Systematic approach to diagnosing suspected Creutzfeldt-Jakob disease.

Evaluation of rapidly progressive dementia (RPD) is usually challenging. In most cases, patients progress to dementia in weeks to months, and the differential diagnosis is broad. In this case, a woman in her 60s presented with a 1-month history of episodic vertigo, cognitive decline, ataxia and myoclonus. Cerebrospinal fluid total tau was markedly elevated, which was helpful in establishing the diagnosis and discussing prognosis/end-of-life measures with the patient's family. This case summarises a stepwise diagnostic approach for patients with RPD and highlights recent literature on biomarkers of Creutzfeldt-Jakob disease and autoimmune encephalitis.

Microbial electrosynthesis of acetate from CO2 in three-chamber cells with gas diffusion biocathode under moderate saline conditions.

The industrial adoption of microbial electrosynthesis (MES) is hindered by high overpotentials deriving from low electrolyte conductivity and inefficient cell designs. In this study, a mixed microbial consortium originating from an anaerobic digester operated under saline conditions (∼13 g L-1 NaCl) was adapted for acetate production from bicarbonate in galvanostatic (0.25 mA cm-2) H-type cells at 5, 10, 15, or 20 g L-1 NaCl concentration. The acetogenic communities were successfully enriched only at 5 and 10 g L-1 NaCl, revealing an inhibitory threshold of about 6 g L-1 Na+. The enriched planktonic communities were then used as inoculum for 3D printed, three-chamber cells equipped with a gas diffusion biocathode. The cells were fed with CO2 gas and operated galvanostatically (0.25 or 1.00 mA cm-2). The highest production rate of 55.4 g m-2 d-1 (0.89 g L-1 d-1), with 82.4% Coulombic efficiency, was obtained at 5 g L-1 NaCl concentration and 1 mA cm-2 applied current, achieving an average acetate production of 44.7 kg MWh-1. Scanning electron microscopy and 16S rRNA sequencing analysis confirmed the formation of a cathodic biofilm dominated by Acetobacterium sp. Finally, three 3D printed cells were hydraulically connected in series to simulate an MES stack, achieving three-fold production rates than with the single cell at 0.25 mA cm-2. This confirms that three-chamber MES cells are an efficient and scalable technology for CO2 bio-electro recycling to acetate and that moderate saline conditions (5 g L-1 NaCl) can help reduce their power demand while preserving the activity of acetogens.

Trigeminal neuralgia: Comparison of characteristics and impact in patients with or without multiple sclerosis.

OBJECTIVES: The commonest secondary cause for trigeminal neuralgia (TN) is multiple sclerosis (MS) and little is known about this group of patients in terms of their presentation and treatments. We compared patients with TN and MS (pwTNMS) with a cohort of patients with primary TN, who had been referred to the same specialist unit, both in terms of characteristics and impact on quality of life at the time of their first assessment. METHODS: Using a prospective patient database we extracted key clinical data and results from psychometrically tested questionnaires of 26 pwTNMS and compared them to an age and gender-matched set of 68 patients with primary TN. RESULTS: Our findings suggest that pwTNMS exhibit a more severe clinical phenotype than primary TN. Prior to referral, pwTNMS are more likely to have used more healthcare services and undergone more neurosurgical interventions. Strikingly, pwTNMS exhibit reduced lengths and duration of remission periods and fewer identifiable triggers. Furthermore, pwTNMS report significant impact on quality of life comparable to those in primary TN, scoring highly in measures of anxiety, depression, and catastrophizing, but also report greater sleep disturbance, and overall interference in activities of daily living. CONCLUSIONS: pwTNMS have a more intractable TN, one which may necessitate a more complex approach to treatment, earlier referral to secondary care and an extensive assessment of mental health. Quality of life in pwTNMS is severely affected by both their MS and their TN, suggesting management should occur in specialist centres with access to a multidisciplinary team.

Understanding a role for hypoxia in lesion formation and location in the deep and periventricular white matter in small vessel disease and multiple sclerosis.

The deep and periventricular white matter is preferentially affected in several neurological disorders, including cerebral small vessel disease (SVD) and multiple sclerosis (MS), suggesting that common pathogenic mechanisms may be involved in this injury. Here we consider the potential pathogenic role of tissue hypoxia in lesion development, arising partly from the vascular anatomy of the affected white matter. Specifically, these regions are supplied by a sparse vasculature fed by long, narrow end arteries/arterioles that are vulnerable to oxygen desaturation if perfusion is reduced (as in SVD, MS and diabetes) or if the surrounding tissue is hypoxic (as in MS, at least). The oxygen crisis is exacerbated by a local preponderance of veins, as these can become highly desaturated 'sinks' for oxygen that deplete it from surrounding tissues. Additional haemodynamic deficiencies, including sluggish flow and impaired vasomotor reactivity and vessel compliance, further exacerbate oxygen insufficiency. The cells most vulnerable to hypoxic damage, including oligodendrocytes, die first, resulting in demyelination. Indeed, in preclinical models, demyelination is prevented if adequate oxygenation is maintained by raising inspired oxygen concentrations. In agreement with this interpretation, there is a predilection of lesions for the anterior and occipital horns of the lateral ventricles, namely regions located at arterial watersheds, or border zones, known to be especially susceptible to hypoperfusion and hypoxia. Finally, mitochondrial dysfunction due to genetic causes, as occurs in leucodystrophies or due to free radical damage, as occurs in MS, will compound any energy insufficiency resulting from hypoxia. Viewing lesion formation from the standpoint of tissue oxygenation not only reveals that lesion distribution is partly predictable, but may also inform new therapeutic strategies.