Longitudinal Neuropsychological and Genetic Linkage Analysis of Persons at Risk for Huntington's Disease

Verbal episodic memory declines prior to diagnosis in Huntington's disease

“…These findings replicate similar previous results (Berrios et al, 2002;Campodonico et al, 1998;Hahn-Barma et al, 1998;Lemiere et al, 2004), but are in contrast to others (de Boo et al, 1999;Giordani et al, 1995;Rothlind et al, 1993;Strauss & Brandt, 1990). Given that effect sizes were small in the CL0 group and medium in the CL1 group, previous null findings may well reflect inadequate power to detect these subtle effects, especially in samples that include only individuals with a near or total absence of motor signs or who are very far from estimated age of onset.…”

“…Comparatively few studies have directly assessed verbal episodic memory in pre-HD, and the results have been mixed. In several studies, memory impairments have not been detected (de Boo et al, 1999;Giordani et al, 1995;Rothlind, Brandt, Zee, Codori, & Folstein, 1993;Strauss & Brandt, 1990), yet others have found evidence of memory decline in the prodromal period. For example, Diamond and colleagues (1992) found that individuals with pre-HD had poorer memory performance on several tests when compared to non-CAG expanded individuals.…”

Rate of caudate atrophy in presymptomatic and symptomatic stages of Huntington's disease

“…Neuropsychological testing has also demonstrated longitudinal declines in symptomatic 22 but not presymptomatic carriers of the HD gene mutation. 23,24 Longitudinal change in samples of symptomatic patients have also been demonstrated for latency and velocity of horizontal saccades 25 and in somatosensory evoked potentials. 26 Investigators are beginning to develop and test new treatments for HD, 27,28 although none has yet been found to be effective.…”

Proximity to Clinical Onset Influences Motor and Cognitive Performance in Presymptomatic Huntington Disease Gene Carriers

“…[8][9][10][11][12][13][14] By contrast, several other studies have reported no cognitive deficits in PSGCs. [15][16][17][18][19] These conflicting findings are likely to be due in part to differences in subject characteristics including how presymptomatic status is defined and how close PSGCs are to likely onset of symptoms. Supporting this are findings that deficits in PSGCs disappeared after subjects who already showed cognitive impairments were removed from analyses, 8,14 and that greater deficits are evident in PSGCs closer to estimated symptomatic HD onset.…”

“…[22][23][24] Other studies showed no motor differences in comparison to nongene carriers. 17,19 Many previous studies have used neurologic examination of motor function rather than tasks designed to study the processes associated with more complex movement. Some authors have suggested that computerized movement tasks may provide a more objective assessment of motor changes and possibly a valuable addition to diagnostic methods.…”