eMedicine Specialties > Psychiatry > Adult

Huntington Disease Dementia: Follow-up

Author: Idan Sharon, MD, Consulting Staff, Departments of Neurology and Psychiatry, Cornell New York Methodist Hospital; Private Practice
Coauthor(s): Tulay Ersan, MD, Chief of Geriatrics, Department of Internal Medicine, Division of Geriatrics, Monmouth Medical Center; Roni Sharon, University of Michigan
Contributor Information and Disclosures

Updated: Aug 20, 2007

Follow-up

Prognosis

  • HD has a great impact on patients' physical and psychosocial well-being, the latter being more severely affected. Even though the symptoms of HD are fairly well characterized, their progression, especially in the early and middle stages, remains unpredictable. With the approach of late-stage HD, affected individuals begin to experience speech difficulty and weight loss. In the late stage, patients lose bowel and bladder control. Clarification of disease progression is vital to improved understanding of the pathogenesis of HD and to the evaluation of therapeutic agents that are designed to slow the progression of disease.
  • In longitudinal analyses, longer disease duration and better neuropsychological performance at baseline were associated with a less rapid rate of decline in the Total Functional Capacity Scale score, whereas depressive symptomatology at baseline was associated with a more rapid decline in the Independence Scale score. These rates of functional decline and the co-variates that modify them should be considered in estimating statistical power and designing future therapeutic trials involving patients with HD who have early or moderately severe disease.

Patient Education

  • Genetic testing has been available for HD for longer than any other adult-onset genetic disorder.
    • Predictive genetic testing presents unique issues in the legal and ethical debate concerning disclosure of information within the physician-patient relationship.
    • A duty to disclose information to family members has been found when the disclosure is likely to result in the ability to mitigate the damaging effects of the disease.
    • When evaluating a situation in which an individual is at risk of HD, the analysis must be different and necessitates an ethical and legal examination of the consequences of receipt of the information on family members, ie, those known to be at risk but who may not know they are at risk of inheriting a genetic disease.
    • The situation presented by HD is unique and demands a different framework for analysis, given the late onset and lack of curative or ameliorative treatment. Also, the ethical standards should be invoked when considering violating the privacy of a patient or a family member. The principles of autonomy and self-determination of family members are considered, compared with the risk of harm and the privacy interest in not knowing potentially devastating information.
    • The discovery of the genetic mutation causing HD made possible the use of predictive testing to identify currently unaffected carriers. Concerns have been raised that predictive testing may lead to an increase in deaths by suicide among identified carriers.
  • A fact that might be comforting to patients with HD (or family members) is the significantly lower prevalence of cancer among these patients. The lower prevalence of cancer among patients with HD seems to be related to intrinsic biologic factors. One explanation may be that the modified protein, huntingtin, encountered in patients with HD protects against cancer by inducing or increasing the rate of naturally occurring programmed cell death in preneoplastic cells.
  • When possible, the patient and the family members/caregivers should be prepared for the HD progression, from early involuntary movements and emotional changes to more overt motor symptoms and difficulty with activities of daily living.
  • Family therapy, support groups, and caregiver education is extremely important due to the degenerative and emotionally exhausting course of the disorder.  Family therapy may encourage genetic testing and provide for support functionality to the families and caregivers. It provides for a deeper understanding of the condition and ways to deal with the deterioration as it comes about.
  • For excellent patient education resources, visit eMedicine's Dementia Center. Also, see eMedicine's patient education articles Huntington Disease Dementia, Dementia Overview, and Dementia Medication Overview.

Miscellaneous

Medicolegal Pitfalls

Because the general appreciation of the role of genes in diseases is growing, the demand for genetic counseling has increased. Patients who are concerned about their own risk for a genetic disorder or about the risk of genetic disease (eg, HD) in their offspring should consult their physician with questions and expect explanations; therefore, all physicians should be familiar with the principles of medical genetics and should use this knowledge to counsel patients, which may help avoid medical or legal problems.

  • The essentials, explained in language understandable to those being counseled, provides effective communication.
  • Written notes and diagrams frequently are helpful.
  • Summary letters and reviews at subsequent visits help to correct misconceptions and to increase retention of the information.

Special Concerns

Adolescents who are actively requesting HD predictive testing of their own accord pose dilemmas to those providing testing. Asymptomatic adult children at risk for HD should receive careful genetic counseling prior to DNA testing because a positive result may have serious emotional and social consequences. Adhering to published testing and counseling protocols is recommended.

 


More on Huntington Disease Dementia

Overview: Huntington Disease Dementia
Differential Diagnoses & Workup: Huntington Disease Dementia
Treatment & Medication: Huntington Disease Dementia
Follow-up: Huntington Disease Dementia
References
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Further Reading

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Keywords

Huntington disease, dementia, HD, Huntington's disease, movement disorders, progressive dementia, psychiatric illness, behavioral disturbance, mental illness, psychosis, depression, schizophrenia, benign familial chorea, inherited ataxias, neural acanthocytosis, familial Alzheimer disease, AD, Parkinson disease, Parkinsonism, PD

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Contributor Information and Disclosures

Author

Idan Sharon, MD, Consulting Staff, Departments of Neurology and Psychiatry, Cornell New York Methodist Hospital; Private Practice
Idan Sharon, MD is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Medical Society of the State of New York
Disclosure: Nothing to disclose.

Coauthor(s)

Tulay Ersan, MD, Chief of Geriatrics, Department of Internal Medicine, Division of Geriatrics, Monmouth Medical Center
Tulay Ersan, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Geriatrics Society, and American Medical Association
Disclosure: Nothing to disclose.

Roni Sharon, University of Michigan
Disclosure: Nothing to disclose.

Medical Editor

Alan D Schmetzer, MD, Professor and Vice-Chair for Education, Department of Psychiatry, Director of Residency Training, Indiana University School of Medicine
Alan D Schmetzer, MD is a member of the following medical societies: American Medical Association and American Psychiatric Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

David Bienenfeld, MD, Vice-Chair, Program Director, Professor, Department of Psychiatry, Wright State University School of Medicine
David Bienenfeld, MD is a member of the following medical societies: American Medical Association, American Psychiatric Association, and Association for Academic Psychiatry
Disclosure: Nothing to disclose.

CME Editor

Harold H Harsch, MD, Program Director of Geropsychiatry, Department of Geriatrics/Gerontology, Associate Professor, Department of Psychiatry and Department of Medicine, Froedtert Hospital, Medical College of Wisconsin
Harold H Harsch, MD is a member of the following medical societies: American Psychiatric Association
Disclosure: lilly Honoraria Speaking and teaching; BMS Honoraria Speaking and teaching; Forest Labs Honoraria Speaking and teaching; AstraZeneca Honoraria Speaking and teaching; Pfizer Grant/research funds Other; Northstar Grant/research funds Other; Novartis  Other

Chief Editor

Stephen Soreff, MD, President of Education Initiatives, Nottingham, NH; Faculty, Metropolitan College of Boston University, Boston, MA
Stephen Soreff, MD is a member of the following medical societies: American College of Mental Health Administration
Disclosure: Nothing to disclose.

 
 
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