Columbia University Medical Center   discover care educate lead hdsa center of excellence    
at the NY state psychiatric institute

If you notice any problems with the website, please email site editor.

Two major problems facing people with Huntington's disease that change quality of life:

Loss of executive function:

This cognitive impairment includes the inability to plan and initiate an activity, the inability to switch from one plan to another, diminished verbal fluency, and perseverative behaviors.

APPROACH: Patients with HD should be presented with two choices instead of open-ended questions. They should be presented with an activity and encouraged to begin with verbal prompting. To overcome apathy, non-verbal modeling can be a helpful trigger to start. Interest in the activity can be sustained if the caregiver also participates in the activity. Perseveration requires distraction, coaxing onto another topic, change of place, and a positive response. Repeated demands by the patient should be addressed as quickly as possible to avoid escalation to angry outbursts, as calmly as possible to get the person's attention, and with a positive response. A refusal will not stop perseveration. For example: a patient with HD wants a cigarette before the scheduled time. Instead of saying, "No. It's not time yet," a better response is "Yes, you can. I'll give you a cigarette in 15 minutes. Why don't you go ahead to the room, and I'll meet you there."

Social implications of abnormal movements:

There are two categories of abnormal movements: abnormal involuntary movements (AIMs) and loss of volitional control. Abnormal involuntary movements include dance-like movements (chorea), twisting or sustained postures (dystonia), rigidity, motor restlessness, tremor and jerky movements (myoclonus). Loss of volitional control, including motor impersistence, tends to be more disabling. Interrupted saccadic and smooth pursuit, and uncoordinated speech, swallowing, grasp and gait with AIMs make the person appear awkward, drunk, boisterous, cognitively and behaviorally more impaired than they actually are. When strangers react with curiosity and families with embarrassment, this intensifies anxiety and amplifies movements. Staying home, only attending events with close friends and family, abandoning favorite group activities in favor of solitary ones may reduce the patient's anxiety but the net result is social isolation, guilt, boredom, loneliness, grief and loss for the person with HD and increased burden expressed by the caregiver family.

APPROACH: Anxiolytics and anti-depressants have helped people resume favorite activities and accept the risk but not assume the inevitability of social embarrassment. Structured group activities--dancing, martial arts, exercise, swimming, hiking and camping--allow re-entry into a social life outside the family and provide respite for caregivers.



Copyright 2000-2009 HDNY.ORG : At NY State Psychiatric Institute
Columbia University Medical Center : Department of Neurology : Last updated 27-Jun-2009