| Autor: |
Harrison, Emma L, Devlin, Michael J, Stoodley, Marcus A, White, Richard P |
| Zdroj: |
Journal of Neurology, Neurosurgery, & Psychiatry (JNNP); 2018, Vol. 89 Issue: 6 pA38-A38, 1p |
| Abstrakt: |
IntroductionInitially described in Japan in 1957, moyamoya angiopathy is recognised in other populations, particularly within Europe and North America. Moyamoya disease (MMD) is a rare chronic progressive cerebrovascular disorder of unknown aetiology characterised by concentric stenosis of large intracranial vessels with collateralisation from moyamoya vessels and the leptomeningeal vessels. Affected individuals may present with transient ischaemic attacks or ischaemic stroke, however presentations with intracerebral haemorrhage, epilepsy or cognitive impairment in both children and adults also occur. Those with moyamoya syndrome (MMS), MMD in the setting of an associated disease such as neurofibromatosis, type one diabetes mellitus and trisomy 21, appear to follow a similar clinical course.MethodsWe conducted a retrospective review of patients with Moyamoya angiopathy who presented to the Townsville or Mackay Hospital Health Service between January 2012 and January 2018.ResultsWe identified eleven cases of moyamoya angiopathy between 2012 and 2018. The cumulative incidence in our study population was 0.436 per 1 00 000 persons per year. The most common presentation was ischaemic stroke. We will present additional morphologic data, functional cerebral blood flow studies and outcomes following revascularisation (including ECIC bypass).ConclusionThe findings from our small case series suggests the incidence of moyamoya angiopathy within Northern Queensland is higher than anticipated in European and Northern American populations. Additional research is required to ascertain whether this is primarily due to an increased awareness of the condition and extended diagnostic workup within our centre, specific patient characteristics which predispose to the disease or due to increasing prevalence of moyamoya angiopathy in the general population. |
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