Utility of the Gold Coast criteria for amyotrophic lateral sclerosis: Experience with fast progressors.Journal of the neurological sciences • January 30, 2025
Matthew Kiernan, Hannah Timmins
In the absence of a definitive diagnostic test for amyotrophic lateral sclerosis (ALS), various criteria have been developed by those working in the field, particularly over recent decades [1]. These criteria were typically clinically based, and described features indicative of upper and lower motor neurone disease involvement, as identified by Charcot [3]. This sustained and iterative process has culminated in consensus approaches, as promoted by the World Federation of Neurology (WFN), initially with the El Escorial criteria, later revised, and with subsequent incorporation of technological advance, particularly in the realm of clinical neurophysiology [2]. Each of these more recent criteria incorporated degrees of certainty: possible, probable or definite disease. However, a considerable proportion of patients labelled as ‘possible ALS’ ultimately succumb to the disease without ever meeting the criteria for more definitive diagnostic categories. Furthermore, these classifications often led to misunderstandings among both patients and clinicians, being mistakenly perceived as an assessment of ALS likelihood rather than a structured diagnostic framework [18]. In contrast, the most recent WFN consensus, the Gold Coast criteria, removed diagnostic uncertainty, in part to lower barriers for patient enrolment to clinical trials [16]. Now the diagnosis is ALS, or it isn't, based on the supportive information, including clinical investigations, typically used to rule out other conditions. Having established consensus criteria, it was important for validation studies, which have overwhelmingly supported a more streamlined diagnostic process, and clinical outcome [11]. In further support, a study in the current issue of Journal of Neurological Sciences has confirmed the superiority of the Gold Coast criteria when assessing ALS patients with a fast disease progression, while at the same time further validating the criteria in a further cohort of Japanese patients [13].
An Annotated Multi-Site and Multi-Contrast Magnetic Resonance Imaging Dataset for the study of the Human Tongue Musculature.Scientific data • January 15, 2025
Fernanda Ribeiro, Xiangyun Zhu, Xincheng Ye, Sicong Tu, Shyuan Ngo, Robert Henderson, Frederik Steyn, Matthew Kiernan, Markus Barth, Steffen Bollmann, Thomas Shaw
This dataset provides the first annotated, openly available MRI-based imaging dataset for investigations of tongue musculature, including multi-contrast and multi-site MRI data from non-disease participants. The present dataset includes 47 participants collated from three studies: BeLong (four participants; T2-weighted images), EATT4MND (19 participants; T2-weighted images), and BMC (24 participants; T1-weighted images). We provide manually corrected segmentations of five key tongue muscles: the superior longitudinal, combined transverse/vertical, genioglossus, and inferior longitudinal muscles. Other phenotypic measures, including age, sex, weight, height, and tongue muscle volume, are also available for use. This dataset will benefit researchers across domains interested in the structure and function of the tongue in health and disease. For instance, researchers can use this data to train new machine learning models for tongue segmentation, which can be leveraged for segmentation and tracking of different tongue muscles engaged in speech formation in health and disease. Altogether, this dataset provides the means to the scientific community for investigation of the intricate tongue musculature and its role in physiological processes and speech production.
The Neonatal Microbiome: Implications for Amyotrophic Lateral Sclerosis and Other Neurodegenerations.Brain Sciences • January 13, 2025
Andrew Eisen, Matthew Kiernan
Most brain development occurs in the "first 1000 days", a critical period from conception to a child's second birthday. Critical brain processes that occur during this time include synaptogenesis, myelination, neural pruning, and the formation of functioning neuronal circuits. Perturbations during the first 1000 days likely contribute to later-life neurodegenerative disease, including sporadic amyotrophic lateral sclerosis (ALS). Neurodevelopment is determined by many events, including the maturation and colonization of the infant microbiome and its metabolites, specifically neurotransmitters, immune modulators, vitamins, and short-chain fatty acids. Successful microbiome maturation and gut-brain axis function depend on maternal factors (stress and exposure to toxins during pregnancy), mode of delivery, quality of the postnatal environment, diet after weaning from breast milk, and nutritional deficiencies. While the neonatal microbiome is highly plastic, it remains prone to dysbiosis which, once established, may persist into adulthood, thereby inducing the development of chronic inflammation and abnormal excitatory/inhibitory balance, resulting in neural excitation. Both are recognized as key pathophysiological processes in the development of ALS.
The comparative effect of propolis and chlorhexidine mouthwash on oral nitrite-producing bacteria and blood pressure regulation.Journal of oral microbiology • December 18, 2024
R Bescos, L Du Toit, A Redondo Rio, P Warburton, T Nicholas, M Kiernan, R Burton, L Belfield, G Montagut, A Benavente, W Vevers, T Gabaldón, Z Brookes, P Casas Agustench
Propolis mouthwash (PROP-M) has demonstrated antibacterial properties like those of chlorhexidine mouthwash (CHX-M). However, its impact on the abundance of oral nitrite-producing species (NPS) and nitrite-producing activity (NPA) remains unexplored. Forty-five healthy individuals were randomised into 2 groups to rinse their mouth twice a day for seven days with either CHX-M (n = 21) or PROP-M (n = 24). Metagenomic sequencing (16S rRNA) was performed on saliva samples collected before and after each treatment. Additionally, salivary biomarkers and blood pressure were measured. CHX-M increased the relative abundance of NPS (p < 0.001) but significantly impaired the NPA (p < 0.001) compared to baseline and PROP-M. No significant differences in the relative abundance of NPS and NPA were observed in the PROP-M group. However, a significant increase of plasma nitrate (+7 µmol/L, p = 0.047) and a decrease in systolic BP (-2 mmHg, p = 0.022) was observed in this group compared to the baseline. The results indicate that PROP-M had a smaller effect on the abundance of NPS and NPA compared to CHX-M. Additionally, PROP-M reduced blood pressure in healthy individuals, but this effect was not associated with changes in the oral microbiome.
Post-infectious acute cerebellar ataxia in a young adult.BMJ case reports • January 27, 2025
Tal Koren, Matthew Kiernan
Acute cerebellar ataxia is a clinical syndrome that involves loss of balance and coordination, typically within less than 72 hours. It usually presents in children and rarely affect adults. A woman in her early 20s presented with acute onset dizziness, vertigo, truncal ataxia and dysarthria 2 weeks following an acute viral illness. Cerebral MRI identified evidence of dural enhancement, and positron emission topography brain imaging showed cortical reduction of glucose metabolism, consistent with an encephalopathic process. Lumbar puncture established a normal cerebrospinal fluid protein and glucose, with seven white cells ×106/L. Subsequent investigations identified evidence of a recent Epstein-Bar virus, in keeping with a post-infectious acute cerebellar ataxia syndrome. Following treatment with intravenous methylprednisolone, symptoms resolved gradually over months. While post-infectious acute cerebellar ataxia is rare in adults, it is an important cause of an acute presentation of ataxia that should be recognised by paediatric and adult physicians.
