Neurophysiological assessment of cortical motor function: A direct comparison of methodologies.

Objective: Assessment of cortical function with threshold tracking transcranial magnetic stimulation (TT-TMS) has developed as a biomarker to inform disease pathophysiology, particularly in neurodegenerative disease and dementia. At present, a fully integrated testing system does not exist. To advance clinical utility, and to streamline software design to integrate with diagnostic approaches in an outpatient setting, the present series of studies assessed the effects of altering diagnostic paradigms to measure interstimulus interval (ISI) including serial ascending [T-SICIs] and parallel [T-SICIp] methodologies as measures of cortical motor function (the MagXite software).

Muscle ultrasound aids diagnosis in amyotrophic lateral sclerosis.

Objective: There is a need for improved diagnostic tools in Amyotrophic Lateral Sclerosis (ALS). Our objective was to assess muscle ultrasound as a diagnostic tool in patients with ALS and determine a simplified screening protocol to aid implementation in clinical practice.

Methods: Ultrasound of bulbar and limb muscles was prospectively performed on all patients referred to a single centre with suspected ALS.

Amyotrophic lateral sclerosis represents corticomotoneuronal system failure.

Several decades have passed since the anterograde corticomotoneuronal hypothesis for amyotrophic lateral sclerosis (ALS) was proposed. The intervening years have witnessed its emergent support based on anatomical, pathological, physiological, neuroimaging, and molecular biological studies. The evolution of an extensive corticomotoneuronal system appears restricted to the human species, with ALS representing a uniquely human disease.

Amyotrophic lateral sclerosis established as a multistep process across phenotypes.

Background And Purpose: Given the accepted multistep process of disease causation in amyotrophic lateral sclerosis (ALS), the present study was undertaken to determine the number of steps required for disease onset across each of the ALS phenotypes.

Methods: Clinical and demographic data were prospectively accumulated using the Australian Motor Neurone Disease Registry (2005-2016), and age-specific incidence rates were calculated. Poisson regression was utilized to assess the relationship between log age-specific incidence and log age of onset, with McFadden's R used to assess the goodness of fit of the model.

A scoping review of the unique landscape and challenges associated with dementia in the Western Pacific region.

Unlabelled: Dementia is a leading public health crisis that is projected to affect 152.8 million individuals by 2050, over half of whom will be living in the Western Pacific region. To determine the challenges and opportunities for capacity building in the region, this scoping review searched databases.

Risk factors for the neurodegenerative dementias in the Western Pacific region.

The Western Pacific Region (WPR) is characterized by a group of socioeconomically, culturally, and geopolitically heterogenous countries and represents a microcosm of the global endemic of neurodegeneration. This review will chart the known risk factors for dementia across the WPR. We explore the intersection between the established risk factors for dementia including the (cardiovascular and metabolic disease, sleep, hearing loss, depression, alcohol, smoking, traumatic brain injury, genetics) and (social disadvantage, limited education, systemic racism) as well as incorporate neuroimaging data, where available, to predict disease progression in the WPR.

Validity of Patient-Reported Outcome Measures in Evaluating Nerve Damage Following Chemotherapy.

Importance: Chemotherapy-induced peripheral neuropathy (CIPN) is a substantial adverse effect of anticancer treatments. As such, the assessment of CIPN remains critically important in both research and clinic settings.

Objective: To compare the validity of various patient-reported outcome measures (PROMs) with neurophysiological and sensory functional measures as the optimal method of CIPN assessment.

Central neurodegeneration in Kennedy's disease accompanies peripheral motor dysfunction.

Spinal and bulbar muscular atrophy (SBMA), or Kennedy's disease (KD), is a rare hereditary neuromuscular disorder demonstrating commonalities with amyotrophic lateral sclerosis (ALS). The current study aimed to define functional and central nervous system abnormalities associated with SBMA pathology, their interaction, and to identify novel clinical markers for quantifying disease activity. 27 study participants (12 SBMA; 8 ALS; 7 Control) were recruited.

Diagnostic criteria for amyotrophic lateral sclerosis.

Purpose Of Review: The present review will discuss the evolution of diagnostic criteria for amyotrophic lateral sclerosis (ALS) and biomarker considerations.

Recent Findings: To address the limitations of existing ALS diagnostic criteria, a consortium of key stakeholders developed the Gold Coast consensus criteria (GCC). The GCC has similar or greater sensitivity compared with the revised El Escorial (rEEC) and Awaji criteria (AC), particularly for atypical phenotypes, maintained across disease duration, severity, and site of onset.

Pathological insights derived from neuroimaging in amyotrophic lateral sclerosis: emerging clinical applications.

Purpose Of Review: Neuroimaging has been instrumental in shaping current understanding of the pathoanatomical signature of amyotrophic lateral sclerosis (ALS) across clinically well defined patient cohorts. The potential utility of imaging as an objective disease marker, however, remains poorly defined.

Recent Findings: Increasingly advanced quantitative and computational imaging studies have highlighted emerging clinical applications for neuroimaging as a complementary clinical modality for diagnosis, monitoring, and modelling disease propagation.

Nanoplastics and Neurodegeneration in ALS.

Plastic production, which exceeds one million tons per year, is of global concern. The constituent low-density polymers enable spread over large distances and micro/nano particles (MNPLs) induce organ toxicity via digestion, inhalation, and skin contact. Particles have been documented in all human tissues including breast milk.

Short tandem repeat expansions in are absent in cohorts of familial and sporadic amyotrophic lateral sclerosis patients of European ancestry.

In patients of Asian ancestry, a heterozygous CGG repeat expansion of >100 units in is the cause of oculopharyngodistal myopathy type 1 (OPDM1). Repeat lengths of between 61 and 100 units have been associated with rare amyotrophic lateral sclerosis (ALS) cases of Asian ancestry, although with unusually long disease duration and without significant upper motor neuron involvement. This study sought to determine whether CGG repeat expansions were also present in ALS patients of European ancestry.

An attentional and working memory theory of hallucination vulnerability in frontotemporal dementia.

The rate and prevalence of hallucinations in behavioural variant frontotemporal dementia is well established. The mechanisms for underlying vulnerability however are the least well described in FTD compared with other neuropsychiatric conditions, despite the presence of these features significantly complicating the diagnostic process. As such, this present study aimed to provide a detailed characterization of the neural, cognitive and behavioural profile associated with a predisposition to hallucinatory experiences in behavioural variant frontotemporal dementia.

Novel approaches to assessing upper motor neuron dysfunction in motor neuron disease/amyotrophic lateral sclerosis: IFCN handbook chapter.

Identifying upper motor neuron (UMN) dysfunction is fundamental to the diagnosis and understanding of disease pathogenesis in motor neuron disease (MND). The clinical assessment of UMN dysfunction may be difficult, particularly in the setting of severe muscle weakness. From a physiological perspective, transcranial magnetic stimulation (TMS) techniques provide objective biomarkers of UMN dysfunction in MND and may also be useful to interrogate cortical and network function.

Characterising vincristine-induced peripheral neuropathy in adults: symptom development and long-term persistent outcomes.

Background: Decades following the introduction of vincristine as treatment for haematological malignancies, vincristine-induced peripheral neuropathy (VIPN) remains a pervasive, untreatable side-effect. However there remains a gap in understanding the characteristics of VIPN in adults. This study presents a comprehensive phenotyping of VIPN.

Consensus for experimental design in electromyography (CEDE) project: Checklist for reporting and critically appraising studies using EMG (CEDE-Check).

The diversity in electromyography (EMG) techniques and their reporting present significant challenges across multiple disciplines in research and clinical practice, where EMG is commonly used. To address these challenges and augment the reproducibility and interpretation of studies using EMG, the Consensus for Experimental Design in Electromyography (CEDE) project has developed a checklist (CEDE-Check) to assist researchers to thoroughly report their EMG methodologies. Development involved a multi-stage Delphi process with seventeen EMG experts from various disciplines.

Reduction in short interval intracortical inhibition from the early stage reflects the pathophysiology in amyotrophic lateral sclerosis: A meta-analysis study.

Background And Purpose: Cortical hyperexcitability has been identified as a diagnostic and pathogenic biomarker of amyotrophic lateral sclerosis (ALS). Cortical excitability is assessed by transcranial magnetic stimulation (TMS), a non-invasive neurophysiological technique. The TMS biomarkers exhibiting highest sensitivity for cortical hyperexcitability in ALS remain to be elucidated.

Axonal excitability as an early biomarker of nerve involvement in hereditary transthyretin amyloidosis.

Objectives: The treatment of hereditary transthyretin amyloidosis polyneuropathy (ATTRv-PN) has been revolutionised by genetic therapies, with dramatic improvements in patient outcomes. Whilst the optimal timing of treatment initiation remains unknown, early treatment is desirable. Consequently, the aim of the study was to develop biomarkers of early nerve dysfunction in ATTRv-PN.