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Stroke in myopathies
Josef Finsterer1, Claudia Stollberger
1Neurological Department, Krankenanstalt Rudolfstiftung, Vienna, Austria. fifigs1@yahoo.de
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.
Insights
Stroke in myopathy patients is rare, but metabolic stroke, particularly in mitochondrial disorders (MIDs), is common. Differentiating between ischemic and metabolic stroke is crucial for diagnosis and treatment.
Area of Science:
- Neurology
- Genetics
- Internal Medicine
Background:
- Limited data exist on cerebral stroke risk in myopathy patients.
- Understanding stroke in primary/secondary myopathies is crucial.
Purpose of the Study:
- To review current knowledge on stroke frequency, pathogenesis, and outcomes in myopathies.
- To differentiate between ischemic and metabolic stroke in myopathy patients.
Main Methods:
- Comprehensive literature review of human studies on stroke in primary/secondary myopathies.
Main Results:
- Stroke in myopathies can be ischemic (cardioembolic, angiopathic, hemodynamic, thrombophilic) or metabolic.
- Metabolic stroke, often presenting as stroke-like episodes, is characteristic of mitochondrial disorders (MIDs), especially MELAS syndrome.
- Ischemic stroke subtypes are linked to cardiac issues, atherosclerosis, vasculitis, or thrombophilia.
Conclusions:
- While ischemic strokes are rare in myopathies, metabolic stroke is a frequent and significant feature of MIDs.
- Distinct diagnostic and therapeutic approaches are necessary for metabolic stroke in MIDs compared to ischemic stroke.
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