Pseudobulbar affect as a negative prognostic indicator in amyotrophic lateral sclerosis

Tortelli, Rosanna; Arcuti, Simona; Copetti, Massimiliano; Barone, Roberta; Zecca, Chiara; Capozzo, Rosa; Barulli, Maria Rosaria; Simone, Isabella Laura; Logroscino, Giancarlo

doi:10.1111/ane.12918

Cited by 9 publications

(8 citation statements)

References 31 publications

(41 reference statements)

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“…It is not certain if prognostic factors affecting ALSFRS-R decline rate influence the survival. To date, there has been only one study describing a possible impact of EL on the ALS outcome [45]. It showed a slightly shorter survival of patients with EL, without reaching statistical significance (p=0.25).…”

Section: Discussionmentioning

confidence: 97%

Emotional Lability at Disease Onset Is an Independent Prognostic Factor of Faster Disease Progression in Amyotrophic Lateral Sclerosis

Barć¹,

Szacka²,

Nieporęcki³

et al. 2020

Aging and disease

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Amyotrophic lateral sclerosis (ALS) is a fast progressing neurodegenerative disease leading to quadriplegia, anarthria and respiratory insufficiency. A large variety of phenotypes and disability progression requires individually tailored management. Identification of predictors of poor prognosis may not only improve management, but also allow for more precise patients' stratification for clinical trials or research studies. The aim of the study was to investigate the influence of emotional lability present at disease onset on ALS progression by exploring its direct impact on the decay of the ALS Functional Rating Scale-Revised (ALSFRS-R). The study was performed in a group of 1145 patients from Germany, Poland, Portugal and Turkey between 2014 and 2018. The analysis showed that the presence of emotional lability at ALS onset was linked to a faster decline of ALSFRS-R (0.70 vs 0.50, p<0.0001), in case of either bulbar (0.80 vs 0.65, p<0.05) or limb disease onset (0.59 vs 0.46, p <0.01). It was most prominent in the bulbar subscore of ALSFRS-R. A multiple regression analysis showed a direct influence of emotional lability at ALS onset on disease progression, regardless of age, gender, site of onset, weight loss, cognitive impairment and diagnosis delay (β=0.071; p=0.019). It can therefore be concluded that the presence of emotional lability at the disease onset is an independent factor of faster disease progression in ALS.

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Section: Discussionmentioning

confidence: 97%

Emotional Lability at Disease Onset Is an Independent Prognostic Factor of Faster Disease Progression in Amyotrophic Lateral Sclerosis

Barć¹,

Szacka²,

Nieporęcki³

et al. 2020

Aging and disease

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“…We failed to observe a significant impact of PLC on survival in ALS patients in a multivariate Cox regression model analysis. A previous study found that the presence of PLC at recruitment can be a negative prognostic factor for survival in a very early stage of the disease, suggesting a short survival in a subgroup of patients with less functional impairment ( 11 ). However, this study lacked the neuropsychological evaluation for the relationship between PLC and other symptoms such as frontal dysfunction or cognitive impairment in general.…”

Section: Discussionmentioning

confidence: 99%

“…Cognitive dysfunction is a non-motor symptom in ALS with the severity of cognitive decline ranging from mild cognitive impairment to ALS frontotemporal dementia involving executive and non-executive domains ( 10 ). The relationship between PLC and cognitive impairment is controversial in ALS ( 11 ). Further studies with a larger sample size are required to explore the individualized experiences of PLC in ALS.…”

Section: Introductionmentioning

confidence: 99%

Prevalence and Factors Related to Pathological Laughter and Crying in Patients With Amyotrophic Lateral Sclerosis

Wei

Lin

et al. 2021

Front. Neurol.

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Objective: This study aimed to explore the prevalence and clinical correlates of pathological laughter and crying (PLC) in patients with amyotrophic lateral sclerosis (ALS).Methods: A total of 1,031 ALS patients were enrolled between August 2012 and August 2019. The PLC was recorded by a face-to-face interview. Other characteristics of patients, including depression, anxiety, cognition, and behavior function, were also evaluated. The potential associated factors of PLC were explored using forward binary regression analysis. Survival was analyzed in groups using propensity score matching (PSM) and Cox proportional hazards models.Results: The prevalence of PLC was 11.4% in all patients at baseline. Bulbar-onset and female patients had higher prevalence of PLC. The multivariate regression analysis indicated that PLC in ALS was associated with bulbar onset (p < 0.001), late disease stage (p < 0.001), and higher score in the Hamilton Depression Rating Scale (HDRS) (p = 0.012). The higher score of HDRS was significantly and independently associated with PLC occurrence in bulbar-onset patients (p = 0.032). The late disease stage was related to PLC occurrence in spinal-onset patients (p < 0.001). After comparison with matched pairs by using PSM, PLC at baseline had no impact on survival.Conclusion: PLC was not uncommon in ALS, especially in bulbar-onset and female patients. We highlighted that the emotional state other than cognitive function had possible relationship with PLC in ALS.

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“…34,87,88 Cognitive impairment, which has been identified in as many as 51% of PALS, may include frontotemporal dementia, cognitivebehavioral impairment, and nonexecutive findings. 89,90 As cognitive screening instruments validated for bulbar ALS are urgently needed…”

Section: Extrabulbar Itemsmentioning

confidence: 99%

A clinical bulbar assessment scale (CBAS) for amyotrophic lateral sclerosis

et al. 2022

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Introduction/Aims: Comprehensive and valid bulbar assessment scales for use within amyotrophic lateral sclerosis (ALS) clinics are critically needed. The aims of this study are to develop the Clinical Bulbar Assessment Scale (CBAS) and complete preliminary validation. Methods:The authors selected CBAS items from among the literature and expert opinion, and content validity ratio (CVR) was calculated. Following consent, the CBAS was administered to a pilot sample of English-speaking adults with El Escorial defined ALS (N = 54) from a multidisciplinary clinic, characterizing speech, swallowing, and extrabulbar features. Criterion validity was assessed by correlating CBAS scores with commonly used ALS scales, and internal consistency reliability was obtained.Results: Expert raters reported strong agreement for the CBAS items (CVR = 1.00; 100% agreement). CBAS scores yielded a moderate, significant, negative correlation with ALS Functional Rating Scale-Revised (ALSFRS-R) total scores (r = À0.652, p < .001), and a strong, significant, negative correlation with ALSFRS-R bulbar subscale scores (r = À0.795, p < .001). There was a strong, significant, positive correlation with Center for Neurologic Studies Bulbar Function Scale (CNS-BFS) scores (r = 0.819, p < .001). CBAS scores were significantly higher for bulbar onset (mean = 38.9% of total possible points, SD = 22.6) than spinal onset (mean = 18.7%, SD = 15.8; p = .004). Internal consistency reliability (Cronbach's alpha) values were: (a) total CBAS, α = 0.889; (b) Speech subscale, α = 0.903; and (c) Swallowing subscale, α = 0.801. Discussion: The CBAS represents a novel means of standardized bulbar data collection using measures of speech, swallowing, respiratory, and cognitive-linguistic skills.Preliminary evidence suggests the CBAS is a valid, reliable scale for clinical assessment of bulbar dysfunction.

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Pseudobulbar affect as a negative prognostic indicator in amyotrophic lateral sclerosis

Abstract: These preliminary results underlie that the presence of PBA at entry negatively influences survival in a specific subgroup of patients with ALS characterized by less functional impairment.

Cited by 9 publications

References 31 publications

Emotional Lability at Disease Onset Is an Independent Prognostic Factor of Faster Disease Progression in Amyotrophic Lateral Sclerosis

Emotional Lability at Disease Onset Is an Independent Prognostic Factor of Faster Disease Progression in Amyotrophic Lateral Sclerosis

Prevalence and Factors Related to Pathological Laughter and Crying in Patients With Amyotrophic Lateral Sclerosis

A clinical bulbar assessment scale (CBAS) for amyotrophic lateral sclerosis

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