Huntington’s Chorea, A Simple Guide To The Condition, Treatment And Related Conditions

Huntington's Chorea is a progressive degenerative disease affecting basal ganglia characterized by choreiform movements and mental deterioration. The cause is believed to be genetic with autosomal dominance transmission from parent to offspring with full penetrance. In Huntington's Chorea, there are widespread degeneration changes with cell loss and reactive gliosis mainly in the cerebral cortex, basal ganglia and caudate nucleus.
There is deficiency of gamma-aminobutyric acid (GABA). Physical symptoms of Huntington's disease can begin at any age from infancy to old age but usually begin between 30 and 45 years of age.1. Choreiform movements2. Emotional disturbance with mental changes3. Cognitive impairmentThe diagnosis of Huntington's Chorea involve1. Typical history of choreiform movements, emotional and mental impairment with family history.2.
Physical and neurological examinations for brain neurological deficit.3. CT scan or MRI may show selective atrophy of the caudate nucleus helping to differentiate Huntingdon's Chorea from other causes such as dementia. There is no cure for Huntington's Chorea but symptomatic control of choreiform movements with haliperidol or chlorpromazine may help. Tetrabenazine was approved in 2008 for treatment of chorea in Huntington's disease in the US.
Other drugs that help to reduce chorea include neuroleptics and benzodiazepines. Newer agents, such as quetiapine, clozapine, olanzapine, and risperidone, are preferred to older agents because of the lower incidence of extrapyramidal side effects and the decreased risk for tardive syndromes. Results of some studies have suggested that valproic acid and clonazepam may be effective in the treatment of chorea.
Patients with Huntington's disease may see a physical therapist for non-invasive and non-medication-based ways of managing the physical symptoms. TABLE OF CONTENTIntroductionChapter 1 Huntington's ChoreaChapter 2 More Facts of Huntington's ChoreaChapter 3 Treatment of Huntington's ChoreaChapter 4 Cerebral PalsyChapter 5 Parkinson's DiseaseChapter 6 DementiaChapter 7 SeizuresEpilogueOde to Huntington's ChoreaHuntington's Chorea is a progressive degenerative brain conditionThe affected basal ganglia causes choreiform movements and mental deteriorationIt is adult-onset, autosomal dominant inherited disorderMost adult-onset Huntingtons inherit the gene from their mothersThe juvenile Huntingtons inherit the gene from their fathersInheritance through the father can lead to the disease onset earlierThe most important symptom is the choreiform movementsThere is usually emotional disturbance and cognitive impairmentDiagnosis is from the history of chorea and poor mental abilitiesCT scan or MRI may show the caudate nucleus having selective atrophyThere is no cure for Huntington's ChoreaMental impairment may finally lead to dementiaTreatment is with medications to control the symptomsPsychotic drugs are given for psychosis and behavioral problemsDrugs that help to reduce chorea include TetrabenazineOther drugs that help include neuroleptics and benzodiazepines-An original poem by Kenneth Kee