Brain Stem Infarction Due to Basilar Artery Dissection in a Patient with Moyamoya Disease Four Years after Successful Bilateral Revascularization Surgeries

• Published in: Journal of stroke and cerebrovascular diseases Vol. 25; no. 6; pp. e79 - e82
• Main Authors: Abe, Takatsugu, MD, Fujimura, Miki, MD, PhD, Mugikura, Shunji, MD, PhD, Endo, Hidenori, MD, PhD, Tominaga, Teiji, MD, PhD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-06-2016
• Subjects: Activities of Daily Living; Aneurysm, Dissecting - diagnostic imaging; Aneurysm, Dissecting - etiology; Aneurysm, Dissecting - physiopathology; Aneurysm, Dissecting - therapy; Basilar Artery - diagnostic imaging; Basilar artery dissection; brain stem infarction; Brain Stem Infarctions - diagnostic imaging; Brain Stem Infarctions - etiology; Brain Stem Infarctions - physiopathology; Brain Stem Infarctions - therapy; Cardiovascular; Cerebral Angiography - methods; Diffusion Magnetic Resonance Imaging; Disability Evaluation; Female; Humans; Intracranial Aneurysm - diagnostic imaging; Intracranial Aneurysm - etiology; Intracranial Aneurysm - physiopathology; Intracranial Aneurysm - therapy; Magnetic Resonance Angiography; magnetic resonance imaging; Middle Aged; moyamoya disease; Moyamoya Disease - complications; Moyamoya Disease - diagnostic imaging; Moyamoya Disease - surgery; Neurology; Neurosurgical Procedures; Recovery of Function; Time Factors; Treatment Outcome; Basilar artery dissection; magnetic resonance imaging; brain stem infarction; moyamoya disease
• ISSN: 1052-3057; 1532-8511
• DOI: 10.1016/j.jstrokecerebrovasdis.2016.03.024

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.