Search results for "Amy"

Noninvasive Positive Pressure Ventilation in Amyotrophic Lateral Sclerosis

2010

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of unknown origin that affects approximately 1.5 individuals per 100,000 every year; usually, these individuals are between 55 and 75 years of age [1]. ALS causes progressive weakness of voluntary muscle groups, including respiratory ones, and respiratory failure or pneumonia related to respiratory muscle weakness is the most frequent cause of death.

Reaching and Grasping a Glass of Water by Locked-In ALS Patients through a BCI-Controlled Humanoid Robot

2017

Locked-in Amyotrophic Lateral Sclerosis (ALS) patients are fully dependent on caregivers for any daily need. At this stage, basic communication and environmental control may not be possible even with commonly used augmentative and alternative communication devices. Brain Computer Interface (BCI) technology allows users to modulate brain activity for communication and control of machines and devices, without requiring a motor control. In the last several years, numerous articles have described how persons with ALS could effectively use BCIs for different goals, usually spelling. In the present study, locked-in ALS patients used a BCI system to directly control the humanoid robot NAO (Aldebar…

Poor Bone Quality in Patients With Amyotrophic Lateral Sclerosis

2020

Objective: Musculoskeletal functional deterioration in Amyotrophic lateral sclerosis (ALS) is associated with an increase in bone fractures. The purpose of this study was to evaluate the influence of sex, ALS type, on bone quality in patients with ALS compared to healthy controls. The impact on bone health of the clinical status and some metabolic parameters was also analyzed in ALS patients.Methods: A series of 33 voluntary patients with ALS, and 66 healthy individuals matched in sex and age underwent assessment of bone mass quality using quantitative ultrasound (QUS) of the calcaneus. Ultrasonic broadband attenuation (BUA), the speed of sound (SOS), stiffness index and T-score were measur…

Distinct Incidence of Takotsubo Syndrome Between Amyotrophic Lateral Sclerosis and Synucleinopathies : A Cohort Study

2018

Takotsubo syndrome (TTS) is an acute cardiac syndrome characterized by regional left ventricular dysfunction with a peculiar circumferential pattern, which typically results in apical ballooning. Evidence indicates a pivotal role of catecholamines in TTS, and researchers have discussed multiple hypotheses on the etiology, including multivessel coronary spasm, myocardial stunning, excessive transient ventricular afterload, and cardiac sympathetic overactivity with local noradrenaline spillover. Although central nervous system disorders, such as stroke and epilepsy, are known to trigger TTS, the incidence and clinical features of TTS in neurodegenerative disorders are poorly understood. Here,…

P2‐220: Cholesterol and amyloid‐beta: Evidence for a cross‐talk between astrocytes and neuronal cells

2011

Lipid Activates mTORC1 and mTORC2 in the Absorption of Dietary Triglycerides

2018

Mechanistic target of rapamycin (mTOR) senses amino acids; however, its role in lipid metabolism is less established. Organismal lipid requirements are largely met through dietary intake. How nutrient sensing mechanisms in gut interface with dietary fat remains unclear. Here we reveal fundamental and cooperative roles for mTOR complexes 1 and 2 (mTORC1/2) in absorption of dietary triglycerides. Dietary lipid activates mTORC1/2 signaling in gut. Hyperactivating mTORC1 by deleting Tsc1 is sufficient to promote triglyceride absorption and metabolic disease in high fat-fed mice. Conversely, blocking mTORC1/2 by deleting Raptor or Rictor each decreases triglyceride absorption. Loss of Raptor seq…

Continuous ambulatory peritoneal dialysis as a promising therapy for light chain amyloidosis with congestive heart failure

2016

A case of Fitz Hugh Curtis syndrome mimicking an acute abdomen

2021

Fitz Hugh Curtis syndrome, also known as acute perihepatitis, associates pelvic inflammatory disease with the presence of Chlamydia trachomatis or Neisseria gonorrhoeae as the main causative pathogens.
 Symptomatology is a nonspecific one. Right upper quadrant pain, fever, nausea and vomiting are the most commonly encountered symptoms.
 Imaging data are also nonspecific and often show intra-abdominal changes with no particularity.
 As it is difficult to suspect Fitz Hugh Curtis syndrome upon first impression, laparoscopy and direct visualization of the peritoneum and liver adhesions are needed in the diagnostic process. The specific aspect of the fibrinous strands can raise t…

Validation of motor and functional scales for the evaluation of adult patients with 5q spinal muscular atrophy

2021

ABSTRACTObjectiveTo assess in adult spinal muscular atrophy (SMA) patients the construct validity and responsiveness of several outcome measures.MethodsPatients older than 15 years and followed-up at least for 6 months, between October 2015 and August 2020, with one motor function scale (Hammersmith Functional Motor Scale Expanded, HFMSE; Revised Upper Limb module, RULM) in five referral centers were included. Bedside functional scales (Egen Klassification, EK2; Revised Amyotrophic Lateral Sclerosis Functional Rating Scale, ALSFRS-R) were also collected when available. Correlations and regression models were performed to evaluate the construct validity. The monthly slopes of change were use…

2021

Abstract Strong evidence suggests that endoplasmic reticulum stress plays a critical role in the pathogenesis of amyotrophic lateral sclerosis (ALS) through altered regulation of proteostasis. Robust preclinical findings demonstrated that guanabenz selectively inhibits endoplasmic reticulum stress-induced eIF2α-phosphatase, allowing misfolded protein clearance, reduces neuronal death and prolongs survival in in vitro and in vivo models. However, its safety and efficacy in patients with ALS are unknown. To address these issues, we conducted a multicentre, randomized, double-blind trial with a futility design. Patients with ALS who had displayed an onset of symptoms within the previous 18 mon…