Diaphragmatic dysfunction in neuromuscular disease, an MRI study

Abstract

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Chest Dynamic MRI as Early Biomarker of Respiratory Impairment in Amyotrophic Lateral Sclerosis Patients: A Pilot Study

“…However, to date and to our knowledge, there is only one study, performed by Harlaar et al [ 9 ], reporting a functional evaluation of the diaphragm with dynamic MRI in patients with ALS. In our study, we aimed to test the usefulness of dynamic MRI as a biomarker in the identification of early respiratory impairment in ALS patients.…”

“…Moreover, the above-mentioned tests provide only partial information on the pathophysiological mechanisms of respiratory failure in ALS patients [ 8 ]. Therefore, new early biomarkers of respiratory impairment are needed, and while spirometry evaluates overall pulmonary function without distinguishing the specific involvement of different respiratory muscles, chest dynamic magnetic resonance imaging (MRI) can help to specifically study separately the function of the diaphragm, the respiratory muscle most compromised in neuromuscular diseases, and of intercostal muscles [ 9 ]. Although it is still difficult to estimate and characterize in depth the function of the diaphragm, the recent advances in MRI technology, such as new and increasingly faster sequences and MRI protocol optimizations, the wide availability of MR tomography, its non-invasiveness and the non-use of ionizing radiation make this tool an excellent opportunity for studying respiratory muscles, especially the diaphragm, and quantifying different aspects of muscle health as already observed by several authors [ 10 , 11 ].…”

“…Nevertheless, for some neuromuscular diseases, such as Pompe disease and Duchenne muscular dystrophy (DMD), the impairment of the respiratory muscles has been widely studied with dynamic MRI [ 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 ]. To date and to our knowledge, there is only one study, performed by Harlaar et al [ 9 ], reporting a functional evaluation of the diaphragm with dynamic MRI in patients with ALS.…”

A Comprehensive Update on Late-Onset Pompe Disease

“…In the upper limbs, involvement of the trapezius inferior, rhomboid, and subscapularis muscles with later moderate damage of the serratus anterior, deltoid, and supraspinatus muscles is usually described [ 37 , 71 ]. Diaphragmatic MRI abnormalities may be present, even when functional pulmonary tests are still within normal range [ 77 , 78 , 79 ].…”

Der Atmungstherapeut in der Neurologie – Ein Blick auf das Berufsbild und ein Ausblick in die Zukunft

“…Viele diagnostische Verfahren wurden insbesondere für neuromuskuläre Erkrankungen wissenschaftlich untersucht, haben aber noch keinen Eingang in die Routinediagnostik gefunden: ▪ Kortikale und zervikale Magnetstimulation des Zwerchfells mit Ableitung des transdiaphragmalen Drucks [40] ▪ Magnetstimulation des Zwerchfells und der Bauchwandmuskulatur mit invasiver Ableitung der resultierenden Druckgradienten in Thorax und Abdomen [41] ▪ Sonographische Bestimmung der Exkursions-und Kontraktionsfähigkeit des Zwerchfells [42] ▪ Korrelation von sonographischen, neurographischen und invasiv-manometrischen Parametern der Zwerchfell-und Phrenicusfunktion mit volitional erhobenen Messgrößen der Lungenfunktion und Atemmuskelkraft [43] ▪ Spirometrisch gesteuerte Magnetresonanztomographie [44] ▪ Messung der Atemmuskelkraft [45] Atmungstherapeutische Interventionen und Aufgaben "Auf neurologischen Weaningstationen werden zunehmend mehr Atmungstherapeuten eingesetzt. Zielgruppe für die Atmungstherapie im Weaningprozess sind v. a. die Patienten, bei denen aufgrund ihrer neuromuskulären Einschränkung mit negativen Auswirkungen auf Mobilität, Sekretclearance und Einschränkungen der Atempumpe gerechnet werden muss" [46].…”