Neuromuscular Disease Models and Analysis

2016 ◽  
pp. 349-394 ◽  
Author(s):  
Robert W. Burgess ◽  
Gregory A. Cox ◽  
Kevin L. Seburn
Keyword(s):  
Neuromuscular Disease ◽  
Disease Models

#18 NEUROMUSCULAR DISEASE MODELS FOR EXERCISE CIRCULATORY CONTROL

1981 ◽  
Vol 13 (2) ◽  
pp. 126
Author(s):  
S. F. Lewis ◽  
R. G. Haller ◽  
A. Stein Goldings ◽  
C. G. Blomqvist
Keyword(s):  
Neuromuscular Disease ◽  
Disease Models ◽  
Circulatory Control

scholarly journals NMJ-Analyser: high-throughput morphological screening of neuromuscular junctions identifies subtle changes in mouse neuromuscular disease models

2020 ◽  
Author(s):  
Alan Mejia Maza ◽  
Seth Jarvis ◽  
Weaverly Colleen Lee ◽  
Thomas J. Cunningham ◽  
Giampietro Schiavo ◽  
...  
Keyword(s):  
Machine Learning ◽  
High Throughput ◽  
Neuromuscular Disease ◽  
Structural Changes ◽  
Neuromuscular Junctions ◽  
Learning Algorithm ◽  
Neuromuscular Diseases ◽  
Morphological Structure ◽  
Disease Models ◽  
Neuromuscular Synapses

AbstractThe neuromuscular junction (NMJ) is the peripheral synapse formed between a motor neuron axon terminal and a muscle fibre. NMJs are thought to be the primary site of peripheral pathology in many neuromuscular diseases, but innervation/denervation status is often assessed qualitatively with poor systematic criteria across studies, and separately from 3D morphological structure. Here, we describe the development of ‘NMJ-Analyser’, to comprehensively screen the morphology of NMJs and their corresponding innervation status automatically. NMJ-Analyser generates 29 biologically relevant features to quantitatively define healthy and aberrant neuromuscular synapses and applies machine learning to diagnose NMJ degeneration. We validated this framework in longitudinal analyses of wildtype mice, as well as in four different neuromuscular disease models: three for amyotrophic lateral sclerosis (ALS) and one for peripheral neuropathy. We showed that structural changes at the NMJ initially occur in the nerve terminal of mutant TDP43 and FUS ALS models. Using a machine learning algorithm, healthy and aberrant neuromuscular synapses are identified with 95% accuracy, with 88% sensitivity and 97% specificity. Our results validate NMJ-Analyser as a robust platform for systematic and structural screening of NMJs, and pave the way for transferrable, and cross-comparison and high-throughput studies in neuromuscular diseases.

Neuromuscular Disease Models and Analysis

2009 ◽  
pp. 347-393 ◽  
Author(s):  
Robert W. Burgess ◽  
Gregory A. Cox ◽  
Kevin L. Seburn
Keyword(s):  
Neuromuscular Disease ◽  
Disease Models

Neuromuscular Disease: A Case-Based Approach

2013 ◽  
Author(s):  
John H. J. Wokke ◽  
Pieter A. van Doorn ◽  
Jessica E. Hoogendijk ◽  
Marianne de Visser
Keyword(s):  
Neuromuscular Disease ◽  
Case Based

“Does use of the cough assist machine reduce hospitalisation time for children with neuromuscular disease?”

2014 ◽  
Vol 42 (3) ◽  
Author(s):  
Rachel Phillips ◽  
◽  
Elizabeth Edwards ◽  
David McNamara ◽  
Peter Reed ◽  
...  
Keyword(s):  
Neuromuscular Disease

Testes utilizados para avaliação respiratória nas doenças neuromusculares

1999 ◽  
Vol 15 (1) ◽  
pp. 61-70
Author(s):  
Natasha Nicholson de Santa Maria ◽  
Erica Marques Zanelli ◽  
Marina Brito Silva ◽  
Sabrina Kyoko de Paula Asa ◽  
Francis Meire Fávero ◽  
...  
Keyword(s):  
Reference Values ◽  
Neuromuscular Disease ◽  
Vital Capacity

O objetivo deste estudo consiste em identificar e caracterizar os principais testes utilizados na avaliação da função pulmonar de pacientes com doenças neuromusculares citados em artigos científicos publicados nos últimos 40 anos. Utilizou-se de revisão bibliográfica incluindo estudos publicados nos anos de 1965 a 2005 encontrados nas bases de dados MEDLINE, PUBMED, EMBASE, LILACS e SCIELO que utilizaram descritores na língua portuguesa (doenças neuromusculares, espirometria, capacidade vital, valores de referência) e na inglesa (neuromuscular disease, spirometry, vital capacity reference values). Foram encontrados 9 testes subdivididos em medidas, valores de referência e utilidade. Os testes citados foram: testes de função pulmonar que incluem medidas espirométricas, utilizados para avaliar volumes pulmonares, presença de obstrução de vias aéreas, sinais de fraqueza e fadiga dos músculos respiratórios; testes que avaliam a força dos músculos respiratórios, utilizados para avaliar a força dos músculos respiratórios e a atividade diafragmática isolada; teste que avalia a atividade elétrica de nervos, junção neuromuscular e músculos relacionados a respiração; testes que avaliam o limiar de fadiga dos músculos respiratórios; teste que avalia o grau de relaxamento dos músculos respiratórios, utilizado para analisar a recuperação muscular; testes que avaliam a eficácia da tosse; análise da concentração de gases arteriais, capnografia e oximetria de pulso, utilizadas para avaliar principalmente retenção de CO2 e presença de hipoxemia durante o sono, respectivamente.

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