Prevalence and correlates of neuropsychological deficits in amyotrophic lateral sclerosis.
Paul J. Massman,Paul J. Massman,Jamie Sims,Norma Cooke,Lanny J. Haverkamp,Vicki Appel,Stanley H. Appel +6 more
TLDR
Neither the conventional wisdom that cognition is intact in nearly all patients with amyotrophic lateral sclerosis, nor more recent suggestions that Cognition is often at least mildly impaired seems to be correct.Abstract:
OBJECTIVE: To determine the prevalence and correlates of neuropsychological impairment in a large cohort (n = 146) of patients with typical, sporadic (non-familial) amyotrophic lateral sclerosis. METHODS: A battery of neuropsychological tests was administered to patients with amyotrophic lateral sclerosis who were attending a monthly outpatient clinic or who were in hospital undergoing diagnostic tests. RESULTS: Comparing individual patient's scores with relevant normative data, 35.6% of the patients displayed evidence of clinically significant impairment, performing at or below the 5th percentile on at least two of the eight neuropsychological measures. Deficits were most common in the areas of problem solving, attention/mental control, continuous visual recognition memory, word generation, and verbal free recall. Impairment was most prevalent in patients with dysarthria (48.5%), but 27.4% of non-dysarthric patients were also impaired. Impaired patients had more severe or widespread symptoms of amyotrophic lateral sclerosis than non-impaired patients, and had fewer years of education. CONCLUSION: Neither the conventional wisdom that cognition is intact in nearly all patients with amyotrophic lateral sclerosis, nor more recent suggestions that cognition is often at least mildly impaired seems to be correct. A minority of patients with amyotrophic lateral sclerosis displayed evidence of significant impairment. Dysarthria, low education, and greater severity of motor symptoms were risk factors for impairment.read more
Citations
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Contributions to Neuropsychological Assessment
TL;DR: This cornucopia will be coveted and dipped into by those neurologists with a special interest in abnormal movement disorders, but who would not consider themselves to have a research interest in tremor.
Journal ArticleDOI
Prevalence and patterns of cognitive impairment in sporadic ALS.
G. M. Ringholz,Stanley H. Appel,Major R. Bradshaw,Norma Cooke,Diane M. Mosnik,Diane M. Mosnik,Paul E. Schulz,Paul E. Schulz +7 more
TL;DR: These data confirm the presence of cognitive impairment in 50% of patients with ALS and particularly implicate executive dysfunction and mild memory decline in the disease process and have features consistent with FTD.
Journal ArticleDOI
Evidence of widespread cerebral microglial activation in amyotrophic lateral sclerosis: an [11C](R)-PK11195 positron emission tomography study
Martin R Turner,Annachiara Cagnin,Federico Turkheimer,Federico Turkheimer,Christopher C.J. Miller,Christopher Shaw,David J. Brooks,Peter Leigh,Richard B. Banati,Richard B. Banati +9 more
TL;DR: The findings indicate that cerebral microglial activation can be detected in vivo during the evolution of ALS, and support the previous observations that cerebral pathology is widespread, and argue for the development of therapeutic strategies aimed at inflammatory pathways.
Journal ArticleDOI
The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study
Julie Phukan,Marwa Elamin,Peter Bede,Norah Jordan,Laura Gallagher,Susan Byrne,Catherine Lynch,Niall Pender,Orla Hardiman +8 more
TL;DR: Cognitive impairment, predominantly but not exclusively in the form executive dysfunction, is present in more than 40% of ALS patients who have no evidence of dementia, and its manifestations may be more heterogeneous than previously recognised.
Journal ArticleDOI
Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria
Michael J. Strong,Sharon Abrahams,Laura H. Goldstein,Susan C. Woolley,Paula M. McLaughlin,Julie S. Snowden,Eneida Mioshi,Angie Roberts-South,Michael Benatar,Tibor Hortobágyi,Jeffrey Rosenfeld,Vincenzo Silani,Paul G. Ince,Martin R Turner +13 more
TL;DR: These revised consensus criteria expand upon those of 2009 and embrace the concept of the frontotemporal spectrum disorder of ALS (ALS-FTSD), which is a re-conceptualisation that neuropsychological deficits in ALS fall along a spectrum.
References
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Book
A Compendium of Neuropsychological Tests: Administration, Norms, and Commentary
Otfried Spreen,Esther Strauss +1 more
TL;DR: In this paper, the authors present a comprehensive assessment of mood, personality and adaptive functions of individuals in terms of test scores and scores of motor function, executive function, and attention.
Journal ArticleDOI
Contributions to Neuropsychological Assessment
TL;DR: This cornucopia will be coveted and dipped into by those neurologists with a special interest in abnormal movement disorders, but who would not consider themselves to have a research interest in tremor.
Journal ArticleDOI
Education and the prevalence of dementia and Alzheimer's disease
TL;DR: The effect of no or low education on the prevalence of dementia confirms the 1988 prediction of Mortimer2 that education would provide protection against dementia and the recent finding that a close relationship exists between neocortical synaptic density and cognitive decline in AD provides a biological stratum for the postulated “intellectual reserve.”