Characterizing behavioral and cognitive dysexecutive changes in progressive supranuclear palsy

Abstract

Frontal lobe dysfunction is a prominent feature of many neurological disorders. Early diagnosis may be enhanced by establishing a profile of cognitive, behavioral, and emotional change. Traditional psychometric assessment focuses on cognitive dysfunction and fails to identify behavioral changes, particularly those associated with orbitofrontal dysfunction. We examined progressive supranuclear palsy (PSP), a prototypical subcortical dementia with frontal features, using commonly available neuropsychological measures and a modification of the Katz Adjustment Scale-Relatives (KAS-R), an instrument first developed to assess dysexecutive changes in head-injured patients. Executive tests identified deficits in reasoning, planning, set shifting, verbal fluency, information processing speed, and response initiation. On the KAS-R, changes in apathy, social withdrawal, and independence were observed, with little change in belligerence, social irresponsibility, uncooperativeness, obstreperousness, anxiety, and depression. The results show the potential utility of this instrument in characterizing behavioral and emotional changes associated with frontal lobe dysfunction in neurodegenerative disease.

Movement Disorders with Dementia in Older Adults

“…The executive de fi cits are readily observed on brief bedside and cognitive screening measures, such as the Frontal Assessment Battery and the Mattis Dementia Rating Scale (especially on the initiation/perseveration subtest) [ 21 ] . De fi cits observed in CBD 345 22 Movement Disorders with Dementia in Older Adults include compromised planning, problem solving [ 104 ] , and cognitive fl exibility [ 105 ] . Progression of de fi cits in problem solving and cognitive fl exibility may be especially rapid in PSP in comparison to other frontostriatal disorders [ 106 ] .…”

Language Processing in Parkinson's Disease Patients Without Dementia

“…In addition, cognitive impairments, in particular executive dysfunctions associated with alterations within the frontostriatal circuitry, occur (Millar et al, 2006). CBD is characterized by slowly progressing, unilateral Parkinsonism with dystonia or myoclonus, unresponsiveness to Levodopa, and limb apraxia.…”

Treatment of Dementia in Parkinsonian Syndromes with Cholinesterase Inhibitors

“…Usually, executive functions are most severely affected [117,118] . Patients have slowed information processing and motor execution, affected long-term memory, limited attentional resources and difficulties in planning and problem solving as well as in set shifting [115,119] . Breakdown in verbal memory is less pronounced than in AD [120] .…”

“…Personality changes occur frequently in the disease course, particularly apathy and disinhibition. Approximately 40-50% of the patients have signs of depression [114] and sleep disturbances [115] . Occurrence and severity of depression, however, seem to be independent of the cognitive state [116] .…”