HUNTINGTON’S CHOREA

(Chronic progressive chorea)

DEFINITION OF HUNTINGTON’S CHOREA

HUNTINGTON’S CHOREA is a hereditary disease characterised by choreiform movement and progressive dementia caused by degeneration of corpus striatum and the ganglion cells of the forebrain.

AETIOLOGY AND PATHOLOGY

The disease is inherited as an autosomal dominant. Responsible gene is identified on chromosome 4 in its short arm. At 4p 16.3 there is an expanded and unstable CAG trinucleotide repeat. The cause of this disease is unknown. The disease continues from one generation to the next one. Heredity plays an important part. There is atrophy of the cerebral cortex, corpus striatum, caudate nucleus and putamen. Massive depletion of enzyme synthesizing Acetyl choline and GABA is seen in corpus striatum. There is also depletion of GABA, ACE and metencephalin in substantia nigra. Somatostatin level of corpus striatum is high.

CLINICAL FEATURES

Onset is insidious.

Age 30-45 years.

Sex: Both.

First symptom is jerky involuntary movement of the face and upper extremity. Gradually dysarthria and ataxia develop and finally the patient becomes demented. The earliest mental change is behavioural disorder with irritability, antisocial behaviour or moodiness. Progressive rigidity and akinesia may also develop in association with dementia.

INVESTIGATIONS

CT scan shows cerebral atrophy and atrophy of caudate nucleus. MRI and Positron emission tomography (PET) show reduced glucose utilisation by caudate nucleus though anatomically this is normal.

PROGNOSIS

Death usually occurs after 15 years.

TREATMENT OF HUNTINGTON’S CHOREA

Symptomatic treatment may be helpful. Phenothiazines may be given. Tetrabenazine 25 mg thrice daily may also control chorea. Reserpine has similar effect. Haloperidol can be given in a dose of 1 gm once or twice daily.

HUNTINGTON’S CHOREA

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