Guidelines for clinical pharmacological practices in Huntington's disease.

Objective: Evidence-based medicine is a difficult goal to achieve in rare diseases where randomized controlled trials are lacking. This report provides guidelines that capitalize on both the literature and expertise of the French National Huntington Disease Reference Centre to optimalize pharmacological therapeutic interventions for Huntington's disease (HD).

Material And Methods: HD experts conducted a systematic analysis of the literature from 1965 to 2013, using a scoring procedure established by the French National Authority for Health.

The neural substrates of script knowledge deficits as revealed by a PET study in Huntington's disease.

Introduction: Previous neuropsychological investigations have suggested that both the prefrontal cortex and the basal ganglia are involved in the management of script event knowledge required in planning behavior.

Methods: This study was designated to map, the correlations between resting-state brain glucose utilization as measured by FDG-PET (positron emission tomography) and scores obtained by means of a series of script generation and script sorting tasks in 8 patients with early Huntington's disease.

Results: These patients exhibited a selectively greater impairment for the organizational aspects of scripts compared to the semantic aspects of scripts.