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The relation of type of initial symptoms and line of transmission to ages at onset and death in Huntington's disease
Author(s) -
Brackenridge C. J.
Publication year - 1971
Publication title -
clinical genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.543
H-Index - 102
eISSN - 1399-0004
pISSN - 0009-9163
DOI - 10.1111/j.1399-0004.1971.tb00289.x
Subject(s) - chorea , psychology , disease , pediatrics , huntington's disease , age of onset , medicine , psychiatry
To determine whether the age at onset and age at death were related to the type of initial symptoms of Huntington's disease, analyses of variance with respect to (A) order of appearance of neurological and psychiatric symptoms, (B) type of muscular disorder (chorea or rigidity), (C) sex of affcctcd subject, (D) sex of transmitting parent, and (E) sex of transmitting grandparent were calculated. Agc at onset and age at death were each significantly affected by order of symptoms, neurological sign, sex of subject, and sex of affected parent. The duration of illness depended only on the order of neurological and psychiatric symptoms. Fathcrs exceeded mothers as transmitting parents of sibships with one or more affected subjects. The proportions of the four parental‐grandparental lines of transmission differed significantly among subjects grouped according to type of first symptoms but not according to muscular signs. The ratio of choreic to rigid cases varied significantly with the order of symptoms and sex of the subject. The earlier ages at onset and death of subjects with Westphal (rigid‐hypokinetic) symptoms than those with typical choreic movements confirm the validity of distinguishing between these variants of the disorder.