Myotonic Dystrophy, Mitral-Valve Prolapse, and Stroke

Cook, Andrew W.; Bird, T.; Spence, Alexander M.; Pagon, Roberta A; Wallace, James F.

doi:10.1016/s0140-6736(78)90113-7

Cited by 21 publications

(9 citation statements)

References 8 publications

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“…Clinical observations of multiple events occurring in several vascular distributions -angiographic observation of intracranial branch occlusions in the absence of atheromatous or other disease and pathologic observations of the value at surgery or postmortem -support this presumed mechanism. 27,28 The shortened platelet survival time in some patients further suggests a thromboembolic mechanism. 29 In spite of the increased prevalence of focal cerebral infarction noted in our patients with mitral valve prolapse, the annual incidence of new symptoms in this group of patients is likely to be low, and we have not advocated preventive treatment (other than antibiotic prophylaxis) in patients with mitral valve prolapse who have not had focal ischemic symptoms.…”

Section: Discussionmentioning

confidence: 99%

Cerebral ischemic events in patients with mitral valve prolapse.

Sandok¹,

Giuliani²

1982

Stroke

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SUMMARY All patients 20 years old or older referred for echocardiographic examination and found to have mitral valve prolapse during the period January 1975 through December 1979 were included in the study. Of the 1,138 patients, two-thirds were women and one-third were men. Their average age was 48.4 years. Forty patients (3.5%) had histories of prior focal cerebrovascular ischemic events. In 26 of the 40 patients, no responsible mechanism other than mitral valve prolapse was identified, and in 4, the ischemic event occurred during an episode of bacterial endocarditis, a known complication of mitral valve prolapse. In 10 of the 26 patients, there was clinical information to suggest an embolic mechanism for the ischemia. A conservative estimate of the prevalence rate for cerebral infarction in this group of patients is four times greater than the rate expected in a normal population. This difference is likely due to the contribution of mitral valve prolapse in the pathogenesis of cerebral infarction. Stroke, Vol 13, No 4, 1982 MITRAL VALVE PROLAPSE is a relatively common condition. Based upon the criteria utilized, mitral valve prolapse may occur in approximately 6% of the young female and a lesser percentage of the young male population.

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Section: Discussionmentioning

confidence: 99%

Cerebral ischemic events in patients with mitral valve prolapse.

Sandok¹,

Giuliani²

1982

Stroke

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“…He had a history of atrial fibrillation but also of dilative cardiomyopathy. Transient ischemic attack and stroke have been also described in a patient with myotonic dystrophy 1 in whom paroxysmal atrial fibrillation was recorded [11]. Screening of 52 patients with DMD, 61 with myotonic dystrophy 1 and 14 with Becker muscular dystrophy revealed atrial flutter in 3 with MD1, and 1 with DMD.…”

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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“…10,22 Although there have been four cases of autopsied fatal embolism reported, in one case there was no thrombus seen in the heart. 23 Two cases had another predisposing factor (one had atrial fibrillation and one had an adenocarcinoma), 24,25 There was only one case with documented thrombus on the atrial surface of the posterior mitral valve leaflet. 6 Second, based on their observations in young stroke patients, this causal relationship between cerebral ischemic attacks and MVP has been postulated mainly by neurologists.…”

Section: Discussionmentioning

confidence: 99%

Mitral Valve Prolapse with Systemic Embolism: Report of Three Cases

et al. 1989

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Mitral valve prolapse (MVP) is a common condition in the developed countries and apparently is the most frequently diagnosed valvular heart disease in the United States, with an overall prevalence of 5% and as high as 17% in young females.1 Several clinical studies have suggested that MVP is associated with certain serious complications such as progressive mitral regurgitation, bacterial endocarditis, arrhythmias, and sudden death. Moreover, cerebral and ophthalmic ischemic episodes, presumably secondary to embolism, have been reported recently as additional complications of MVP. [3][4][5][6][7][8][9][10][11][12][13] It is generally believed that rheumatic fever is still a major problem in the developing countries.14 Thus, it is assumed that the commonest form of valvular heart disease in these areas is rheumatic in origin. A recent study has shown that the prevalence rate for MVP in Saudi Arabia is comparable to that reported from the Western countries. 15There should be an awareness of its occurrence and possible complications in the developing countries.We, therefore, present case reports of three young Saudis with MVP complicated by cerebral and retinal embolism and review the relevant literature. Similar complications have been reported previously only from Canada, 3-5 England, 6 South Africa, 7 France, 8 and the United States, 9-13 but to our knowledge, these are the first cases reported from the Middle East and Asia of cerebral and retinal embolic phenomena associated with MVP. Case Reports Case 1A 16-year-old Saudi girl was referred to the University Hospital because of sudden blindness in the right eye which developed about 12 hours prior to presentation. There was no history of acute rheumatic fever, heart disease, dyspnea, chest pain, palpitations, dizziness, or previous visual symptoms. Ophthalmic examination revealed blindness of the right eye with only perception to light. Features of right central retinal artery occlusion were seen on funduscopy. Carotid pulses were normal, and there was no carotid artery bruit. She was afebrile, and the cardiac apex was in the normal location with no thrill or heaves. First and second heart sounds were normal. There was a grade 3/6 late systolic murmur at the apex, which was accentuated by hand grip. No click, gallop, or opening snap was heard. Results of all hematologic and biochemical investigations were normal. Blood culture showed no growths. Electrocardiograms and chest roentgenogram were normal. No arrhythmia was observed on a 24-hour Holter monitor recording. Echocardiogram showed holosystolic MVP with normal left atrial and left ventricular size. Case 2For a month prior to his referral, a 23-year-old Saudi man experienced recurrent attacks of blurred vision lasting a few minutes at a time. Four days before his visit to the University Hospital, he suddenly developed partial blindness in the mid-zone of the visual field of the left eye. At presentation, he denied any history of rheumatic fever, palpitation, dizziness, chest pain, or dyspnea. Ophthalmic eval...

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Myotonic Dystrophy, Mitral-Valve Prolapse, and Stroke

Cited by 21 publications

References 8 publications

Cerebral ischemic events in patients with mitral valve prolapse.

Cerebral ischemic events in patients with mitral valve prolapse.

Stroke and Stroke-like Episodes in Muscle Disease

Mitral Valve Prolapse with Systemic Embolism: Report of Three Cases

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