That strange rare disease that causes strokes among young people. The indications of the experts – breaking latest news

Moyamoya angiopathy, which tends to affect children or young adults in the third to fourth decades of life, is a chronic and rare cerebrovascular disease which involves a progressive narrowing – up to obstruction – of the pre-cerebral and cerebral arteries, in particular the carotid arteries and their branches. This causes the development of a network of fragile vesselscalled in Japanese Moyamoya (cloud of smoke), just as it appears in cerebral angiography, the diagnostic test indicated for this condition. Now there are expert recommendations on how to treat it. In fact, they were published in the
European Stroke Journal
the first European guidelines dell’ESO (European Stroke Organization) on the treatment of Moyamoya angiopathy (MMA), presented in a recent webinar organized by ESO.

Lead Partner of the working group, which drafted them, the IRCCS Foundation Carlo Besta Neurological Institute of Milan.

The illness

The illness cause of ischemic strokesdue to the narrowing (stenosis) of the vessels, or hemorrhagic strokes, for ruptured and fragile vessels or aneurysms. Angiopathy occurs most frequently in East Asian countries, especially Japan, and ten times less frequently in Western countries. However, it is believed that the illness is underestimated and theIncreasingly widespread use of MRI in juvenile stroke patients will lead to an increase in the diagnosis, and therefore of the health problem, also in Europe.

I study

This study, carried out by a working group of pathology experts, produced the first European guide on diagnosis and treatment of Moyamoya angiopathy which, thanks to the use of the statistical method GRADE (Grading of Recommendations and Assessment, Development and Evaluation) which allows to evaluate the degree of statistical evidence of the recommendations, it will help physicians choose the most effective management strategy for MMA, comments Dr. Anna Bersano, director of the Neurology 9 complex structure – Cerebrovascular diseases of the IRCCS Carlo Besta Neurological Institute Foundation in Milan, who coordinated the working group that led to the publication of the European recommendations, assisted by Dr. Isabella Canavero – of the same structure – and by Dr. Francesco Acerbi, contact person of the departmental structure operating unit of the Revascularization Center.

Main recommendations

The working group, which involved neurologists, neurosurgeons, statisticians and geneticists, identified nine relevant clinical questions, performed systematic reviews of the literature and, when possible, performed a meta-analysis.
In the adult MMA patients with bleeding presentation recommended direct bypass surgery, after assessing the status of collateral vessels and hemodynamics.
L’revascularization surgery suggestedAlthough there are no randomized trials, also in pediatric patients and MMA patients a ischemic presentationwhen supported by the presence of clinical symptoms or haemodynamic insufficiency.
In the asymptomatic MMA patients an a is suggestedconservative attitude unless the patient has bilateral hemodynamic compromise and brain lesions in the same territory. The bypass surgery it should, however, be done in referral centers with neurosurgical expertise specific.
Experts suggest that direct or combined revascularization is preferable to indirect moles adult and pediatric MMA patientsper reduce the risk of stroke.
Here ppatients with MMA a
non-hemorrhagic presentation recommend a long-term antiplatelet treatment.
In all patients MMA recommended a long-term follow-up with neuroimaging to identify individuals at risk of disease progression.