Continuum of frontal lobe impairment in amyotrophic lateral sclerosis.
ABSTRACT To identify the nature and prevalence of cognitive and behavioral abnormalities in patients with amyotrophic lateral sclerosis (ALS).
Survey of clinical characteristics.
Multidisciplinary clinic within a university medical center. Patients A volunteer sample of 30 new patients with ALS were recruited consecutively. Of those invited, 23 participants (20 with sporadic ALS and 3 with familial ALS) enrolled. Participants ranged in age from 27 to 80 years (mean age, 56.5 years); the education level ranged from 12 to 21 years (mean education level, 3.5 years of college); and 17 participants (74%) were male.
Neuropsychological tests, neurobehavioral interviews, and structured magnetic resonance imaging.
Patients were classified into subtypes of frontotemporal lobar degeneration (n = 5), suspected Alzheimer disease (n = 1), and subthreshold variants of cognitive impairment (n = 2), behavioral impairment (n = 4), and cognitively and behaviorally normal (n = 11). Five neuropsychological tests, 2 behavioral abnormalities, and right hemisphere gray matter reductions differentiated patients into normal and abnormal groups.
In this sample, a sizable proportion of patients with ALS possess a range of behavioral and cognitive changes that lie on a spectrum of frontotemporal impairment. Right hemisphere atrophy may be a biomarker for cognitive impairment in patients with ALS.
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ABSTRACT: This paper presents a critical account of research into the non-motor symptoms associated with amyotrophic lateral sclerosis (ALS). Although research examining the cognitive and behavioural features of ALS has been extensively report -ed, social communication and emotion recognition changes have not been comprehensively explored. Furthermore, the current research and diagnostic criteria for diagnosing such changes, which have served as a useful conceptual model, do not provide a means of assessing the subtle cognitive changes reported in non-demented ALS patients. In addition, the impact and challenges of providing care for a person with a diagnosis of ALS who is also experiencing changes in cog-nition and behaviour have been explored only tangentially in the literature, and require immediate attention. We argue that the establishment of criteria to detect mild cognitive impair-ment may serve as a useful model to provide effective clinical interventions for both patients and caregivers at the earliest possible moment.Acta Neuropsychologica 12/2011; 9(4):321-334.
Article: Erfahrungsbericht zum Projekt Korrelation verschiedener Krankheits-Phänotypen und Krankheitsstadien der Amyotrophen Lateralsklerose (ALS) mit Veränderungen in der grauen und weißen Substanz mittels voxel based morphometry (VBM) und diffusion tensor imaging (DTI) Analysen an der University of Oxford (Oxford Centre for Functional MRI of the Brain (FMRIB))Klinische Neurophysiologie 09/2013; 44(03):209-210. · 0.33 Impact Factor