[Psychometric study in Huntington disease]

Thirty-two patients suffering from Huntington's disease (HD) were tested with a short neuropsychological tests battery in order to make a global evaluation of their cognitive functions, and to ascertain whether the tests would be sensitive enough, even in the first stages of the disease, and whatever the individual neuropsychological profile, to be, possibly, an assessment tool for treatment efficacy. Nineteen HD presented with a "mild" form of the disease [stage I and II of Shoulson and Fahn (1979)], 7 men and 12 women, with a duration of illness of nearly 5 years and 13 HD presented with a "moderate" form (stage III and IV), 5 men and 8 women, with a duration of illness of nearly 8 years. These 2 groups had a comparable age at the assessment time (nearly 47) and did not differ, significantly, for the age at onset (39 and 43 respectively), duration of illness or psychiatric disorders. Each test result was reduced to its number of standard-deviations apart the normal mean-value for the same sex, age and study level group.(ABSTRACT TRUNCATED AT 250 WORDS)