Moyamoya disease (MMD) is a rare cerebrovascular disease with an unknown etiology
and is characterized by intrinsic fragility in the intracranial vascular walls such
as the affected internal elastic lamina and thinning medial layer. The association
of MMD with intracranial arterial dissection is extremely rare, whereas that with
basilar artery dissection (BAD) has not been reported previously. A 46-year-old woman
developed brain stem infarction due to BAD 4 years after successful bilateral superficial
temporal artery–middle cerebral artery anastomosis with indirect pial synangiosis
for ischemic-onset MMD. She presented with sudden occipitalgia and subsequently developed
transient dysarthria and mild hemiparesis. Although a transient ischemic attack was
initially suspected, her condition deteriorated in a manner that was consistent with
left hemiplegia with severe dysarthria. Magnetic resonance (MR) imaging revealed brain
stem infarction, and MR angiography delineated a double-lumen sign in the basilar
artery, indicating BAD. She was treated conservatively and brain stem infarction did
not expand. One year after the onset of brain stem infarction, her activity of daily
living is still dependent (modified Rankin Scale of 4), and there were no morphological
changes associated with BAD or recurrent cerebrovascular events during the follow-up
period. The association of MMD with BAD is extremely rare. While considering the common
underlying pathology such as an affected internal elastic lamina and fragile medial
layer, the occurrence of BAD in a patient with MMD in a stable hemodynamic state is
apparently unique.
Key Words
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Article info
Publication history
Published online: April 08, 2016
Accepted:
March 15,
2016
Received:
March 1,
2016
Footnotes
Grant support: This research was partially supported by a grant (J150001575) from the Japan Agency for Medical Research and Development (AMED).
Identification
DOI: https://doi.org/10.1016/j.jstrokecerebrovasdis.2016.03.024
Copyright
© 2016 National Stroke Association. Published by Elsevier Inc. All rights reserved.
