Chorea: Difference between revisions

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==Introduction==
==Introduction==
The word Chorea has Greek origins meaning "to dance". It has a dance-like appearance due to the random and flowing quality of the movement. Chorea is a type of [[Hyperkinetic Movement Disorder|hyperkinetic movement disorder]]. The involuntary movements flow from one body area to another which cannot be predicted beforehand.
[[File:Chorea.jpeg|thumb|Rheumatic chorea]]
Chorea is defined as "a state of excessive, spontaneous movements, irregularly timed, non-repetitive, randomly distributed and abrupt in character. These movements may vary in severity from restlessness with mild intermittent exaggeration of gesture and expression, fidgeting movements of the hands, unstable dance-like gait to a continuous flow of disabling, violent movements."<ref name=":1">Merical B, Sánchez-Manso JC. Chorea. Available: https://www.ncbi.nlm.nih.gov/books/NBK430923/#!po=15.0000<nowiki/>(accessed 19.9.2022)</ref>


== Causes ==
# The word Chorea has Greek origins meaning "to dance". It has a dance-like appearance due to the random and flowing quality of the movement.
They are predominantly of 2 types
# Chorea is a type of [[Hyperkinetic Movement Disorder|hyperkinetic movement disorder]].
# The involuntary movements flow from one body area to another which cannot be predicted beforehand.
 
== Etiology ==
Chorea traditionally is described in Huntington disease, however other disorders that can lead to chorea, for example: Spinocerebellar [[ataxia]] subtypes; [[Wilson's Disease|Wilson disease]]; Sydenham chorea, and structural disease of the basal ganglia. Generally, the root of the pathology is in the [[Basal Ganglia|basal ganglia]].<ref name=":1" />
 
They are predominantly of 2 types<ref name=":0">Termsarasab P. [https://journals.lww.com/continuum/Abstract/2019/08000/Chorea.9.aspx Chorea]. CONTINUUM: Lifelong Learning in Neurology. 2019 Aug 1;25(4):1001-35.</ref>


* Acquired Causes
* Acquired Causes
Line 17: Line 24:
== Phenomenological Features ==
== Phenomenological Features ==


* '''Randomness'''
* Randomness
* '''Flowing Quality'''
* Flowing Quality
* '''Parakinesia'''-Patients blend their chorea-induced movements with their own normal movements.
* Parakinesia: Patients blend their chorea-induced movements with their own normal movements.
* '''Motor impersistence'''- the patient is unable to perform sustained motor activities.
* Motor impersistence: the patient is unable to perform sustained motor activities.
* '''Ballism'''- Variant of chorea which shows large-amplitude flinging movements involving proximal extremities.
* Ballism: Variant of chorea which shows large-amplitude flinging movements involving proximal extremities.
* '''Athetosis'''-Slow writhing movements involving distal limbs sometimes may be facing.
* Athetosis: Slow writhing movements involving distal limbs sometimes may be facing.
* '''Varying velocity of movements'''-quick velocity and low-amplitude movements accompanied by jerks.
* Varying velocity of movements: quick velocity and low-amplitude movements accompanied by jerks.<ref name=":0" />


{{#ev:youtube|RxWEilu-Mf4}}
{{#ev:youtube|RxWEilu-Mf4}}


This video displays the dance-like movements performed by a patient.
This video displays the dance-like movements performed by a patient.<ref>neurosigns.org. Chorea. Available from https://www.youtube.com/watch?v=RxWEilu-Mf4&ab_channel=neurosigns.org</ref>


== Diagnosis ==
== Diagnosis ==
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=== Differential Diagnosis ===
=== Differential Diagnosis ===
Specific diagnosis can be made on the basis of symptoms/clinical features experienced by the patient. These symptoms serves as diagnostic clues and are present as 3 body distributions and other crucial features.
Specific diagnosis can be made on the basis of symptoms/clinical features experienced by the patient.<ref name=":0" />Differential diagnoses include:


1.On Forehead- Huntington Disease.
* [https://www.physio-pedia.com/Huntington_Disease Huntington disease]
* [[Lyme Disease|Lyme disease]]
* [[Multiple System Atrophy|Multiple system atrophy]]
* Neuroacanthocytosis
* Pediatric torticollis surgery
* [[Ramsay Hunt Syndrome|Ramsay Hunt]] syndrome
* Striatonigral degeneration
* Tourette syndrome
* Viral [[encephalitis]]<ref name=":1" />


Wiggling of the eyebrows or activation of the frontalis muscle is present.
== Multimodal Management ==
[[File:Mutimodal management chorea.jpg|right|frameless|387x387px]]
Chorea requires a Multidisciplinary Approach to be treated.<ref>Feinstein E, Walker R. [https://link.springer.com/article/10.1007/s11940-018-0529-y An update on the treatment of chorea. Current Treatment Options in Neurology]. 2018 Oct;20(10):1-5.</ref>                                 


2. On Orobuccolingual region- Tardive Chorea, Acquired Hepatocerebral Degeneration ,Chorea-acanthocytosis, Lesch-Nyhan syndrome, PKAN([[dystonia]]), Lubag Disease([[dystonia]]).
The team includes:


Bonbon sign and problem of inappropriate intake of food is seen which cause biting of lips and tongue.
* Psychiatrist
* Physical medicine and Rehabilitation Specialist
* Physical therapist
* Occupational therapist
* Speech therapists
* Geneticist
* Genetic Counselor
* Social worker
=== Medical Management ===
There is '''no cure''' for this disease but symptoms can be managed. Importantly an evaluation and creation of a patient's support system is needed. With disease progression, the patient will need specialized care. Nutrition management is important, due to difficulty swallowing.<ref name=":1" />


3.Localized to the contralateral subthalamic nucleus(hemichorea)- Nonketonic hyperglycemia, Polycythemia vera, Sydenham Chorea, Others.
1. Dopamine-depleting agents: Tetrabenazine, Deutetrabenazine, Valbenazine.


Some disorders which are differentially diagnosed are given in the table below-
2. Dopamine D2 receptor-blocking agents: Haloperidol, Clozapine, Olanzapine.
{| class="wikitable"
|+
!Disorder/Symptom
!Differentiating Factor from chorea
|-
|Dystonia
|Predictable patterned movements
|-
|Dystonic Tremor
|Irregular and jerky does not have a flowing quality like chorea
|-
|Myoclonic jerks
|Randomness quality of chorea differs the two
|-
|Tics
|Presence of premonitory urge and suppressibility
|-
|Stereotypies
|Has stereotypic movement as if the video is running on a repetitive loop
|-
|Large amplitude cerebellar outflow tremor
|Regular oscillation around the axis and activation of tremor with movements( postural and kinetic components)
|}


== Management ==
3. Anticonvulsants: Valproic acid, Carbamazepine, Benzodiazepines, Levetiracetam.
1. Dopamine-depleting agents


2. Dopamine D2 receptor-blocking agents
4. Anti-glutamatergic agents: Amantadine, Riluzole.


3. Anticonvulsants
5. Cannabinoids: Nabilone.


4. Anti-glutamatergic agents
6. [[Deep Brain Stimulation]]


5. Cannabinoids
== Physiotherapy Management ==
The management will be same as of [https://www.physio-pedia.com/Huntington_Disease?utm_source=physiopedia&utm_medium=search&utm_campaign=ongoing_internal#Physiotherapy_Management Huntington's Disease].  


6. Deep Brain Stimulation
=== Goals of Management ===


7. Physiotherapy
* [[Gait]] re-education
* [[Balance Training|Balance r]]<nowiki/>[[Balance Training|etraining]]
* [[Falls|Fall]] prevention/management
* [[Aerobic Exercise|Aerobic]] capacity
* Muscle [[Strength and Conditioning|strengthening]]
* [[Wheelchair Assessment|Wheelchair]] prescription and training
* [[Respiratory Muscle Training|Respiratory]] function
* Task-specific reach, grasp, and manipulation.


==References==
==References==
[[Category:Neurology]]
[[Category:Neurology]]
[[Category:Neurological - Conditions]]
[[Category:Neurological - Conditions]]

Latest revision as of 12:12, 2 October 2022

Original Editor - Muskan Rastogi Top Contributors - Muskan Rastogi, Lucinda hampton, Chelsea Mclene and Cindy John-Chu

Introduction[edit | edit source]

Rheumatic chorea

Chorea is defined as "a state of excessive, spontaneous movements, irregularly timed, non-repetitive, randomly distributed and abrupt in character. These movements may vary in severity from restlessness with mild intermittent exaggeration of gesture and expression, fidgeting movements of the hands, unstable dance-like gait to a continuous flow of disabling, violent movements."[1]

  1. The word Chorea has Greek origins meaning "to dance". It has a dance-like appearance due to the random and flowing quality of the movement.
  2. Chorea is a type of hyperkinetic movement disorder.
  3. The involuntary movements flow from one body area to another which cannot be predicted beforehand.

Etiology[edit | edit source]

Chorea traditionally is described in Huntington disease, however other disorders that can lead to chorea, for example: Spinocerebellar ataxia subtypes; Wilson disease; Sydenham chorea, and structural disease of the basal ganglia. Generally, the root of the pathology is in the basal ganglia.[1]

They are predominantly of 2 types[2]

  • Acquired Causes
  • Genetic Causes
Acquired Causes of Chorea


Genetic Causes of Chorea


Phenomenological Features[edit | edit source]

  • Randomness
  • Flowing Quality
  • Parakinesia: Patients blend their chorea-induced movements with their own normal movements.
  • Motor impersistence: the patient is unable to perform sustained motor activities.
  • Ballism: Variant of chorea which shows large-amplitude flinging movements involving proximal extremities.
  • Athetosis: Slow writhing movements involving distal limbs sometimes may be facing.
  • Varying velocity of movements: quick velocity and low-amplitude movements accompanied by jerks.[2]

This video displays the dance-like movements performed by a patient.[3]

Diagnosis[edit | edit source]

The phenomenological features mentioned above are observed to make a diagnosis of chorea.

Differential Diagnosis[edit | edit source]

Specific diagnosis can be made on the basis of symptoms/clinical features experienced by the patient.[2]Differential diagnoses include:

Multimodal Management[edit | edit source]

Mutimodal management chorea.jpg

Chorea requires a Multidisciplinary Approach to be treated.[4]

The team includes:

  • Psychiatrist
  • Physical medicine and Rehabilitation Specialist
  • Physical therapist
  • Occupational therapist
  • Speech therapists
  • Geneticist
  • Genetic Counselor
  • Social worker

Medical Management[edit | edit source]

There is no cure for this disease but symptoms can be managed. Importantly an evaluation and creation of a patient's support system is needed. With disease progression, the patient will need specialized care. Nutrition management is important, due to difficulty swallowing.[1]

1. Dopamine-depleting agents: Tetrabenazine, Deutetrabenazine, Valbenazine.

2. Dopamine D2 receptor-blocking agents: Haloperidol, Clozapine, Olanzapine.

3. Anticonvulsants: Valproic acid, Carbamazepine, Benzodiazepines, Levetiracetam.

4. Anti-glutamatergic agents: Amantadine, Riluzole.

5. Cannabinoids: Nabilone.

6. Deep Brain Stimulation

Physiotherapy Management[edit | edit source]

The management will be same as of Huntington's Disease.

Goals of Management[edit | edit source]

References[edit | edit source]

  1. ↑ 1.0 1.1 1.2 1.3 Merical B, Sánchez-Manso JC. Chorea. Available: https://www.ncbi.nlm.nih.gov/books/NBK430923/#!po=15.0000(accessed 19.9.2022)
  2. ↑ 2.0 2.1 2.2 Termsarasab P. Chorea. CONTINUUM: Lifelong Learning in Neurology. 2019 Aug 1;25(4):1001-35.
  3. ↑ neurosigns.org. Chorea. Available from https://www.youtube.com/watch?v=RxWEilu-Mf4&ab_channel=neurosigns.org
  4. ↑ Feinstein E, Walker R. An update on the treatment of chorea. Current Treatment Options in Neurology. 2018 Oct;20(10):1-5.
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