Moyamoya like arteriopathy: Neurosonological suspicion and prognosis in adult asymptomatic patients

Introduction

The epidemiology and the prognosis of asymptomatic moyamoya arteriopathy is virtually unknown, mainly in white western population, while symptomatic moyamoya arteriopathy is a more known disease, both in children and in adult people. We are presenting a single centre case series of six asymptomatic adult people with a neurosonological (Transcranial Colour Coded Sonography – TCCS) suspicion of this type of cerebral arteriopathy, confirmed by Digital Subtraction Angiography (DSA).

Patients and methods

During a time period of three years we collected a series of six patients (5 female and 1 male, mean age 29.16 + 8.45 years) with a neurosonological suspicion and a neuroradiological diagnostic confirmation of moyamoya type arteriopathy. All patients underwent TCCS, brain magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) and DSA, besides the differential diagnosis of immunological or infectious etiology. The mean follow-up was 1.8 years.

Results and discussion

All patients but one had a bilateral internal carotid artery (ICA) stenosis at terminus and M1 middle cerebral artery (MCA) multiple stenoses. There is only one young patient with an atypical unilateral pathway and narrowing of extracranial ICA with prominent posterior cerebral artery (PCA) compensation. No clinical events occurred during the follow-up and also brain MRI failed to find new ischemic lesions, compared with the baseline examination.

Conclusions

Asymptomatic cerebral moyamoya arteriopathy is an infrequent but underestimated condition in young white people. More prognostic informations are needed in order to define the natural course and propose the treatment.