Objectives: Only few data are available about the risk of myopathy patients experiencing a cerebral stroke.
Aims: To review the current knowledge about the frequency, pathogenesis, and outcome of stroke in primary/secondary myopathies.
Methods: Literature review of all human studies dealing with stroke in primary/secondary myopathies.
Results: Stroke in myopathies may be either ischemic, metabolic, or cryptogenic. Ischemic stroke may be further classified as cardioembolic, angiopathic, hemodynamic, or thrombophilic. Cardioembolic stroke occurs if there is cardiac involvement in the form of atrial fibrillation/flutter, dilated cardiomyopathy, or non-compaction. Angiopathic stroke occurs if there is atherosclerosis (frequently associated with mitochondrial disorders (MIDs), vasculitis, or dissection in inflammatory myopathies) or MIDs. Thrombophilic stroke may occur in poly-/dermatomyositis if there is additional anti-phospholipid syndrome. Metabolic stroke usually manifests as stroke-like episode and is a distinct feature of various MIDs, particularly MELAS syndrome. The clinical manifestations are due to a vasogenic edema, showing up as hyperintensity on T(2), DWI, and apparent diffusion coefficient mapping. Differentiation between ischemic and metabolic stroke is essential in terms of diagnosis, therapy, and prognosis.
Conclusions: Ischemic stroke due to cardioembolism, arteriopathy, or thrombophilia are rare events in myopathies, but metabolic stroke is a frequent feature of MIDs, with distinct diagnostic and therapeutic implications.