RISK FACTORS Family history is a definite risk factor. Although a small percent of patients with DNA-proven Huntington disease will have a negative family history.
GENERAL MEASURES
β’ Genetic counseling
β’ Symptomatic treatment (dopamine receptor blocking drugs), such as phenothiazines or haloperidol
β’ Consider electroconvulsive therapy (ECT) for drugresistant depression
β’ Speech and occupational therapy
DIET No special diet, but soft diet with liquid supplements may be needed. Coenzyme Q10 (Ubiquinone),
a popular vitamin supplement, shows promise in reversing the generalized energy defect in HD.
DRUG(S) OF CHOICE
β’ For dyskinesia and/or behavioral problems: Haloperidol (Haldol) 1 mg bid and increased every 3 or 4 days until satisfactory response. Suggested daily maximum 10 mg.
β’ For choreoathetosis: clonazepam (Klonopin), start at 0.5 mg q hs, increasing over several months to 9 mg
maximum in a divided daily dose; reserpine, start at 0.1 mg/day, increase at 7-10 day intervals to 3 mg/day
maximum; tetrabenazine, start at 12.5 mg/day, increase by 12.5 mg every 7 days to 25 mg qid maximum
β’ For rigidity: baclofen (Lioresal), start at 10 mg/day, increase slowly to 120 mg maximum in a divided dose.
May combine with clonazepam.
β’ For depression: fl uoxetine (Prozac), start at 10 mg/day, increase by 10 mg increments to 60 mg/day maximum
ALTERNATIVE DRUGS
β’ Presynaptic dopamine-depleting agents
β’ Postsynaptic dopamine antagonists
β’ Tricyclic antidepressants
β’ Antipsychotics
PATIENT MONITORING
β’ Periodically for behavioral changes
β’ Effect of drug therapy may be monitored using the Unifi ed Huntington Disease Rating Scale, a protocol by which patients are evaluated on a series of motor, behavioral and functional criteria.
PREVENTION/AVOIDANCE
β’ Genetic counseling
β’ Smoking cessation in late (choreiform) stage
POSSIBLE COMPLICATIONS
β’ Choking
β’ Subdural hematoma
β’ Personality changes
β’ Suicide
EXPECTED COURSE/PROGNOSIS Poor, progressive impairment, fatal outcome within 20 years, usually from pneumonia