Moyamoya syndrome: a defensive response to the chronic damage of a large cerebral vessel. Cases report and review of the literature.
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Keywords

Brain ischemia
cerebrovascular accident
cerebral angiography
Moyamoya disease
neurofibromatosis 1
Stent (MeSH)

Abstract

OBJECTIVES:

To present some cases of patients with Moyamoya syndrome secondary to diverse etiologies, and, based on them, to review general aspects of the clinical picture. Supported on two cases, the hypothesis of a new mechanism is proposed.

MATERIALS AND METHODS:

Four clinical cases of Moyamoya syndrome are described, identified in the city of Medellín, Colombia, with their respective images and the evolution of their condition. Based on two of the cases, in which the syndrome was triggered after the implantation of a flow-diverter stent, a new etiology is proposed.

RESULTS:

In two cases, the implantation of a flow-diverter stent was identified as the trigger of the Moyamoya picture. In one case the primary disease was type 1 neurofibromatosis; in the fourth case it was not possible to identify the cause of the syndrome. None of the patients presented neurological compromise despite severe arterial damage.

CONCLUSIONS:

Moyamoya syndrome (MMS), rather than an independent entity, should be understood as a compensatory and defensive mechanism in response to the deprivation of blood flow in the anterior cerebral circulation, which protects patients from ischemic cerebrovascular events. Implantation of flow-diverter stents, as a method of treatment of some cerebral aneurysms, is projected in time as an important cause of this syndrome.

https://doi.org/10.22379/24224022178

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