Behavioural neurology
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Behavioural neurology · Jan 2019
ReviewMolecular Mechanisms of Neurodegeneration Related to C9orf72 Hexanucleotide Repeat Expansion.
Two clinically distinct diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), have recently been classified as two extremes of the FTD/ALS spectrum. The neuropathological correlate of FTD is frontotemporal lobar degeneration (FTLD), characterized by tau-, TDP-43-, and FUS-immunoreactive neuronal inclusions. An earlier discovery that a hexanucleotide repeat expansion mutation in chromosome 9 open reading frame 72 (C9orf72) gene causes ALS and FTD established a special subtype of ALS and FTLD with TDP-43 pathology (C9FTD/ALS). ⋯ However, many more cellular processes are affected by pathological processes in C9FTD/ALS, including nucleocytoplasmic transport, RNA processing, normal function of nucleolus, formation of membraneless organelles, translation, ubiquitin proteasome system, Notch signalling pathway, granule transport, and normal function of TAR DNA-binding protein 43 (TDP-43). Although the exact molecular mechanisms through which C9orf72 repeat expansions account for neurodegeneration have not been elucidated, some potential therapeutics, such as antisense oligonucleotides targeting hexanucleotide GGGGCC repeats in mRNA, were successful in preclinical trials and are awaiting phase 1 clinical trials. In this review, we critically discuss each proposed mechanism and provide insight into the most recent studies aiming to elucidate the molecular underpinnings of C9FTD/ALS.
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Behavioural neurology · Jan 2019
ReviewPsychological Intervention in Traumatic Brain Injury Patients.
To provide a brief and comprehensive summary of recent research regarding psychological interventions for patients surviving a traumatic brain injury. ⋯ CBT outstands as the preferred therapeutic approach for treating behavioural and emotional disturbances. Also, other related therapies such as dialectical behaviour, mindfulness, and acceptance and commitment therapies have been proposed, and probably in the years to come, more literature regarding their effectiveness will be available. On the other hand, evidence showed that interventions from the field of neuropsychology are minimal if compared with its contribution to assessment. Future research should be aimed at performing studies on more diverse populations (e.g., nonmilitary communities and paediatric and Latin American populations) and at controlling designs to examine the therapeutic efficacy of psychotherapeutic and neurocognitive rehabilitation interventions and compare amelioration by injury severity, age of patients, and clinical profile, in the hopes of creating better guidelines for practitioners.
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Behavioural neurology · Jan 2019
Long-Term Follow-Up of Disability, Cognitive, and Emotional Impairments after Severe Traumatic Brain Injury.
To assess the clinical course of disability, cognitive, and emotional impairments in patients with severe TBI (s-TBI) from 3 months to up to 7 years post trauma. ⋯ These findings indicate that disability and cognition seem to improve over time after s-TBI and appear to be relatively stable from 1 year to 7 years. Since cognitive function on some of the BNIS subscales was associated with outcome on the GOSE, these results indicate that both screening and follow-up of cognitive function could be of importance for the rehabilitation of persons with s-TBI.
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Behavioural neurology · Jan 2019
Behavioral and Cognitive Impacts of Mindfulness-Based Interventions on Adults with Attention-Deficit Hyperactivity Disorder: A Systematic Review.
Mindfulness-based interventions (MBIs) are becoming increasingly popular as treatments for physical and psychological problems. Recently, several studies have suggested that MBIs may also be effective in reducing symptoms of attention-deficit hyperactivity disorder (ADHD). Most studies have examined the effectiveness in children, but there are now a sufficient number of individual treatment trials to consider a systematic review in adults. ⋯ In addition, mindfulness meditation training improves some aspects of executive function and emotion dysregulation. Although these are promising findings to support treatment efficacy of MBIs for ADHD, various biases such as absence of randomization and lack of a control group may affect the actual clinical value and implications of the studies. Moreover, the relatively low quality of selection and performance criteria in several studies, as well as relatively high attrition bias across studies, call for caution before considering conducting further analysis.