Exploring clinical management of cognitive and behavioural deficits in MND. A scoping review.

OBJECTIVES: Little is known about how cognitive and behavioural decline in MND is managed clinically. This review aimed to summarise clinical management approaches of cognitive and behavioural decline in MND reported in peer-reviewed and grey literature. METHODS: A scoping review was conducted across Embase, Medline, Psychinfo and Emcare in October 2022. Grey literature was also searched across Google Scholar and Google in October 2022. RESULTS: A total of N = 26 studies and 8 documents were included. Thematic analysis revealed six key areas of clinical management: i. Assessment, ii. Education, iii. Advance Care Planning, iv. Adaptation of Care Plan, v. Communication and vi. Carer Support. CONCLUSIONS: The literature on management of cognitive and behavioural decline in MND is sparse. Most peer-reviewed literature consists of expert commentary and there is a lack of primary data to guide practitioners and families on how to manage cognitive and behavioural change in MND. PRACTICE IMPLICATIONS: Determining as early as practicable the presence of cognitive and behavioural changes in pwMND will enable practitioners to make adaptations to communication, provide education and supported decision-making for forward planning. This will enable individualised care, planned in partnership with families with MND, which incorporates personal needs and wishes.

Frontostriatal grey matter atrophy in amyotrophic lateral sclerosis: a visual rating study / Atrofia da massa cinzenta frontostriatal na esclerose lateral amiotrófica: um estudo de avaliação visual antes da publicação

ABSTRACT: Amyotrophic lateral sclerosis (ALS) is characterised by frontostriatal grey matter changes similar to those in frontotemporal dementia (FTD). However, these changes are usually detected at a group level, and simple visual magnetic resonance imaging (MRI) cortical atrophy scales may further elucidate frontostriatal changes in ALS. Objective: To investigate whether frontostriatal changes are detectable using simple visual MRI atrophy rating scales applied at an individual patient level in ALS. Methods: 21 ALS patients and 17 controls were recruited and underwent an MRI scan. Prefrontal cortex sub-regions of the medial orbitofrontal cortex (MOFC), lateral orbitofrontal cortex (LOFC) and anterior cingulate cortex (ACC), striatal sub-regions of the caudate nucleus (CN) and nucleus accumbens (NAcc) were rated using visual grey matter atrophy 5-point Likert scales. Results: Significantly higher atrophy ratings in the bilateral MOFC only in ALS patients versus controls was observed (p<.05). Patients with greater MOFC atrophy had significantly higher atrophy of the CN (p<.05) and LOFC (p<.05). Conclusion: Use of simple visual atrophy rating scales on an individual level reliably detects frontostriatal deficits specific to ALS, showing MOFC atrophy differences with associated CN and LOFC atrophy. This is an applicable method that could be used to support clinical diagnosis and management.

RESUMO: A esclerose lateral amiotrófica (ELA) é caracterizada por alterações na substância cinzenta frontostriatal, semelhantes às da demência frontotemporal (DFT). No entanto, essas alterações geralmente são detectadas em nível de grupo, e as escalas simples de atrofia cortical por ressonância magnética visual (MRI) podem elucidar ainda mais as alterações frontostriatais na ELA. Objetivo: Investigar se as alterações frontostriatais são detectáveis usando escalas de classificação de atrofia MRI visuais simples aplicadas em um nível de paciente individual em ELA. Métodos: 21 pacientes com ELA e 17 controles foram recrutados e submetidos a uma ressonância magnética. Sub-regiões do córtex pré-frontal do córtex orbitofrontal medial (MOFC), córtex orbitofrontal lateral (LOFC) e córtex cingulado anterior (ACC), sub-regiões estriadas do núcleo caudado (NC) e nucleus accumbens (NAcc) foram classificadas usando escalas de atrofia de substância cinzenta visuais de Likert de 5 pontos. Resultados: Observações de atrofia significativamente maiores no MOFC bilateral em pacientes com ELA versus controles foram observadas apenas (p <0,05). Pacientes com maior atrofia do MOFC tiveram atrofia significativamente maior do CN (p <0,05) e LOFC (p <0,05). Conclusão: O uso de escalas de avaliação de atrofia visuais simples em um nível individual detecta de forma confiável déficits frontostriatais específicos para ELA, mostrando diferenças de atrofia MOFC com atrofia associada de CN e LOFC. Este é um método aplicável que pode ser usado para apoiar o diagnóstico e o gerenciamento clínico.