Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy

Abstract

This article reports the severity and profile of neuropsychological impairment on a prevalent cohort of patients with a clinical diagnosis of either multiple system atrophy (n=372) or progressive supranuclear palsy (n=311) from the Neuroprotection and Natural History in Parkinson Plus Syndromes cohort. The Dementia Rating Scale and Frontal Assessment Battery were used to assess global cognition and executive dysfunction. For the Dementia Rating Scale impairment was observed in approximately 57% of the progressive supranuclear palsy group and 20% of the multiple system atrophy group. In the former, impairment in a single cognitive domain was observed in 40%, with the same number showing impairment in multiple domains, while in the latter the figures were 28.6 and 13.5%, respectively. On the Frontal Assessment Battery, impairment was observed in 62.0% of patients with progressive supranuclear palsy and 31.8% of those with multiple system atrophy. Although the progressive supranuclear palsy group performed worse overall, the cognitive profiles of the two groups on the Dementia Rating Scale subscales were identical, with the main impairment of the Initiation and Perseveration subscale. The impaired patients in the two groups were largely indistinguishable, qualitatively and quantitatively. Impairment was associated with greater age and clinical disability in both groups and was evident even in the early stages (22% in multiple system atrophy and 50% in progressive supranuclear palsy). Where a pathological diagnosis was available, the original clinical diagnosis was confirmed in the majority of cases, including those with significant cognitive impairment. The rate of impairment in those with a confirmed pathological diagnosis was comparable to that of the sample as a whole. These results demonstrate, in the largest prospectively recruited cohort of patients with progressive supranuclear palsy and multiple system atrophy studied to date, the existence of a cognitive profile similar to that previously reported in idiopathic Parkinson's disease. The results indicate a high level of cognitive impairment associated with progressive supranuclear palsy, but also point to comparable dysfunction in a substantial proportion of the patients with multiple system atrophy. Significant cognitive impairment appears consistent with a diagnosis of multiple system atrophy, even early in the disease, with important implications for diagnosis, research and management.

Cognition in a multiple system atrophy series of cases from Argentina

“…A frontal-type cognitive impairment profile has been described in MSA patients 4,5 and although dementia appears as a non-supportive feature of MSA, recent evidence suggests that cognitive dysfunction may be more frequent than previously reported, with a prevalence between 17-40% [6][7][8] . In some studies, patients with MSA-P have shown involvement of verbal retrieval and visuospatial and executive abilities, and patients with MSA-C have shown compromise in visuospatial functions, learning new verbal information, verbal fluency and attention tests 6,9,10 .…”

Cognitive and neuropsychiatric disorders in extrapyramidal diseases

“…В осно-ве синдрома лежит нарушение внутренней организации ког-нитивных процессов, которая затрудняет актуализацию имеющихся и приобретение новых знаний и навыков [16,17]. Особые сложности возникают при выполнении много-этапных заданий, которые требуют планирования и дли-тельной концентрации внимания, принятия решения, пере-ключения с одного этапа интеллектуальной деятельности на другой.…”

“…Указанный профиль когнитивных рас-стройств был подробно описан у больных прогрессирующим надъядерным параличом, болезнью Паркинсона, болезнью Гентингтона и получил название «подкорковая деменция» [11,16,18]. По ряду проявлений «подкорковая деменция» действительно отличалась от классического варианта демен-ции, свойственного болезни Альцгеймера, который был пре-имущественно связан с дегенерацией височно-теменной ко-ры и получил название корковой деменции.…”

Functional impairment in progressive supranuclear palsy

“…RESULTS There were 113 men and 89 women in this sample, with a mean age of 68.0 years (SD 6.6, range 53-87), mean education of 15.0 years (SD 3.5, range [8][9][10][11][12][13][14][15][16][17][18][19][20], and mean duration of symptoms of 3.8 years (SD 1.6, range 0.6-10.0). For the entire group, motor signs were evident (UPDRS motor: mean 30.5, SD 12.8, range 8-66), cognition tended to be impaired (DRS-2 total: mean 124.5, SD 11.9, range 88-144), and most demonstrated psychiatric symptoms (NPI total: mean 18.8, SD 5 9.2, range 1-50).…”

“…7 Conversely, in a large international cohort, the average patient with PSP was partially dependent, needing assistance with half of his or her daily chores. 8 Given this variability in functional abilities in PSP and the importance of this topic, the current study examined functional profiles of patients with early-stage PSP in a large prospective, multisite study. It was expected that functional disability would be high, even in this early phase cohort, and that functional disability would correlate with motor, cognitive, and psychiatric symptoms.…”