Nens van Alfen1, Jonne Doorduin2, Marieke H J van Rosmalen2, Jeroen J J van Eijk2, Yvonne Heijdra2, Andrea J Boon2, Michael A Gaytant2, Ries J M van den Biggelaar2, Roy T M Sprooten2, Peter J Wijkstra2, Jan T Groothuis2. 1. From the Department of Neurology (N.v.A., J.D., M.H.J.v.R., J.J.J.v.E.) and Department of Rehabilitation (J.T.G.), Donders Institute for Brain, Cognition and Behaviour, and Department of Pulmonary Diseases (Y.H.), Radboud Institute for Health Sciences, Radboud University Medical Center, Nijmegen; Department of Neurology (J.J.v.E.), Jeroen Bosch Hospital, Den Bosch; Radboud University Medical Center, Nijmegen, the Netherlands; Department of Physical Medicine and Rehabilitation (A.J.B.), Mayo Clinic and Foundation, Rochester, MN; Department of Pulmonary Diseases (M.A.G.), Center for Home Mechanical Ventilation, University Medical Center Utrecht; Department of Pulmonary Diseases (R.J.M.v.d.B.), Center for Home Mechanical Ventilation, Erasmus Medical Center, Rotterdam; Department of Pulmonary Diseases (R.T.M.S.), Center for Home Mechanical Ventilation, Maastricht University Medical Center; and Department of Pulmonary Diseases (P.J.W.), Center for Home Mechanical Ventilation, University Medical Center Groningen, the Netherlands. nens.vanalfen@radboudumc.nl. 2. From the Department of Neurology (N.v.A., J.D., M.H.J.v.R., J.J.J.v.E.) and Department of Rehabilitation (J.T.G.), Donders Institute for Brain, Cognition and Behaviour, and Department of Pulmonary Diseases (Y.H.), Radboud Institute for Health Sciences, Radboud University Medical Center, Nijmegen; Department of Neurology (J.J.v.E.), Jeroen Bosch Hospital, Den Bosch; Radboud University Medical Center, Nijmegen, the Netherlands; Department of Physical Medicine and Rehabilitation (A.J.B.), Mayo Clinic and Foundation, Rochester, MN; Department of Pulmonary Diseases (M.A.G.), Center for Home Mechanical Ventilation, University Medical Center Utrecht; Department of Pulmonary Diseases (R.J.M.v.d.B.), Center for Home Mechanical Ventilation, Erasmus Medical Center, Rotterdam; Department of Pulmonary Diseases (R.T.M.S.), Center for Home Mechanical Ventilation, Maastricht University Medical Center; and Department of Pulmonary Diseases (P.J.W.), Center for Home Mechanical Ventilation, University Medical Center Groningen, the Netherlands.
Abstract
OBJECTIVE: To describe the clinical phenotype and recovery of diaphragm dysfunction caused by neuralgic amyotrophy in a large cohort of patients, to improve accurate awareness of this entity, and to encourage adoption of a standardized approach for diagnosis and treatment. METHODS: This observational cohort study recruited adult patients with neuralgic amyotrophy and symptoms of idiopathic phrenic neuropathy from the database of the Dutch expert center for neuralgic amyotrophy and the Dutch centers for home mechanical ventilation. Demographic and clinical information on diagnosis, symptoms, and recovery was obtained from chart review. We attempted to contact all patients for a follow-up interview. RESULTS: Phrenic neuropathy occurs in 7.6% of patients with neuralgic amyotrophy. Unilateral diaphragmatic dysfunction and bilateral diaphragmatic dysfunction are frequently symptomatic, causing exertional dyspnea, orthopnea, disturbed sleep, and excessive fatigue. Diagnostic practices varied widely and were often not optimally targeted. The majority of patients experienced at least moderate recovery within 2 years. CONCLUSION: We recommend screening every patient with neuralgic amyotrophy for diaphragm dysfunction by asking about orthopnea and by performing upright and supine vital capacity screening and diaphragm ultrasound in cases of suspected phrenic neuropathy to optimize diagnosis and care.
OBJECTIVE: To describe the clinical phenotype and recovery of diaphragm dysfunction caused by neuralgic amyotrophy in a large cohort of patients, to improve accurate awareness of this entity, and to encourage adoption of a standardized approach for diagnosis and treatment. METHODS: This observational cohort study recruited adult patients with neuralgic amyotrophy and symptoms of idiopathic phrenic neuropathy from the database of the Dutch expert center for neuralgic amyotrophy and the Dutch centers for home mechanical ventilation. Demographic and clinical information on diagnosis, symptoms, and recovery was obtained from chart review. We attempted to contact all patients for a follow-up interview. RESULTS:Phrenic neuropathy occurs in 7.6% of patients with neuralgic amyotrophy. Unilateral diaphragmatic dysfunction and bilateral diaphragmatic dysfunction are frequently symptomatic, causing exertional dyspnea, orthopnea, disturbed sleep, and excessive fatigue. Diagnostic practices varied widely and were often not optimally targeted. The majority of patients experienced at least moderate recovery within 2 years. CONCLUSION: We recommend screening every patient with neuralgic amyotrophy for diaphragm dysfunction by asking about orthopnea and by performing upright and supine vital capacity screening and diaphragm ultrasound in cases of suspected phrenic neuropathy to optimize diagnosis and care.
Authors: H Stephan Goedee; Ingrid J T Herraets; Leo H Visser; Hessel Franssen; Jan-Thies H van Asseldonk; W Ludo van der Pol; Leonard H van den Berg Journal: Muscle Nerve Date: 2019-07-24 Impact factor: 3.217