![]() Difficulty in initiating ocular saccades, slow and hypometric saccades, and problems in gaze fixation may be seen in up to 75% of symptomatic individuals. Oculomotor disturbances occur early and worsen progressively. Motor speed, fine motor control, and gait are affected. Affected individuals and their families describe clumsiness in common daily activities. ![]() Impairment in voluntary motor function is an early sign. With advancing disease duration, other involuntary movements such as bradykinesia, rigidity, and dystonia occur. The choreic movements are continuously present during waking hours, cannot be suppressed voluntarily, and are worsened by stress. Chorea is present in more than 90% of individuals, and typically increases in severity during the first ten years. Chorea, an involuntary movement disorder consisting of nonrepetitive, nonperiodic jerking of limbs, face, or trunk, is the major sign of the disease. Disturbances of both involuntary and voluntary movements occur in individuals with HD. The average age at death is 54 to 55 years. The median survival time after onset is 15 to 18 years (range: 5 to >25 years). In late stages of HD, motor disability becomes severe and the individual is often totally dependent, mute, and incontinent. The impairment is usually considerable, sometimes with intermittent outbursts of aggressive behaviors and social disinhibition. Most individuals are forced to give up their employment and depend increasingly on others for help, although they are still able to maintain a considerable degree of personal independence. In the next stage, chorea becomes more prominent, voluntary activity becomes increasingly difficult, and dysarthria and dysphagia worsen. These individuals have chorea, gait disturbances, and dysphagia, but a more prolonged and benign course than the typical individual. In approximately 25% of individuals with HD, the onset is delayed until after age 50 years, with some after age 70 years. Affected individuals are usually able to perform most of their ordinary activities and to continue working. In the early stages following diagnosis, manifestations include subtle changes in eye movements, coordination, minor involuntary movements, difficulty in mental planning, and often a depressed or irritable mood (see Table 3). ![]() About two thirds of affected individuals first present with neurologic manifestations others present with psychiatric changes. The mean age of onset for HD is approximately 45 years. Requires motor DCL = 4, plus cognitive changes Other: Children and adolescents with a parent with HD may benefit from referral to a local HD support group for educational materials and psychological support. ![]() Surveillance: Regular evaluations of the appearance and severity of chorea, rigidity, gait abnormalities, depression, behavioral changes, and cognitive decline routine assessment of functional abilities using the Behavior Observation Scale Huntington (BOSH) and the Unified Huntington's Disease Rating Scale (UHDRS).Īgents/circumstances to avoid: L-dopa-containing compounds (may increase chorea), alcohol consumption, smoking. Prevention of secondary complications: Attention to the usual potential complications in persons requiring long-term supportive care and to side effects associated with pharmacologic treatments. Supportive care with attention to nursing needs, dietary intake, special equipment, and eligibility for state and federal benefits. Treatment of manifestations: Pharmacologic therapy including typical neuroleptics (haloperidol), atypical neuroleptics (olanzapine), benzodiazepines, or the monoamine-depleting agent tetrabenazine for choreic movements anti-parkinsonian agents for hypokinesia and rigidity psychotropic drugs or some types of anti-seizure medication for psychiatric disturbances (depression, psychotic symptoms, outbursts of aggression) valproic acid for myoclonic hyperkinesia.
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