Automatic single-trial classification of prefrontal hemodynamic activity in an individual with Duchenne muscular dystrophy

2012 ◽  
Vol 16 (1) ◽  
pp. 67-72 ◽  
Author(s):  
Sarah Dianne Power ◽  
Tom Chau
2020 ◽  
Vol 21 (11) ◽  
pp. 3795
Author(s):  
Eleni Peristeri ◽  
Athina-Maria Aloizou ◽  
Paraskevi Keramida ◽  
Zisis Tsouris ◽  
Vasileios Siokas ◽  
...  

Myopathies represent a wide spectrum of heterogeneous diseases mainly characterized by the abnormal structure or functioning of skeletal muscle. The current paper provides a comprehensive overview of cognitive deficits observed in various myopathies by consulting the main libraries (Pubmed, Scopus and Google Scholar). This review focuses on the causal classification of myopathies and concomitant cognitive deficits. In most studies, cognitive deficits have been found after clinical observations while lesions were also present in brain imaging. Most studies refer to hereditary myopathies, mainly Duchenne muscular dystrophy (DMD), and myotonic dystrophies (MDs); therefore, most of the overview will focus on these subtypes of myopathies. Most recent bibliographical sources have been preferred.


2010 ◽  
Vol 25 (9) ◽  
pp. 1103-1109 ◽  
Author(s):  
Susan Sienko Thomas ◽  
Cathleen E. Buckon ◽  
Alina Nicorici ◽  
Anita Bagley ◽  
Craig M. McDonald ◽  
...  

Neurology ◽  
2019 ◽  
Vol 93 (13) ◽  
pp. e1312-e1323 ◽  
Author(s):  
Eric P. Hoffman ◽  
Benjamin D. Schwartz ◽  
Laurel J. Mengle-Gaw ◽  
Edward C. Smith ◽  
Diana Castro ◽  
...  

ObjectiveTo study vamorolone, a first-in-class steroidal anti-inflammatory drug, in Duchenne muscular dystrophy (DMD).MethodsAn open-label, multiple-ascending-dose study of vamorolone was conducted in 48 boys with DMD (age 4–<7 years, steroid-naive). Dose levels were 0.25, 0.75, 2.0, and 6.0 mg/kg/d in an oral suspension formulation (12 boys per dose level; one-third to 10 times the glucocorticoid dose in DMD). The primary goal was to define optimal doses of vamorolone. The primary outcome for clinical efficacy was time to stand from supine velocity.ResultsOral administration of vamorolone at all doses tested was safe and well tolerated over the 24-week treatment period. The 2.0–mg/kg/d dose group met the primary efficacy outcome of improved muscle function (time to stand; 24 weeks of vamorolone treatment vs natural history controls), without evidence of most adverse effects of glucocorticoids. A biomarker of bone formation, osteocalcin, increased in vamorolone-treated boys, suggesting possible loss of bone morbidities seen with glucocorticoids. Biomarker outcomes for adrenal suppression and insulin resistance were also lower in vamorolone-treated patients with DMD relative to published studies of glucocorticoid therapy.ConclusionsDaily vamorolone treatment suggested efficacy at doses of 2.0 and 6.0 mg/kg/d in an exploratory 24-week open-label study.Classification of evidenceThis study provides Class IV evidence that for boys with DMD, vamorolone demonstrated possible efficacy compared to a natural history cohort of glucocorticoid-naive patients and appeared to be tolerated.


2021 ◽  
Vol 90 ◽  
pp. 291-292
Author(s):  
I. Vandekerckhove ◽  
E. Papageorgiou ◽  
M. Van den Hauwe ◽  
A. Van Campenhout ◽  
L. De Waele ◽  
...  

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