A Family Study of Huntington's Chorea with Unusual Manifestations

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Abstract
A family with Huntington''s Chorea has been observed with 3 affected members showing unusual manifestations of the disease. The propositus showed extreme rigidity, dystonia and athetosis, while one of his sisters had the classical features as well as mild cogwheeling of the upper extremities. In addition, a 12 year old cousin was found to have mild but typical choreiform movements and emotional lability of recent onset. Extrapyramidal rigidity as a form of Huntington''s Chorea may occur anywhere along the course of the illness and is more frequently present in cases with onset in early life. In some typical cases, the choreiform movements may evolve into athetosis, and torsion-dystonia and athetosis may appear. A significant number of cases begin before the age of 25 years, and it is in this group that establishing a definite diagnosis may be very difficult. Pyramidal tract signs are seen in a small number of cases and may accompany extrapyramidal rigidity.

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