Benton Judgment of Line Orientation (JoLO) Test: A Brief and Useful Measure for Assessing Visuospatial Abilities in Manifest, but not Premanifest, Huntington's Disease.

BACKGROUND

Visuospatial deficits have been described in Huntington's disease (HD); however, the extent of these deficits remains unclear. The Benton Judgment of Line Orientation (JoLO) Test, commonly used to assess visuospatial ability, requires minimal motor involvement. It has demonstrated sensitivity to visuospatial deficits in Parkinson's disease; however, few studies have examined performance on this test in HD.

OBJECTIVE

The objective of the current study was to assess visuospatial ability in premanifest and manifest HD using the JoLO.

METHODS

A global cognitive measure, the Mattis Dementia Rating Scale (DRS), was used to stratify manifest HD patients as mild (DRS ≥129) vs. moderate-severe (DRS ≤128). Fifty mild, 42 moderate-severe, and 30 premanifest HD subjects, as well as 35 matched controls, were administered the JoLO. HD Burden of Pathology (BOP) scores were used as a measure of disease severity.

RESULTS

Results revealed that the total manifest HD sample (p <  0.001), in addition to the mild (p = 0.028), and moderate-severe (p <  0.001), but not premanifest, HD subjects scored significantly lower on the JoLO compared to normal controls.

CONCLUSIONS

Our results suggest that the JoLO is useful for detecting visuospatial deficits across various stages of manifest HD. However, any visuospatial impairment that might be present during the premanifest stage of HD was not detected using the JoLO in the present sample.