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Letters   |    
“The Fainted Man:” Hypoperfusion Encephalopathy in a Patient With Moyamoya
Ana Filipa Santos, M.D.; João Pinho, M.D.; Carla Ferreira, M.D.; Celeste Pereira, M.D.; Manuel Ribeiro, M.D.; Jaime Rocha, M.D.
The Journal of Neuropsychiatry and Clinical Neurosciences 2014;26:E31-E33. doi:10.1176/appi.neuropsych.13020026
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Neurology Department, Hospital de Braga, Braga, Portugal

Internal Medicine Department, Hospital Santa Maria Maior Barcelos, Barcelos, Portugal

Neuroradiology Department, Hospital de Braga, Braga, Portugal

Correspondence: Ana Filipa Santos, M.D.; e-mail: filipasantos@hotmail.com

Copyright © 2014 by the American Psychiatric Association

Extract

To the Editor: Moyamoya disease is a progressive cerebral vasculopathy of unknown etiology, characterized by severe bilateral stenosis/occlusion of the main arteries of the circle of Willis, with a pattern of collateral vessels that look like a “puff of smoke” on angiography.1 It is more common in Asian populations and more prevalent in women.1 The diagnosis peaks in two age-groups: children approximately 5 years old, and adults in their mid-40s.1 It may have variable clinical presentation: ischemic stroke, transient ischemic attack, intracranial hemorrhage, headache, seizures, involuntary movements, cognitive deficits, and psychiatric changes.1 In adults, it usually presents with intracranial hemorrhage or ischemic symptoms.2 Acute cerebral ischemia occurring after intracranial bleeding has rarely been reported and is not well understood.2,3 We describe a case of hypoperfusion encephalopathy after intracerebral hemorrhage occurring as the first manifestation of moyamoya disease.

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FIGURE 1. Transverse Perfusion CT Map Shows Global Decreased Cerebral Blood Flow (CBF), Mainly in Middle Cerebral Arteries (MCA) and Anterior Cerebral Arteries (ACA) Areas

FIGURE 2. Right (A1/A3) and Left (A2/A4) Internal Carotid Angiograms (ICAs) Show Severe Stenosis of the Bilateral Distal Internal Carotid Artery, With Retrograde Filling of the Peri-Callosal Branches (*) of the ACAs and Basal Moyamoya Vessels “Puff of Smoke” Sign (arrow)
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References

Scott  RM;  Smith  ER:  Moyamoya disease and moyamoya syndrome.  N Engl J Med 2009; 360:1226–1237
[CrossRef] | [PubMed]
 
Kim  DS;  Jang  DK;  Huh  PW  et al:  Ischaemic stroke after acute intracranial haemorrhage in patients with moyamoya disease: six new cases and a short literature review.  Acta Neurochir (Wien) 2011; 153:1253–1261
[CrossRef] | [PubMed]
 
Shibamoto  K;  Aoyama  K;  Taki  H  et al:  Acute cerebral ischemia after intracranial bleeding in unilateral moyamoya disease: case report.  Neurol Med Chir (Tokyo) 2012; 52:346–349 [Tokyo]
[PubMed]
 
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