain patients in specific cases.
While cell transplantation is controversial as early-stage research shows varied HD outcomes.
Neurology
In the immediate assessment and stabilization phase, the goal is to identify and treat life-threatening causes to prevent complications.
Pharmacologic therapy is effective in the treatment phase as it includes the use of antipsychotic agents, monoamine depleting agents, benzodiazepines, and anticonvulsants.
In supportive care and management phase, patients should receive required attention such as lifestyle modification and interventional therapies.
The regular follow-up visits with the neurologist are scheduled to check the improvement of patients along with treatment response.
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