“The Fainted Man:” Hypoperfusion Encephalopathy in a Patient With Moyamoya

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Back to table of contents Previous article Next article LettersFull Access“The Fainted Man:” Hypoperfusion Encephalopathy in a Patient With MoyamoyaAna Filipa Santos, M.D., João Pinho, M.D., Carla Ferreira, M.D., Celeste Pereira, M.D., Manuel Ribeiro, M.D., and Jaime Rocha, M.D.Ana Filipa SantosSearch for more papers by this author, M.D., João PinhoSearch for more papers by this author, M.D., Carla FerreiraSearch for more papers by this author, M.D., Celeste PereiraSearch for more papers by this author, M.D., Manuel RibeiroSearch for more papers by this author, M.D., and Jaime RochaSearch for more papers by this author, M.D.Published Online:1 Jan 2014https://doi.org/10.1176/appi.neuropsych.13020026AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail 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.A 77-year-old male patient, with a long-standing history of uncontrolled hypertension, was admitted to our stroke unit upon being found unconscious. His blood pressure was 174/100 mmHg and Glasgow Coma Scale rated 3 (no eye opening, no verbal response, no motor response), with reactive pupils and right extensor plantar reflex. Computed tomographic (CT) scan showed left basal ganglia hemorrhage, extending into the ventricles and subarachnoid space. Antihypertensive and hyperosmotic therapy was started, and the initial evolution was favorable, with recovery of consciousness, confused verbal response, and right hemiparesis Grade 3 (MRC). By Day 14, multifocal myoclonus appeared, with decreased arousal and ensuing severe encephalopathy. Follow-up CT revealed hemorrhage reabsorption and no hydrocephaly. Laboratory studies were unrevealing. An electroencephalogram showed only slow background activity (5–6 Hz). Infections, metabolic, and toxic causes were also excluded. Brain diffusion-weighted imaging (DWI) MRI was negative for acute ischemic lesions. At this point, hypoperfusion encephalopathy was suspected and confirmed by perfusion CT (Figure 1). CT angiography disclosed stenosis of the distal intracranial internal carotid artery (ICA) bilaterally, extending to the proximal anterior (ACA) and middle cerebral (MCA) arteries. Moyamoya disease was diagnosed and was classified as Grade IV (Suzuki Grading System) by conventional angiography (Figure 2). Because of his clinical condition, revascularization was not considered.FIGURE 1. Transverse Perfusion CT Map Shows Global Decreased Cerebral Blood Flow (CBF), Mainly in Middle Cerebral Arteries (MCA) and Anterior Cerebral Arteries (ACA) AreasFIGURE 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)We report this case because of the rarity of moyamoya diagnosis in elderly persons and because of the atypical presentation. Our patient seemed to be permanently “fainted;” he had global hypoperfusion, but no real ischemic changes. The initial manifestation was interpreted as a hypertensive intracerebral hemorrhage. Later, when we detected hypoperfusion encephalopathy, Moyamoya was diagnosed. Vasospasm, in association with both subarachnoid and intraventricular hemorrhage, and hypotension and dehydration, resulting from the administration of hyperosmotic agents to control hemorrhage-related increased intracranial pressure, may have caused hemodynamic compromise, being possible contributing factors to the hypoperfusion encephalopathy in our patient. Other factors, such as hypocapnia and shrinkage of the moyamoya vessels, have also been proposed as mechanisms in acute cerebral ischemia occurring after intracranial bleeding.2,3 The initial clinical presentation of hemorrhage and the presence of severe bilateral ICA stenosis affecting cerebral perfusion are predictors of very poor outcome in our patient.Neurology Department, Hospital de Braga, Braga, PortugalInternal Medicine Department, Hospital Santa Maria Maior Barcelos, Barcelos, PortugalNeuroradiology Department, Hospital de Braga, Braga, PortugalCorrespondence: Ana Filipa Santos, M.D.; e-mail: [email protected]comReferences1 Scott RM, Smith ER: Moyamoya disease and moyamoya syndrome. N Engl J Med 2009; 360:1226–1237Crossref, Medline, Google Scholar2 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; 153:1253–1261Crossref, Medline, Google Scholar3 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]Crossref, Medline, Google Scholar FiguresReferencesCited byDetailsCited ByNone Volume 26Issue 1 Winter 2014Pages E31-E33 Metrics PDF download History Published online 1 January 2014 Published in print 1 January 2014