Huntington’s Disease: A Primer on Chorea

Huntington’s Disease

Huntington’s disease (HD) is an incurable and progressive neurogenic disorder.^1,2 It is categorized as a movement disorder, alongside Parkinson’s disease, Lewy body dementia, multiple system atrophy, and progressive supranuclear palsy, and is the most common hereditary neurodegenerative disorder.^3,4 Age of onset ranges from childhood to the eighth decade of life; the most common subset of patients being in their 30s and 40s with a mean duration of 15 to 20 years from the time of onset to death. ⁵ A small subset of patients present with symptoms before the age of 20, termed juvenile HD.⁶

The advanced stage of HD can last for a decade or longer. Complications of immobility, such as aspiration pneumonia and other infections, result in death 10 to 40 years after disease onset.⁷ In adults, HD progresses over time in a triad of cognitive, psychiatric, and motor changes, especially chorea.^3,6 Patients with juvenile-onset HD typically present with cognitive and psychiatric features at the time of onset; these patients will develop some motor changes over time; however, chorea is notably absent in most.⁶ Regardless of disease onset or stage, there are no curative or disease-modifying treatments currently available, making supportive care the focus of therapy.^3,8

Table 1: Symptoms of Huntington Disease⁶

For more information on the natural history and symptoms of HD, refer to the Healthcare Professional Resources section of the Huntington’s Disease Society of America (HDSA) website.⁹ A non-CE on-demand course, HD 101 Series, is hosted there. This video series was recorded in 2013; however, it provides basic and useful information for clinicians unfamiliar with the disease.¹⁰

Chorea Overview

Chorea, considered the hallmark symptom of HD, is described as brief, abrupt, involuntary movements that develop in the limbs (arms and legs), face, and trunk that inevitably impact gait and balance. Chorea first manifests as short-lived and suppressible fidgety movements in the distal extremities; considered mild in severity, it is often misinterpreted as restlessness.^1,2,7 Over time, chorea typically becomes severe and leads to falls and exhaustion. As the disease continues to advance, chorea may lead to burn out and the more difficult to treat motor signs and symptoms like dystonia, rigidity, bradykinesia, gait problems, and loss of balance. Difficulty swallowing and speaking are also observed in later stages.^1,2,7

Chorea can be quantified for purposes of assessment of symptom improvement and/or disease progression using The Unified Huntington’s Disease Rating Scale (UHDRS). Determine the score by assessing the severity of chorea in each body region. The total chorea score is the sum of each region. A UHDRS total score of greater than 8, or chorea that interferes with function (resulting in injury, falls, difficulty with speech and swallowing, and poor sleep, and contributing to pain and weight loss), usually indicates need for medication management.^1,10

Table 2: The Unified Huntington’s Disease Rating Scale¹⁰

Behavioral/Non-Pharmacologic Management of Chorea

Chorea management is patient-specific—the impact of symptoms vary by patient and disease stage. Symptoms are initially progressive but are thought to peak at approximately 10 years after onset. Symptoms may plateau and then lessen in some patients, while others find that symptoms continue to worsen.² In late-stage HD other symptoms (e.g., bradykinesia or rigidity) may be more prominent than chorea.

Chorea that is not debilitating or bothersome may not require medication management. A patient’s overall goals of care should also be considered as the medications used to treat chorea may worsen other symptoms of HD (e.g., parkinsonism, depression). Employ the behavioral and non-pharmacologic interventions outlined below^1,8 throughout the course of disease and reassess chorea at regular intervals for the necessity of initiation or adjustment of medication therapy.

• Patient/caregiver education.
• Assessments of family/caregiver coping and psychosocial needs.
• Physical therapy, including gait/balance training.
• Occupational therapy, including home modifications, and orthotics and leg weights to assist with upright posture.
• Speech therapy.
• Strict routines to lessen anxiety, short-term memory deficits, intrusive thoughts, and fear of abandonment.
• Calm, predictable, and structured environment along with management of mood disorders to improve anxiety and stress.
• Assistive equipment such as helmets, padded reclining chairs, low beds, and protective padding of the environment, especially in severe chorea.
• Advance care planning (documentation of decisions on medical power of attorney and patient preferences regarding life-sustaining treatment such as mechanical ventilation, nutrition, hydration, and cardiopulmonary resuscitation) as early as possible before the onset of cognitive impairment.

Medication Management of Chorea

Medication choice is based on individual patient comorbidities and medication side effect profiles. Vesicular monoamine transporter 2 (VMAT2) inhibitors (e.g., tetrabenazine, deutetrabenazine) and atypical antipsychotics (e.g., risperidone, olanzapine) are the primary drug classes used to manage symptomatic patients.¹ A generalized approach is to assess the patient for comorbidities that might also be managed with antipsychotics (e.g., agitation, psychosis) and for conditions with contraindications to antipsychotics or VMAT2 inhibitors. Avoid VMAT2 inhibitors in patients with depression as it is a significant side effect associated with this medication class.

Vesicular Monoamine Transporter Type 2 (VMAT2) Inhibitors^1,11

• Consider as first line to treat chorea associated with HD and no history of depression.
  • VMAT2 inhibitors carry a boxed warning against use in patients with concurrent depression as they are associated with an increased incidence of depression and suicidal ideation.
• Prohibitive costs and product availability issues may dissuade their use. Tetrabenazine is the least costly.
• Common side effects include sedation, akathisia, orthostatic hypotension, and depressed mood.
• May cause reversible parkinsonism (not tardive dyskinesia, as associated with antipsychotics).
• If a patient is switching from a VMAT2 inhibitor to an antipsychotic, it is recommended to gradually taper the VMAT2 inhibitor after the antipsychotic therapy is initiated.¹

Atypical Antipsychotics^1,11

• Consider as first line therapy for chorea in patients with a history of depression.
• Doses may require titration to achieve symptom management.
• Clozapine and quetiapine are not recommended for chorea as they require high doses (> 400mg/day) to be effective.
• First generation (conventional) antipsychotics like haloperidol and chlorpromazine are reserved for more severe symptoms due to their side effect profiles.

Other Medications

• Short-term use of benzodiazepines:¹
  • Clonazepam or lorazepam may decrease severe episodes of chorea, however not considered useful or well tolerated as long-term therapy.
  • These medications may worsen cognition and there are no studies to determine dosing—use clinical judgment before initiating.
• Limited or inconsistent evidence of efficacy exists for treating chorea with cannabinoids, amantadine, and anticonvulsants. Further study is needed to determine benefit and side effect impact in HD:¹
  • Cannabinoids – Guidelines from the American Academy of Neurology concluded that nabilone may modestly improve HD chorea.¹² Nabilone is a controlled substance with a potential for dependency and not available in the U.S.
  • Amantadine – Its utility for chorea in patients with HD is uncertain as it can worsen cognitive function, particularly at high doses (e.g., ≥ 300mg/day).
  • Anticonvulsants – Levetiracetam reduced chorea in a small open-label study.¹³ Other anticonvulsants, such as topiramate, have been reported to improve chorea in case reports, however not specifically HD chorea.¹⁴

Summary and Recommendations

• Non-pharmacologic interventions for chorea include patient/caregiver education, family/caregiver support, PT, OT and speech therapy. In addition, establishing routines and a predictable and structured environment will help lessen the anxiety and stress that can exacerbate chorea, and the use of assistive equipment and protective padding will help ensure safety.
• Address advance care planning as early as possible before the onset of cognitive impairment.
• Reserve medication therapy of chorea for patients with symptoms that interfere with functioning, as treatment may worsen other aspects of HD, including parkinsonism, cognition, and mood.¹
• VMAT2 inhibitors are cost-prohibitive for most patients—switching therapy within the class to tetrabenazine may help lower costs.
• Management of chorea is highly individualized, and it is important to continually assess the appropriateness of both non-pharmacologic and medication therapy interventions as HD progresses to the advanced stage.

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REFERENCES

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14. Gatto EM, Uribe Roca C, Raina G, et al. Vascular hemichorea/hemiballism and topiramate. Mov Disord 2004;19:836.