Unlabelled: Although impaired insight is a core diagnostic criterion for establishing the diagnosis of frontotemporal dementia (FTD), insight has rarely been studied in amyotrophic lateral sclerosis (ALS).
Objective: To determine differences between patient and informant (caregiver) reports of behavior and behavioral change in amyotrophic lateral sclerosis.
Methods: Behavioral data for 17 patients with ALS and 4 patients with ALS-FTD were analyzed. Behavioral changes were evaluated using the Frontal Systems Behavior Scale (FrSBe). We compared premorbid to current behavioral profiles and patient self-reports with those of their informants to determine the level of awareness regarding behavioral changes since the onset of ALS.
Results: ALS patients without FTD had normal insight, as defined by this study, although self-reports suggested mild behavioral abnormalities. In contrast, patients with ALS-FTD revealed a marked loss of insight regarding profound changes in behavior.
Conclusions: Patients with ALS-FTD exhibit a profound lack of insight, which is not found in non-demented ALS patients. Patients without dementia have normal insight, although they report mild behavioral changes that might reflect a psychological response to the disease.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.3109/17482960903171110 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Thoracic Electric Impedance Tomography Detects Lung Volume Changes in Amyotrophic Lateral Sclerosis.
Muscle Nerve
January 2025
Division of Neurology, Department of Medicine, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.
Introduction/aims: Spirometry is the conventional means to measure lung function in amyotrophic lateral sclerosis (ALS), but is dependent on patient effort and bulbar strength. We aimed to use electric impedance tomography (EIT), an emerging non-invasive imaging modality, to measure dynamic lung volume changes.
Methods: Twenty-one patients with ALS underwent sitting and supine spirometry for forced vital capacity (FVC), and sitting and supine EIT.
Capecitabine and temozolomide or temozolomide alone in patients with atypical carcinoids.
Endocrine
January 2025
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum - Università di Bologna, Bologna, Italy.
Background: Lung neuroendocrine neoplasms (NENs) represent about 20% of all lung cancers. Few therapeutic options are available for atypical carcinoids (ACs). Single-agent temozolomide (TEM) is active in lung NENs, but whether the addition of capecitabine (CAPTEM) is associated with improved outcomes, is unknown.
View Article and Find Full Text PDFDeep learning analyses of splicing variants identify the link of PCP4 with amyotrophic lateral sclerosis.
Brain
January 2025
State Key Laboratory of Cardiology and Medical Innovation Center, Shanghai East Hospital, Clinical Center for Brain and Spinal Cord Research, School of Medicine, Tongji University, 200331, Shanghai, China.
Amyotrophic lateral sclerosis (ALS) is a severe motor neuron disease, with most sporadic cases lacking clear genetic causes. Abnormal pre-mRNA splicing is a fundamental mechanism in neurodegenerative diseases. For example, TAR DNA-binding protein 43 (TDP-43) loss-of-function (LOF) causes widespread RNA mis-splicing events in ALS.
View Article and Find Full Text PDFEnhanced Interleukin 6 Trans-Signaling Modulates Disease Process in Amyotrophic Lateral Sclerosis Mouse Models.
Brain Sci
January 2025
Department of Biochemistry, Wake Forest University School of Medicine, Winston-Salem, NC 27157, USA.
Charcot first described ALS in 1869, but the specific mechanisms that mediate the disease pathology are still not clear. Intense research efforts have provided insight into unique neuroanatomical regions, specific neuronal populations and genetic associations for ALS and other neurodegenerative diseases; however, the experimental results also suggest a convergence of these events to common toxic pathways. We propose that common toxic pathways can be therapeutically targeted, and this intervention will be effective in slowing progression and improving patient quality of life.
View Article and Find Full Text PDFPatients' choices regarding ventilatory support affect opioid use in amyotrophic lateral sclerosis.
Amyotroph Lateral Scler Frontotemporal Degener
January 2025
Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan.
Objective: To investigate the impact of different ventilatory support options on opioid use among patients with amyotrophic lateral sclerosis (ALS).
Methods: We retrospectively reviewed 889 consecutive patients with ALS and enrolled 399 eligible patients. All patients were followed until death or tracheostomy.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!