Martin Winterholler1, Christian Holländer2, Frank Kerling2, Irina Weber2, Sven Dittrich2, Matthias Türk2, Rolf Schröder2. 1. From the Department of Neurology, Hospital Rummelsberg, Schwarzenbruck, Germany (M.W., F.K., I.W.); Rummelsberger Diakonie, Wichernhaus Altdorf, Altdorf, Germany (C.H.); and Department of Pediatric Cardiology (S.D.), Department of Neurology (M.T.), and Institute of Neuropathology (R.S.), Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany. martin.winterholler@sana.de. 2. From the Department of Neurology, Hospital Rummelsberg, Schwarzenbruck, Germany (M.W., F.K., I.W.); Rummelsberger Diakonie, Wichernhaus Altdorf, Altdorf, Germany (C.H.); and Department of Pediatric Cardiology (S.D.), Department of Neurology (M.T.), and Institute of Neuropathology (R.S.), Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
Abstract
BACKGROUND AND PURPOSE: Duchenne muscular dystrophy (DMD) is the most frequent skeletal muscle myopathy. Nearly all patients develop cardiomyopathy in their second decade of life. The purpose of this study was to evaluate the frequency, cause, and outcome of stroke in a German cohort of patients with DMD. METHODS: Retrospective analysis of medical records of 54 DMD patients, who lived in a regional facility for handicapped people (Wichernhaus Altdorf, Germany) between 1963 and 2013. RESULTS: Fifty-four DMD patients were followed up for 7.4 years on average. Mean age at admission and discharge from the long-term care facility or death were 11.4 and 18.8 years, respectively. Covering a total observation period of 400 patient-years, we identified 4 DMD patients with juvenile arterial ischemic strokes. Off-label systemic thrombolysis in 2 patients resulted in a nearly complete regression of stroke-related symptoms, but 1 patient died of septic pneumonia and cardiac failure 24 days after thrombolysis therapy. In the other 2 patients, who had their ischemic strokes in 1994 and 1998, severe infarction-related symptoms persisted, and 1 patient died 13 days later. DMD-associated cardiomyopathy without evidence of atrial fibrillation was the only risk factor for ischemic stroke in all patients. CONCLUSIONS: This study indicates an increased risk for ischemic strokes in DMD patients. Regular cardiological assessment of all DMD patients is mandatory to evaluate the individual risk profile for cardioembolic events and to adapt therapeutic strategies.
BACKGROUND AND PURPOSE:Duchenne muscular dystrophy (DMD) is the most frequent skeletal muscle myopathy. Nearly all patients develop cardiomyopathy in their second decade of life. The purpose of this study was to evaluate the frequency, cause, and outcome of stroke in a German cohort of patients with DMD. METHODS: Retrospective analysis of medical records of 54 DMDpatients, who lived in a regional facility for handicapped people (Wichernhaus Altdorf, Germany) between 1963 and 2013. RESULTS: Fifty-four DMDpatients were followed up for 7.4 years on average. Mean age at admission and discharge from the long-term care facility or death were 11.4 and 18.8 years, respectively. Covering a total observation period of 400 patient-years, we identified 4 DMDpatients with juvenile arterial ischemic strokes. Off-label systemic thrombolysis in 2 patients resulted in a nearly complete regression of stroke-related symptoms, but 1 patient died of septic pneumonia and cardiac failure 24 days after thrombolysis therapy. In the other 2 patients, who had their ischemic strokes in 1994 and 1998, severe infarction-related symptoms persisted, and 1 patient died 13 days later. DMD-associated cardiomyopathy without evidence of atrial fibrillation was the only risk factor for ischemic stroke in all patients. CONCLUSIONS: This study indicates an increased risk for ischemic strokes in DMDpatients. Regular cardiological assessment of all DMDpatients is mandatory to evaluate the individual risk profile for cardioembolic events and to adapt therapeutic strategies.
Authors: David C Schorling; Cornelia K Müller; Astrid Pechmann; Sabine Borell; Thorsten Langer; Simone Thiele; Maggie C Walter; Barbara Zieger; Janbernd Kirschner Journal: Acta Myol Date: 2020-03-01
Authors: Sven Dittrich; Erika Graf; Regina Trollmann; Ulrich Neudorf; Ulrike Schara; Antje Heilmann; Maja von der Hagen; Brigitte Stiller; Janbernd Kirschner; Robert Dalla Pozza; Wolfgang Müller-Felber; Katja Weiss; Katja von Au; Markus Khalil; Reinald Motz; Christoph Korenke; Martina Lange; Ekkehard Wilichowski; Joseph Pattathu; Friedrich Ebinger; Nicola Wiechmann; Rolf Schröder Journal: Orphanet J Rare Dis Date: 2019-05-10 Impact factor: 4.123
Authors: Maria Razzoli; Angus Lindsay; Michelle L Law; Christopher M Chamberlain; William M Southern; Madeleine Berg; John Osborn; William C Engeland; Joseph M Metzger; James M Ervasti; Alessandro Bartolomucci Journal: EBioMedicine Date: 2020-03-16 Impact factor: 8.143