Revisiting the pathoanatomy of pseudobulbar affect: mechanisms beyond corticobulbar dysfunction

Bede, Peter; Finegan, Eoin

doi:10.1080/21678421.2017.1392578

Cited by 28 publications

(18 citation statements)

References 37 publications

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“…A number of radiological cues have been associated with ALS, such as high signal along the pyramidal tracts on T2 weighted or FLAIR imaging, low signal in the precentral gyrus on GRE/SWI, isolated motor cortex atrophy on T1W, but these qualitative visual cues are not specific to ALS and are not sensitive for diagnostic or monitoring purposes (48). Quantitative imaging studies of ALS on the other hand have successfully captured the cortical (UMN) components of bulbar dysfunction in a somatotopic distribution (49, 50) and characterized the pathological substrate of pseudobulbar affect (51, 52). With relentless methodological (53) and conceptual advances in neuroimaging (54), the establishment of multicenter data repositories (55) and the increasing availability of 7 Tesla systems (56), the anatomical underpinnings of bulbar dysfunction are likely to be characterized in further detail.…”

Section: Tools For Diagnosing and Screening For Bulbar Alsmentioning

confidence: 99%

Clinical Measures of Bulbar Dysfunction in ALS

et al. 2019

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Bulbar impairment represents a hallmark feature of Amyotrophic Lateral Sclerosis (ALS) that significantly impacts survival and quality of life. Speech and swallowing dysfunction are key contributors to the clinical heterogeneity of ALS and require well-timed and carefully coordinated interventions. The accurate clinical, radiological and electrophysiological assessment of bulbar dysfunction in ALS is one of the most multidisciplinary aspects of ALS care, requiring expert input from speech-language pathologists (SLPs), neurologists, otolaryngologists, augmentative alternative communication (AAC) specialists, dieticians, and electrophysiologists—each with their own evaluation strategies and assessment tools. The need to systematically evaluate the comparative advantages and drawbacks of various bulbar assessment instruments and to develop integrated assessment protocols is increasingly recognized. In this review, we provide a comprehensive appraisal of the most commonly utilized clinical tools for assessing and monitoring bulbar dysfunction in ALS based on the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) evaluation framework. Despite a plethora of assessment tools, considerable geographical differences exist in bulbar assessment practices and individual instruments exhibit considerable limitations. The gaps identified in the literature offer unique opportunities for the optimization of existing and development of new tools both for clinical and research applications. The multicenter validation and standardization of these instruments will be essential for guideline development and best practice recommendations.

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Section: Tools For Diagnosing and Screening For Bulbar Alsmentioning

confidence: 99%

Clinical Measures of Bulbar Dysfunction in ALS

et al. 2019

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“…Research over the past 30 years has led to a revision of the traditional model of PCL; studies have provided strong evidence to refute a simple causal relationship between PCL and corticobulbar tract dysfunction (58). Instead, a disruption within a widely dispersed network of emotional control appears to underlie the disorder.…”

Section: Disease Modelsmentioning

confidence: 99%

Pathological Crying and Laughing in Motor Neuron Disease: Pathobiology, Screening, Intervention

et al. 2019

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Pathological crying and laughing (PCL) has significant quality-of-life implications in amyotrophic lateral sclerosis (ALS); it can provoke restrictive life-style modifications and lead to social isolation. Despite its high prevalence and quality of life implications, it remains surprisingly understudied. Divergent pathophysiological models have been proposed, centered on corticobulbar tract degeneration, prefrontal cortex pathology, sensory deafferentation, and impaired cerebellar gate-control mechanisms. Quantitative MRI techniques and symptom-specific clinical instruments offer unprecedented opportunities to elucidate the anatomical underpinnings of PCL pathogenesis. Emerging neuroimaging studies of ALS support the role of cortico–pontine–cerebellar network dysfunction in context-inappropriate emotional responses. The characterization of PCL-associated pathophysiological processes is indispensable for the development of effective pharmacological therapies.

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“…Patients with pseudobulbar affect or pathological crying and laughing exhibit sudden situation-inappropriate emotional responses ( 120 – 122 ) which may have a negative impact on their quality of life ( 123 ) and lead to social isolation or social stigma. It is most commonly associated with UMN-type bulbar dysfunction ( 124 ), but frontal abnormalities, executive dysfunction, basal ganglia pathology and impaired cerebellar gating mechanisms have also been linked this symptom ( 27 , 122 , 125 – 128 ).…”

Section: Cognitive Dysfunctionmentioning

confidence: 99%

Clinical and Radiological Markers of Extra-Motor Deficits in Amyotrophic Lateral Sclerosis

et al. 2018

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Amyotrophic lateral sclerosis (ALS) is now universally recognized as a complex multisystem disorder with considerable extra-motor involvement. The neuropsychological manifestations of frontotemporal, parietal, and basal ganglia involvement in ALS have important implications for compliance with assistive devices, survival, participation in clinical trials, caregiver burden, and the management of individual care needs. Recent advances in neuroimaging have been instrumental in characterizing the biological substrate of heterogeneous cognitive and behavioral deficits in ALS. In this review we discuss the clinical and radiological aspects of cognitive and behavioral impairment in ALS focusing on the recognition, assessment, and monitoring of these symptoms.

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Revisiting the pathoanatomy of pseudobulbar affect: mechanisms beyond corticobulbar dysfunction

Cited by 28 publications

References 37 publications

Clinical Measures of Bulbar Dysfunction in ALS

Clinical Measures of Bulbar Dysfunction in ALS

Pathological Crying and Laughing in Motor Neuron Disease: Pathobiology, Screening, Intervention

Clinical and Radiological Markers of Extra-Motor Deficits in Amyotrophic Lateral Sclerosis

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