Cognitive impairment and dementia in basal ganglia disorders

Abstract

We present an update focusing on research from the past 2 years on cognitive impairment and dementia in basal ganglia disorders, including Huntington's disease, progressive supranuclear palsy, Parkinson's disease, Parkinson's disease dementia, and dementia with Lewy bodies. In addition to the many recent papers that aim to refine descriptions of the cognitive phenotypes in the basal ganglia disorders, the current literature addresses the use of cognitive assessment in differential diagnosis of clinically overlapping disorders, the effects of surgical and pharmacologic treatments on cognitive functions, and the relationship between cognitive impairment and functional disability. We also discuss opportunities for enhancing the understanding of cognition in basal ganglia disorders through the use of improved study design and assessment strategies.

Mild cognitive impairment in prediagnosed Huntington disease

“…In those mid and near diagnosis, processing speed and episodic memory were most commonly affected. DISCUSSION Cognitive deficits have been widely reported in pre-HD and manifest HD, [11][12][13][14][15] but the prevalence of MCI in HD has not been previously examined. In a large cohort of individuals who were estimated to be over 14 years from a motor diagnosis of HD, we found that nearly 40% displayed mild impairments in episodic memory, processing speed, executive functioning, and/or visuospatial perception.…”

Cognitive deficits in Huntington's disease on the Repeatable Battery for the Assessment of Neuropsychological Status

“…Rosser & J. R. Hodges, 1994; Rothlind, et al, 1993; Snowden, et al, 2001; Stout & Johnson, 2005), and HD remains an important disorder for neuropsychology, as it represents a protypical “subcortical” dementia. However, as clinical trials continue to advance for this disease, brief yet sensitive batteries will be needed to measure cognitive functioning in these patients.…”

“…E. Rosser & J. R. Hodges, 1994; Snowden, Craufurd, Griffiths, Thompson, & Neary, 2001; Stout & Johnson, 2005). These cognitive changes typically develop gradually; however, at later stages of the disease, the cognitive decline can progress more rapidly (Montoya, et al, 2006).…”

Parkinson’s Disease

“…Similarly, for depression or behavioral change the following clinical features will influence the composition of the epidemiological data sets: premorbid ‘Parkinson Personality,’ anxiety and apathy, fatigue, amotivational syndromes and severe vs. mild depression vs. adjustment reactions. A number of excellent studies over the last 25 years have described the epidemiological and phenomenological characteristics and longitudinal course, diagnosis and management of these vexing disorders (Mayeux et al 1981; Brown and Marsden 1984; Raskin et al 1990; Cummings 1992; Shulman et al 2001; McDonald et al 2003; Aarsland et al 2005; McKeith et al 2005; Stout and Johnson 2005; Emre et al 2007; Weintraub and Burn 2011; Aarsland et al 2012). …”