![]() Offspring of an individual with a pathogenic variant have a 50% chance of inheriting the disease-causing allele. HD is inherited in an autosomal dominant manner. 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 problems, 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 antiepileptic drugs for psychiatric disturbances (depression, psychotic symptoms, outbursts of aggression) valproic acid for myoclonic hyperkinesia. The diagnosis of HD rests on positive family history, characteristic clinical findings, and the detection of an expansion of 36 or more CAG trinucleotide repeats in HTT. The mean age of onset is 35 to 44 years, and the median survival time is 15 to 18 years after onset. Huntington disease (HD) is a progressive disorder of motor, cognitive, and psychiatric disturbances.
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