Quantitative Susceptibility MRI to Detect Brain Iron in Amyotrophic Lateral Sclerosis.

Purpose To investigate the whole-brain landscape of iron-related abnormalities in amyotrophic lateral sclerosis (ALS) by using the in vivo MRI technique of quantitative susceptibility mapping (QSM). Materials and Methods For this prospective study, 28 patients with ALS (mean age, 61 years; age range, 43-77 years; 18 men [mean age, 61 years; range, 43-77 years] and 10 women [mean age, 61 years; range, 47-74 years]) recruited between January 17, 2014, and September 4, 2015, and 39 matched control subjects (mean age, 61 years; age range, 39-77 years; 24 men [mean age, 62 years; range, 39-77 years] and 15 women [mean age, 59 years; range, 39-73 years]) were examined by using structural and susceptibility 3.0-T MRI techniques.

Differential involvement of forearm muscles in ALS does not relate to sonographic structural nerve alterations.

Objective: We aimed to assess whether differential peripheral nerve involvement parallels dissociated forearm muscle weakness in amyotrophic lateral sclerosis (ALS).

Methods: The analysis comprised 41 ALS patients and 18 age-, sex-, height- and weight-matched healthy controls. Strength of finger-extension and -flexion was measured using the Medical Research Council (MRC) scale.

Peripheral nerve atrophy together with higher cerebrospinal fluid progranulin indicate axonal damage in amyotrophic lateral sclerosis.

Introduction: We aimed to investigate whether sonographic peripheral cross-sectional nerve area (CSA) and progranulin (PGRN), a neuritic growth factor, are related to each other and whether they interact to predict clinical and paraclinical measures in amyotrophic lateral sclerosis (ALS).

Methods: We included 55 ALS patients who had forearm median and ulnar nerve CSA, cerebrospinal fluid (CSF) PGRN, and serum PGRN measures available. CSF PGRN was normalized against the CSF / serum albumin ratio (Q ).

FIG4 variants in central European patients with amyotrophic lateral sclerosis: a whole-exome and targeted sequencing study.

We aimed to identify the genetic cause of the devastating neurodegenerative disease amyotrophic lateral sclerosis (ALS) in a German family with two affected individuals, and to assess the prevalence of variants in the identified risk gene, FIG4, in a central European ALS cohort. Whole-exome sequencing (WES) and an overlapping data analysis strategy were performed in an ALS family with autosomal dominant inheritance and incomplete penetrance. Additionally, 200 central European ALS patients were analyzed using whole-exome or targeted sequencing.

Structural and diffusion imaging versus clinical assessment to monitor amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis is a progressive neurodegenerative disease that affects upper and lower motor neurons. Observational and intervention studies can be tracked using clinical measures such as the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) but for a complete understanding of disease progression, objective in vivo biomarkers of both central and peripheral motor pathway pathology are highly desirable. The aim of this study was to determine the utility of structural and diffusion imaging as central nervous system biomarkers compared to the standard clinical measure, ALSFRS-R, to track longitudinal evolution using three time-point measurements.

Age and education-matched cut-off scores for the revised German/Swiss-German version of ECAS.

The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has been developed to assess cognition and behaviour in patients with amyotrophic lateral sclerosis (ALS). Cognitive impairments of ALS-specific and ALS-non-specific functions can be determined using cut-off scores based on performance of healthy subjects. However, detailed analyses show that older healthy subjects perform worse than younger ones, whereas highly-educated individuals perform better than those with lower education levels.

Quantifying disease progression in amyotrophic lateral sclerosis using peripheral nerve sonography.

Introduction: In this study we investigated whether peripheral nerve sonography could be used as a biomarker to monitor disease progression in amyotrophic lateral sclerosis (ALS).

Methods: In 37 patients, ulnar and median nerve cross-sectional area (CSA) was determined in at least 2 ultrasound sessions; mean follow-up was 14.5 months.

Executive Dysfunctions and Event-Related Brain Potentials in Patients with Amyotrophic Lateral Sclerosis.

A growing body of evidence implies psychological disturbances in amyotrophic lateral sclerosis (ALS). Specifically, executive dysfunctions occur in up to 50% of ALS patients. The recently shown presence of cytoplasmic aggregates (TDP-43) in ALS patients and in patients with behavioral variants of frontotemporal dementia suggests that these two disease entities form the extremes of a spectrum.

Basal ganglia pathology in ALS is associated with neuropsychological deficits.

Objectives: To evaluate basal ganglia changes along the amyotrophic lateral sclerosis (ALS)-ALS-frontotemporal dementia (FTD) continuum using multiple, complementary imaging techniques.

Methods: Sixty-seven C9orf72-negative patients with ALS and 39 healthy controls were included in a cross-sectional quantitative MRI study. Seven patients with ALS met criteria for comorbid behavioral variant FTD (ALS-FTD), 18 patients met the Strong criteria for cognitive and/or behavioral impairment (ALS-Plus), and 42 patients had no cognitive impairment (ALS-Nci).

Impaired set-shifting in amyotrophic lateral sclerosis: An event-related potential study of executive function.

Objective: Executive dysfunctions in patients suffering from amyotrophic lateral sclerosis (ALS) are often described but poorly understood. Specifically, research on patients' ability to flexibly shift between cognitive sets is still scarce and unsystematic. The present study set out to compensate for this lack by providing an in-depth analysis of ALS-related set-shifting impairments.

A new approach using high volume blood patch for prevention of post-dural puncture headache following intrathecal catheter pump exchange.

Background: In an observational study, complications of intrathecal catheter pumps necessitating surgical exchange were analyzed. Also the use of a high-volume prophylactic epidural blood patch (EBP) during surgery for preventing post-dural puncture headache (PDPH) with a follow-up for 1 year is described.

Materials And Methods: In 22 patients with refractory chronic pain of cancer/noncancer origin or severe spasticity, who were receiving intrathecal morphine including adjuvants or baclofen for symptom relief, catheter exchange with or without pump was performed.

Interaction of physical function, quality of life and depression in Amyotrophic lateral sclerosis: characterization of a large patient cohort.

Background: Due to lack of any curative therapy for ALS, symptomatic treatment and maintenance of quality of life (QoL) is very important. We aimed to characterize the affected domains of QoL in ALS patients and to identify factors which are associated with reduced QoL and increased depression.

Methods: 159 ALS patients answered standardized questionnaires (Beck Depression Inventory-II, SF-36 Health Survey questionnaire, revised ALS functional rating scale).

Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: a prospective observational study.

Weight loss is increasingly considered as a negative prognostic marker in amyotrophic lateral sclerosis (ALS). Despite the critical importance of nutritional issues in ALS, and the common use of percutaneous endoscopic gastrostomy (PEG), there is a general lack of knowledge on peri-interventional treatment, optimal parameters of enteral nutrition, its timing during disease progression and its potential disease-modifying effects in ALS patients. Here we report the results of a multi-center prospective study of percutaneous endoscopic gastrostomy (PEG) in ALS.

The Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen: a cross-sectional comparison of established screening tools in a German-Swiss population.

The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has recently been developed as a fast and easy cognitive screening tool specifically designed for patients with motor impairments in routine clinical use. The German/Swiss-German version of the ECAS was validated in a German-Swiss consortium. One hundred and thirty-six non-demented ALS patients and 160 healthy controls were included in the study.

Peripheral nerve ultrasound in amyotrophic lateral sclerosis phenotypes.

Introduction: In this study we sought to determine the cross-sectional area (CSA) of peripheral nerves in patients with distinct subtypes of amyotrophic lateral sclerosis (ALS).

Methods: Ulnar and median nerve ultrasound was performed in 78 ALS patients [classic, n = 21; upper motor neuron dominant (UMND), n = 14; lower motor neuron dominant (LMND), n = 20; bulbar, n = 15; primary lateral sclerosis (PLS), n = 8] and 18 matched healthy controls.

Results: Compared with controls, ALS patients had significant, distally pronounced reductions of ulnar CSA (forearm/wrist level) across all disease groups, except for PLS.

Central white matter degeneration in bulbar- and limb-onset amyotrophic lateral sclerosis.

Previous studies using diffusion tensor imaging (DTI) have examined for differences between bulbar- and limb-onset amyotrophic lateral sclerosis (ALS). Findings between studies have been markedly inconsistent, though possibly as a consequence of poor matching for confounding variables. To address this problem, this study contrasted the DTI profiles of limb-onset (ALS-L) and bulbar-onset (ALS-B) in groups that were tightly matched for the potential confounding effects of power, age, cognitive impairment and motor dysfunction.

The impact of physical impairment on emotional well-being in ALS.

There has been evidence that subjective quality of life in patients with amyotrophic lateral sclerosis (ALS) is comparatively good, unrelated to the state of physical functioning, so called 'disability paradox'. Other studies show weak to moderate correlations between disease severity and emotional well-being. Our aim was to analyse the impact of physical impairment on emotional well-being when assessed disease-specifically and seen through the patient's eyes with additional clinical evaluation.

Information needs and information-seeking preferences of ALS patients and their carers.

Our objective was to investigate information-seeking behaviour in patients with ALS and their caregivers and their rating of the usefulness of different information sources in Germany. Surveys were made on 106 patients and 100 caregivers in two university ALS outpatient clinics. Before seeing a doctor, 28% of patients and 23% of caregivers had used other sources to find symptom related information, mostly the internet.

Hippocampal degeneration in patients with amyotrophic lateral sclerosis.

There is increasing appreciation of non-motor system involvement in amyotrophic lateral sclerosis (ALS), although its full extent and clinical significance remains to be established. This study tested the hypothesis that memory impairment in patients with ALS is related to hippocampal degeneration. Consecutive patients with ALS (58) and 29 matched controls participated in standardized neuropsychological assessment and magnetic resonance imaging.

Memory deficits in amyotrophic lateral sclerosis are not exclusively caused by executive dysfunction: a comparative neuropsychological study of amnestic mild cognitive impairment.

Background: Recent work suggests that ALS and frontotemporal dementia can occur together and share at least in part the same underlying pathophysiology. However, it is unclear at present whether memory deficits in ALS stem from a temporal lobe dysfunction, or are rather driven by frontal executive dysfunction. In this study we sought to investigate the nature of memory deficits by analyzing the neuropsychological performance of 40 ALS patients in comparison to 39 amnestic mild cognitive impairment (aMCI) patients and 40 healthy controls (HC).

Making endotracheal intubation easy and successful, particularly in unexpected difficult airway.

Background: Difficult intubation, most often due to poor view of the vocal cords on laryngoscopy is an intermittent and often challenging problem for clinically practicing anesthesiologists, maxillofacial surgeons, ear nose, and throat (ENT), emergency, and critical care physicians.

Purpose: We present a new approach for facilitating difficult intubation and evaluate its efficacy in a retrospective observational study.

Settings And Design: Operating room, emergency department, intensive care unit (ICU), retrospective observational study.

Lesions along the upper motor neuronal pathway with locked-in features after lightning strike and cardiac arrest: a case-review analysis.

Objective: This study describes a case of lesions of the upper motor neuronal pathway with locked-in features after lightning strike and cardiac arrest.

Design: A case-review analysis.

Methods: In a 29-year-old male who was hit by a lightning strike during farming activities, cardiopulmonary resuscitation was provided first by co-workers and continued with success by the medical rescue service.

Working memory in ALS patients: preserved performance but marked changes in underlying neuronal networks.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease which affects the motor system but also other frontal brain regions. In this study we investigated changes in functional neuronal networks including posterior brain regions that are not directly affected by the neurodegenerative process. To this end, we analyzed the contralateral delay activity (CDA), an ERP component considered an online marker of memory storage in posterior cortex, while 23 ALS patients and their controls performed a delayed-matching-to-sample working memory (WM) task.

Pulmonary aspiration in perioperative medicine.

Perioperative aspiration into the lungs is an infrequent but potentially serious cause of anesthesia-related morbidity and mortality. It is still a leading cause of death from pulmonary complications. Aspiration occurs in approximately three per 10.