What is Huntington’s Chorea?
Huntington’s Chorea is a progressive degenrative disease affecting basal ganglia characterized by choeiform movements (movements such as writhing, twisting, and turning in a constant, uncontrollable dancing motion) and mental deterioration.
What causes Huntington’s Chorea?
The cause is believed to be genetic with autosomal dominance transmission from parent to offspring with full penetrance.
In Huntington’s Chorea, there is widespread degeneration changes with cell loss and reactive gliosis mainly in the cerebral cortex (thinking and perception), basal ganglia (balance) and caudate nucleus (co-ordination).
There is the belief that the deficiency of gamma-aminobutyric acid (GABA) in the cells may have contribute to the impairment of the nerve cells.
Who is at risk of Huntington’s Chorea?
Huntington’s Chorea affects sexes in equal numbers.
It is an inherited condition. On average 50 per cent of children of the sufferers will be affected.
What are the symptoms of Huntington’s Chorea?
The typical course of Huntington’s Chorea usually appear between 30 to 45 years but may be earlier or later.
1.choreiform movements (movements such as writhing, twisting, and turning in a constant, uncontrollable dancing motion )
2.emotional disturbance with mental changes
4.mood swings with inertia followed by irritability
8.delusion and hallucinations
9.The speech can become slurred and vital functions, such as eating, speaking, swallowing and especially walking, begin to decline.
10.All the above may occur to varying degree but rate of progression is generally parallel.
How is the diagnosis of Huntington’s Chorea made?
The diagnosis of Huntington’s Chorea involve:
1.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 and putamen. In addition there are enlargement of fluid-filled cavities within the brain called ventricles. These tests do not completely differentiate Huntingson’s Chorea from other conditions such as dementia but they will together with the choreiform movements,emotional and mental impairment point towards Huntingdon’s chorea.
What are the complications for Huntington’s Chorea ?
1.Progression of nerve involvement to whole body with paralysis
2.Dementia may gradually result from mental impairment
What is the treatment for Huntington’s Chorea?
The is no cure for Huntington’s Chorea but symptomatic control of choreiform movements and delusions with haliperidol or clonazepam may help.
Proper nutrition , fluids and exercise will help the patient to stay healthy and fit.
As the condition progress hospitalization or institutional care (such as nursing homes) is usually indicated.
Genetic counseling is important for treatment and prevention of
What is the prognosis of Huntington’s Chorea?
Except for a few cases there is a relentless progression to death.
Death usually occurs in 10 to 15 years although the course may more acute or prolonged.