Intracardiac Thrombus and Cerebral Infarction in a Patient With Duchenne Muscular Dystrophy

Gimenez-Munoz, A; Capablo, J.L.; Alarcia, Raquel; Torné, Laura; Errea, José M.

doi:10.1097/cnd.0b013e3181b51833

Cited by 7 publications

(5 citation statements)

References 7 publications

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“…Ischemic strokes in DMD have previously been described in a small number of patients, [2][3][4][5][6][7] but epidemiological data on cerebral infarction in DMD and in primary myopathies in general are scarce. 9 In a prospective observational study published in Stroke 1987, 10 none of 52 DMD patients were reported as having cerebral infarction.…”

Section: Discussionmentioning

confidence: 99%

“…Although cardiomyopathy is a frequent and prognosis-defining feature of DMD, just a small number of cases with stroke in DMD patients has been reported to date (Table). [2][3][4][5][6][7] In this study, we retrospectively analyzed the frequency, cause, and outcome of stroke in a German cohort of 54 DMD patients, who lived in a regional facility for handicapped people between 1963 and 2013.…”

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confidence: 99%

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Stroke in Duchenne Muscular Dystrophy

Winterholler

Holländer

Kerling

et al. 2016

Stroke

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To analyze the frequency, cause, and outcome of ischemic and hemorrhagic stroke in DMD, we retrospectively reviewed the clinical data of 59 male patients, who lived at the Wichernhaus in Altdorf, a specialized learning and living facility for handicapped children and young adults near Nuremberg in Germany, between 1963 and 2013. Data were collected from the DMD patients' medical files obtained from the treating Department of Neurology and Department of Orthopedics of the Rummelsberg Hospital, a teaching hospital of the Friedrich-Alexander University of Erlangen-Nürnberg, Germany, and from a local general practitioner serving the facility. Data collection was authorized by the ethics committee of the FriedrichAlexander University of Erlangen-Nürnberg. Informed consent from patients was not required based on the retrospective analyses of medical records.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.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Stroke in Duchenne Muscular Dystrophy

Winterholler

Holländer

Kerling

et al. 2016

Stroke

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“…However, cases of left ventricle non-compaction have been reported in DMD [22]. Cardiac thrombus and cerebral infarction are rarely reported, mainly in DMD patients with severe heart failure [23]. Cardiac thrombus may be related to a significant activation of the coagulation system in DMD with heart failure [24].…”

Section: Cardiomyopathy Arrhythmia and Conduction Abnormalitiesmentioning

confidence: 99%

Cardiac Involvement Classification and Therapeutic Management in Patients with Duchenne Muscular Dystrophy

Fayssoil

Abasse²,

Silverston

2017

JND

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Duchenne muscular dystrophy (DMD) is an inherited myogenic disorder due to mutations in the dystrophin gene on chromosome Xp21.1. The clinical picture included peripheral muscle weakness, cardiomyopathy and chronic respiratory insufficiency. In this paper, the authors review cardiac involvement in patients with DMD, propose a cardiac impairment classification and discuss therapeutic management options.

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“…Cardiac involvement predisposing for ischemic stroke includes sarrhythmias, such as atrial fibrillation, heart failure from hypertrophic cardiomoypathy, dilative cardiomyopathy, restrictive cardiomoypathy, or noncompaction without heart failure. Another risk factor for ischemic stroke may be cryptogenic intraventricular thrombus formation in the absence of atrial fibrillation or heart failure, as has been described in Duchenne muscular dystrophy (DMD) [7]. …”

Section: Risk Factors For Stroke In Myopathiesmentioning

confidence: 99%

“…In a single patient with DMD but without a history of atrial fibrillation or heart failure ischemic stroke occurred due to embolisation of an intracardiac thrombus [7]. The cause of thrombus formation in this patient, however, remained elusive.…”

Section: Etiology Of Ischemic Stroke In Myopathiesmentioning

confidence: 99%

Stroke and Stroke-like Episodes in Muscle Disease

Finsterer¹

2012

TONEUJ

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Background:Though not obvious at a first glance, myopathies may be associated with ischemic stroke. Stroke-like episodes resemble ischemic stroke only to some extent but are a unique feature of certain mitochondrial disorders with a pathogenesis at variance from that of ischemic stroke. Only limited data are available about ischemic stroke in pri-mary myopathies and the management of stroke-like episodes in mitochondrial disorders. This review aims to summarize and discuss current knowledge about stroke in myopathies and to delineate stroke-like episodes from ischemic stroke. Methods:Literature review via PubMED using the search terms “stroke”, “cerebrovascular”, “ischemic event”, “stroke-like episode”, “stroke-mimic”, “mitochondrial disorder”. Results:Stroke in myopathies is most frequently cardioembolic due to atrial fibrillation or atrial flutter, dilated cardio-myopathy, or left-ventricular hypertrabeculation (noncompaction). The second most frequent cause of stroke in myopathies is angiopathy from atherosclerosis or vasculitis, which may be a feature of inflammatory myopathies. Athero-sclerosis may either result from classical risk factors, such as diabetes, arterial hypertension, hyperlpidemia, or smoking, associated with muscle disease, or may be an inherent feature of a mitochondrial disorder. In case of severe heart failure from cardiomyopathy as a manifestation of muscle disease low flow infarcts may occur. Thrombophilic stroke has been described in polymyositis and dermatomyositis in association with anti-phospholipid syndrome. Stroke-like episodes occur particularly in mitochondrial encephalopathy, lactacidosis and stroke-likeepisode syndrome but rarely also in Leigh-syndrome and other mitochondrial disorders. Stroke-like episodes are at variance from ischemic stroke, pathogenically, clinically and on imaging. They may be the manifestation of a vascular, metabolic or epileptic process and present with predominantly vasogenic but also cytotoxic edema on MRI. Differentiation between ischemic stroke and stroke-like episodes is essential in terms of management and prognosis. Management of ischemic stroke in patients with myopathy is not at variance from the treatment of ischemic stroke in non-myopathic patients. There is no standardized treatment of stroke-like episodes but there is increasing evidence that these patients profit from the administration of L-arginine and conse-quent antiepileptic treatment if associated with seizure activity. Conclusions:Ischemic stroke may be a complication of myopathy and needs to be delineated from stroke-like episodes, which are unique to mitochondrial disorders, particularly mitochondrial encephalopathy, lactacidosis and stroke-likeepisode syndrome. Ischemic stroke in myopathies is most frequently cardioembolic and treatment is not at variance from non-myopathic ischemic stroke. Treatment of stroke-like episodes is not standardized but seems to respond to L-arginine and adequate antiepileptic treatment.

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Intracardiac Thrombus and Cerebral Infarction in a Patient With Duchenne Muscular Dystrophy

Cited by 7 publications

References 7 publications

Stroke in Duchenne Muscular Dystrophy

Stroke in Duchenne Muscular Dystrophy

Cardiac Involvement Classification and Therapeutic Management in Patients with Duchenne Muscular Dystrophy

Stroke and Stroke-like Episodes in Muscle Disease

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