Cognitive impairment within and beyond the FTD spectrum in ALS: development of a complementary cognitive screen.

Objective: To investigate cognitive impairments in amyotrophic lateral sclerosis (ALS), extending both within and beyond the established frontotemporal dementia (FTD) spectrum, using the Complementary Cognitive ALS Screen (C-CAS).

Methods: The C-CAS, designed to complement the Edinburgh cognitive and behavioural ALS screen (ECAS), explores cognitive (sub)domains not investigated by the ECAS. Normative data were collected, and models adjusted for age, sex, and education level were developed.

osl-ephys: a Python toolbox for the analysis of electrophysiology data.

We describe OHBA Software Library for the analysis of electrophysiology data (osl-ephys). This toolbox builds on top of the widely used MNE-Python package and provides unique analysis tools for magneto-/electro-encephalography (M/EEG) sensor and source space analysis, which can be used modularly. In particular, it facilitates processing large amounts of data using batch parallel processing, with high standards for reproducibility through a config API and log keeping, and efficient quality assurance by producing HTML processing reports.

Urinary sodium analysis: The key to effective diuretic titration? European Journal of Heart Failure expert consensus document.

In patients with heart failure, neurohumoral activation leads to increased renal sodium avidity across the entire renal tubules, resulting in a positive sodium and water balance, leading to decompensated heart failure requiring intravenous diuretics. As the dose of diuretic therapy required to achieve euvolaemia is difficult to estimate due to considerable intra- and interindividual differences, the European Society of Cardiology recommends assessment of the diuretic response within hours either via evaluation of the urinary sodium concentration or via urinary volume after initial diuretic administration. All diuretic agents enhance sodium excretion to a different extent depending on their side of action across the renal tubules, and renal adaptation mechanisms due to neurohumoral stimulation.

An observational study of pleiotropy and penetrance of amyotrophic lateral sclerosis associated with CAG-repeat expansion of ATXN2.

Spinocerebellar ataxia type 2 (SCA2) and amyotrophic lateral sclerosis (ALS) are both associated with a CAG-repeat expansion in ATXN2 and with TDP-43-positive neuronal cytoplasmic inclusions. The two disorders have been viewed as distinct entities, where an intermediate length expansion of 31-33 CAG-repeats is associated with sporadic ALS and a full length expansion of ≥34 CAG-repeats is associated with SCA2. We report the clinical phenotype of ATXN2-positive patients and their relatives, identified in three specialist ALS clinics, which force a reconsideration of this dichotomy.

Neuroimaging correlates of domain-specific cognitive deficits in amyotrophic lateral sclerosis.

Background: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with frequent extra-motor involvement. In the present study, we investigated whether specific cognitive and behavioral deficits in ALS correlate with distinct extra-motor neurodegeneration patterns on brain MRI.

Methods: We performed multimodal brain MRI and Edinburgh cognitive and behavioral ALS screen (ECAS) in 293 patients and 237 controls.