Experiences of patients with facioscapulohumeral dystrophy with facial weakness: a qualitative study.

Purpose: This study focuses on the functional and psychosocial consequences of facial weakness of patients with facioscapulohumeral muscular dystrophy (FSHD) and how they manage their daily lives.

Materials And Methods: We conducted a qualitative study. Sixteen FSHD patients with varying degrees of facial weakness were interviewed using a semi-structured interview guide.

A visual brain-computer interface as communication aid for patients with amyotrophic lateral sclerosis.

Objective: Brain-Computer Interface (BCI) spellers that make use of code-modulated Visual Evoked Potentials (cVEP) may provide a fast and more accurate alternative to existing visual BCI spellers for patients with Amyotrophic Lateral Sclerosis (ALS). However, so far the cVEP speller has only been tested on healthy participants.

Methods: We assess the brain responses, BCI performance and user experience of the cVEP speller in 20 healthy participants and 10 ALS patients.

Implicit Motives as Determinants of Networking Behaviors.

In today's world of work, networking behaviors are an important and viable strategy to enhance success in work and career domains. Concerning personality as an antecedent of networking behaviors, prior studies have exclusively relied on trait perspectives that focus on how people feel, think, and act. Adopting a motivational perspective on personality, we enlarge this focus and argue that beyond traits predominantly tapping social content, motives shed further light on instrumental aspects of networking - or why people network.

Detecting fasciculations in cranial nerve innervated muscles with ultrasound in amyotrophic lateral sclerosis.

Introduction: Cranial muscle fasciculations may be difficult to detect in amyotrophic lateral sclerosis (ALS). Ultrasound (US) detection of fasciculations in these muscles may have clinical utility.

Methods: Patients with suspected ALS were prospectively enrolled.

Botulinum toxin-A injections vs radiotherapy for drooling in ALS.

Objectives: Botulinum neurotoxin (BoNT) injections in the salivary glands and radiotherapy (RT) on these glands are commonly used to alleviate severe drooling in patients with amyotrophic lateral sclerosis (ALS). This study compares BoNT type A with RT based on patient-rated evaluations.

Materials & Methods: A prospective randomized controlled pilot study to compare RT (n = 10; on the parotid and the posterior part of the submandibular glands) with BoNT-A treatment (n = 10; in the parotid glands only, because of the risk of increasing oropharyngeal weakness) in patients with ALS.

The ALS-FTD-Q: a new screening tool for behavioral disturbances in ALS.

Objective: The assessment of behavioral disturbances in amyotrophic lateral sclerosis (ALS) is important because of the overlap with the behavioral variant of frontotemporal dementia (ALS-bvFTD). Motor symptoms and dysarthria are not taken into account in currently used behavioral questionnaires. We examined the clinimetric properties of a new behavioral questionnaire for patients with ALS (Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire [ALS-FTD-Q]).

Is the Frontal Assessment Battery reliable in ALS patients?

The assessment of frontal functions in ALS patients is important because of the overlap with the behavioural variant of frontotemporal dementia (bvFTD). We investigated the applicability and reliability of the Frontal Assessment Battery (FAB) within a cohort of predominantly prevalent ALS patients. The FAB was administered to 85 ALS patients and eight ALS-bvFTD patients.

Prognostic value of decreased tongue strength on survival time in patients with amyotrophic lateral sclerosis.

Decreased tongue strength (TS) might herald bulbar involvement in patients with amyotrophic lateral sclerosis (ALS) well before dysarthria or dysphagia occur, and as such might be prognostic of short survival. The purpose of this study was to investigate the prognostic value of a decreased TS, in addition to other prognostic factors, such as site of onset, bulbar symptoms, bulbar signs, age, sex, maximum phonation time, time from symptoms to diagnosis, and gastrostomy, for survival time in patients with ALS. TS was measured in four directions in 111 patients who attended the diagnostic outpatient motor neuron clinic of our university hospital.

[Amyotrophic lateral sclerosis].

Amyotrophic lateral sclerosis is one of the most severe and disabling diseases of the nervous system. Amyotrophic lateral sclerosis leads to the progressive weakening of the muscles in the arms, legs, face, mouth and trunk. The onset of the disease is insidious, starting with weakness in the hands or feet or with slurred speech.