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NEUROLOGY 2005;65:E28
© 2005 American Academy of Neurology


Resident and Fellow Page

Atypical territorial infarction in moyamoya disease

J. Y. Lee, MD, K. S. Kim, MD, S. K. Song, MD, S. H. Ahn, MD, H. S. Nam, MD and J. H. Heo, MD, PhD

From the Department of Neurology, National Core Research Center for Nanomedical Technology, Brain Korea 21 Project for Medical Sciences, Yonsei University College of Medicine, Seoul, Korea.

Address correspondence to Dr. Ji Hoe Heo, Department of Neurology, Yonsei University College of Medicine 134 Shinchon-dong, Seodaemoon-ku, 120-752, Seoul, Korea; e-mail: jhheo{at}yumc.yonsei.ac.kr

A 71-year-old woman presented with sudden akinetic mutism. MRI showed an infarction on the anterior portion of the brain. Angiographic studies demonstrated occlusion of both internal carotid arteries at the supraclinoid portion and extensive collaterals (figure), which were suggestive of moyamoya disease.1 Although symptom onset at age 71 is unusual in moyamoya disease, underlying diseases that may be associated with moyamoya-like vasculopathies (moyamoya syndrome) were not found despite the extensive work-ups. An infarction involved only the anterior part of the anterior and middle cerebral artery territories,2 which indicated that distorted vascular territories in moyamoya disease caused an atypical territorial infarction.



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Figure. (A) Diffusion-weighted axial MRI shows an acute infarction involving the bilateral frontal lobes and anterior portions of the basal ganglia. (B) Carotid (CA) and vertebral (VA) angiograms demonstrate occlusion of both internal carotid arteries and well developed bilateral leptomeningeal and basal collaterals from the posterior cerebral and external carotid arteries.

 


Footnotes

Supported by KOSEF through National Core Research Center for Nanomedical Technology (R15-2004-024-00000-0).

Disclosure: The authors report no conflicts of interest.

Received June 16, 2005. Accepted in final form August 3, 2005.

References

  1. Fukui M. Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis ("moyamoya" disease). Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan. Clin Neurol Neurosurg 1997;99(Suppl 2): S238–S240.
  2. Tatu L, Moulin T, Bogousslavsky J, Duvernoy H. Arterial territories of the human brain: Cerebral hemispheres. Neurology 1998;50:1699–1708.[Abstract]



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