Abnormalities of the contingent negative variation in Huntington's disease: correlations with clinical features

The contingent negative variation (CNV) is a neurophysiological pattern related to planning of external - paced, voluntary movements. The aim of the study, was to examine the CNV in a cohort of mild demented and non-medicated HD patients, evaluating the CNV amplitude modifications in the light of clinical features and performing Low Resolution Brain Electromagnetic Tomography (LORETA) analysis in order to show the CNV multiple generators. Fourteen HD patients and 25 sex and age-matched controls were studied. All subjects were evaluated by the motor section of UHDRS, MMSE and WAIS. The CNV was recorded by 19 scalp electrodes, with a red light flash as visual warning stimulus (S1), followed by a blue light flash (S2) after a fixed interval of 3 s. The amplitude of early CNV was significantly reduced in HD, compared to controls: the amplitude reduction was significantly correlated with the bradikinesia score. LORETA analysis of early CNV significantly discriminated patients from controls, for a prevalent activation of the posterior part of anterior cingulate cortex in HD. An abnormal activation of the associative cortex devoted to the processing of attention preceding voluntary movement may be supposed in HD, probably mediated by the altered basal ganglia modulation.