Diffuse Brain Hypoperfusion in Advanced Leukoencephalopathy with Calcifications and Cysts

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• 作者：Emanuele Bartolini ; MD^* ; ^&dagger ; ; ^{emanuele.bartolini@meyer.it" class="auth_mail" title="E-mail the corresponding author} ; Andrea Bianchi ; MD^&Dagger ; ; ^§ ; ; Ilaria Bartolomei ; MD ; PhD^‖ ; Alessandra Vella ; MD ; PhD^¶ ; ; Lapo Sali ; MD ; PhD^§ ; ; Antonio Ciccarone^# ; Fabrizio Salvi ; MD ; PhD^‖ ; Mario Mascalchi ; MD ; PhD^&Dagger ; ; ^§ ;
• 关键词：White matter ; perfusion MRI ; regional CBF ; arterial spin labeling ; 3-T MRI ; perfusion ; neuroimaging
• 刊名：Journal of Stroke and Cerebrovascular Diseases
• 出版年：2016
• 出版时间：August 2016
• 年：2016
• 卷：25
• 期：8
• 页码：e111-e113
• 全文大小：406 K

文摘

Leukoencephalopathy with calcifications and cysts (LCC) is an uncommon condition of unknown etiology occurring in children and adults. Pathological findings include obliterative hyalinosis of the small vessels, myelin loss, intense gliosis, Rosenthal fiber formation, microcalcifications, and hemosiderin deposits. Herein we report a 55-year-old man with LCC documented 10 years ago, in whom we examined brain perfusion by pseudocontinuous arterial spin labeling technique. We demonstrated diffused hypoperfusion of the affected white matter (WM) and of the subcortical gray matter (GM) and cortical GM in the patient in comparison to a group of healthy control subjects, using both qualitative evaluation and region of interest analysis. WM and subcortical GM hypoperfusion reflects the known distribution of LCC microangiopathy. We speculate that cortical hypoperfusion may be related to cerebral atrophy or may reflect deafferentation secondary to severe leukoencephalopathy, and may possibly contribute to severe motor and cognitive impairment. Further studies addressing cerebral blood flow in LCC are necessary.