METHODS:
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: