scholarly journals Respiratory management for patients with neuromuscular disorders during the COVID-19 pandemic

2020 ◽  
Vol 14 ◽  
pp. 175346662095378
Author(s):  
Tai-Heng Chen ◽  
Jong-Hau Hsu
Keyword(s):  
Neuromuscular Disorders ◽  
Material Section ◽  
Respiratory Management

The reviews of this paper are available via the supplemental material section.

Respiratory muscle imaging by ultrasound and MRI in neuromuscular disorders

2021 ◽  
pp. 2100137
Author(s):  
Jeroen L.M. van Doorn ◽  
Francesca Pennati ◽  
Hendrik H.G. Hansen ◽  
Baziel G.M. van Engelen ◽  
Andrea Aliverti ◽  
...  
Keyword(s):  
Respiratory Muscle ◽  
Imaging Techniques ◽  
Neuromuscular Disorders ◽  
Respiratory Muscles ◽  
Daily Practice ◽  
Structure And Function ◽  
Muscle Structure ◽  
Respiratory Management ◽  
Technological Developments ◽  
And Function

Respiratory muscle weakness is common in neuromuscular disorders and leads to significant respiratory difficulties. Therefore, reliable and easy assessment of respiratory muscle structure and function in neuromuscular disorders is crucial. In the last decade, ultrasound and MRI emerged as promising imaging techniques to assess respiratory muscle structure and function. Respiratory muscle imaging directly measures the respiratory muscles and, in contrast to pulmonary function testing, is independent of patient effort. This makes respiratory muscle imaging suitable to use as tool in clinical respiratory management and as outcome parameter in upcoming drug trials for neuromuscular disorders, particularly in children. In this narrative review, we discuss the latest studies and technological developments in imaging of the respiratory muscles by US and MR, and its clinical application and limitations. We aim to increase understanding of respiratory muscle imaging and facilitate its use as outcome measure in daily practice and clinical trials.

scholarly journals Perioperative Optimization of Patients With Neuromuscular Disorders Undergoing Scoliosis Corrective Surgery: A Multidisciplinary Team Approach

2020 ◽  
pp. 219256822090169
Author(s):  
Fady Sedra ◽  
Roozbeh Shafafy ◽  
Ahmed-Ramadan Sadek ◽  
Syed Aftab ◽  
Alexander Montgomery ◽  
...  
Keyword(s):  
Treatment Plan ◽  
Neuromuscular Disorders ◽  
Multiple Organ ◽  
Neuromuscular Scoliosis ◽  
High Rate ◽  
Wound Complications ◽  
Team Approach ◽  
Corrective Surgery ◽  
Organ Systems ◽  
Respiratory Management

Study Design: Narrative review. Objective: The high rate of complications associated with the surgical management of neuromuscular spinal deformities is well documented in the literature. This is attributed to attenuated protective physiological responses in multiple organ systems. Methods: Review and synthesis of the literature pertaining to optimization of patients with neuromuscular scoliosis undergoing surgery. Our institutional practice in the perioperative assessment and management of neuromuscular scoliosis is also described along with a clinical vignette. Results: Respiratory complications are the most common to occur following surgery for neuromuscular disorders. Other categories include gastrointestinal, cardiac, genitourinary, blood loss, and wound complications. A multidisciplinary approach is required for perioperative optimization of these patients and numerous strategies are described, including respiratory management. Conclusion: Perioperative optimization for patients with neuromuscular disorders undergoing corrective surgery for spinal deformity is multifaceted and complex. It requires a multidisciplinary evidence-based approach. Preadmission of patients in advance of surgery for assessment and optimization may be required in certain instances to identify key concerns and formulate a tailored treatment plan.

scholarly journals Common recessive limb girdle muscular dystrophies differential diagnosis: why and how?

2014 ◽  
Vol 72 (9) ◽  
pp. 721-734 ◽  
Author(s):  
Ana Cotta ◽  
Elmano Carvalho ◽  
Antonio Lopes da-Cunha-Júnior ◽  
Júlia Filardi Paim ◽  
Monica M. Navarro ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Muscular Dystrophy ◽  
Muscle Biopsy ◽  
Neuromuscular Disorders ◽  
Limb Girdle Muscular Dystrophy ◽  
Muscular Dystrophies ◽  
Muscle Involvement ◽  
Respiratory Management ◽  
Diagnostic Flowchart ◽  
And Function

Limb girdle muscular dystrophies are heterogeneous autosomal hereditary neuromuscular disorders. They produce dystrophic changes on muscle biopsy and they are associated with mutations in several genes involved in muscular structure and function. Detailed clinical, laboratorial, imaging, diagnostic flowchart, photographs, tables, and illustrated diagrams are presented for the differential diagnosis of common autosomal recessive limb girdle muscular dystrophy subtypes diagnosed nowadays at one reference center in Brazil. Preoperative image studies guide muscle biopsy site selection. Muscle involvement image pattern differs depending on the limb girdle muscular dystrophy subtype. Muscle involvement is conspicuous at the posterior thigh in calpainopathy and fukutin-related proteinopathy; anterior thigh in sarcoglycanopathy; whole thigh in dysferlinopathy, and telethoninopathy. The precise differential diagnosis of limb girdle muscular dystrophies is important for genetic counseling, prognostic orientation, cardiac and respiratory management. Besides that, it may probably, in the future, provide specific genetic therapies for each subtype.

Expression of myogenic regulatory factors (MRFs) in human neuromuscular disorders

1997 ◽  
Vol 23 (6) ◽  
pp. 475-482 ◽  
Author(s):  
M. Olive ◽  
J. A. Martinez-Matos ◽  
P. Pirretas ◽  
M. Povedano ◽  
C. Navarro ◽  
...  
Keyword(s):  
Neuromuscular Disorders ◽  
Myogenic Regulatory Factors ◽  
Regulatory Factors

Management of cardiac involvement in neuromuscular disorders

2006 ◽  
Vol 48 (10) ◽  
pp. 863
Author(s):  
Josef Finsterer ◽  
Claudia Stöllberger
Keyword(s):  
Cardiac Involvement ◽  
Neuromuscular Disorders
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