Basal ganglia volume is strongly related to P3 event-related potential in premanifest Huntington's disease.

Background:  The P3 event-related potential (ERP) is presumably partly generated by the basal ganglia. Because degeneration of these brain structures starts many years before clinical disease onset in Huntington’s disease (HD), studying the interplay between P3 characteristics and basal ganglia volumes in ‘premanifest’ carriers might lead to new insights into the disease process. Methods:  Fourteen premanifest\ HD mutation carriers and twelve non-mutation carriers underwent clinical, MRI and P3-ERP investigations. The P3 was measured during the Sustained Attention to Response Task. Results:  P3 amplitude and latency did not differ between groups. In carriers, longer P3 latency during Go-trials was strongly associated with smaller caudate, putamen and globus pallidus volumes (r values up to −0.827, P ≤ 0.001). Conclusion:  The exceptionally strong relations of P3 latency with basal ganglia volumes in carriers suggest that the P3 may provide a marker for disease progression in HD.