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Christina Huang, Elias Rizk, Mark Iantosca, Andrea L. Zaenglein, Klaus F. Helm, Arabinda K. Choudhary, and Mark S. Dias

Multifocal lymphangioendotheliomatosis with thrombocytopenia is a congenital vascular disorder characterized by the presence of multiple cutaneous macules, papules, and plaques. The number and size of these lesions vary, but hundreds can be seen ranging in size from a few millimeters to several centimeters. Similar lesions can also be found in other organs, including the gastrointestinal tract most commonly, as well as the lung, synovium, muscle, bone, bone marrow, kidney, liver, spleen, and brain. The gastrointestinal lesions can be quite numerous and involve the

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Vijay Letchuman, Leonel Ampie, Panagiotis Mastorakos, Daniel M. S. Raper, Ryan T. Kellogg, and Min S. Park

Model Development As patients with MMD exhibit features of chronic cerebral hypoperfusion (CCH), one of the most common methods of creating an experimental MMD animal model is through occlusion or stenosis of the common carotid arteries (CCAs) and ICAs at various points. 13 , 23–26 Similar to other cognitive and vascular disorders, cerebral perfusion in MMD is diminished, and a variety of techniques have been developed both to recreate these hypoperfused states in animal models and to grade and treat patients with CCH. Given the underlying pathophysiological

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Kenichi Amagasaki, Hiromichi Yamazaki, Kimie Ohmori, Hidehito Koizumi, Kazuhiro Hashizume, and Nobuo Sasaguchi

lymphomatosis that demonstrated an unusual association with venous stenosis manifesting as multifocal vascular disease and rapidly progressive dementia. This disease is one of the diagnostic possibilities that must be considered in any patient with a multifocal vascular disorder and rapidly progressive dementia. Case Report This 55-year-old man presented at another neurosurgical institution with paraparesis, sensory disturbance of the lower extremities, and dysuria. Neurological examination revealed paraparesis with hypoactive reflexes of the lower extremities and sensory

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Marian Gaballah, Phillip B. Storm, Deborah Rabinowitz, Rebecca N. Ichord, Robert W. Hurst, Ganesh Krishnamurthy, Marc S. Keller, Adeka McIntosh, and Anne Marie Cahill

during microsurgical removal of arteriovenous malformations in children. Clinical article . J Neurosurg Pediatr 6 : 435 – 443 , 2010 10.3171/2010.8.PEDS10134 13 Folkman J , Klagsbrun M : Angiogenic factors . Science 235 : 442 – 447 , 1987 10.1126/science.2432664 14 Ghosh S , Levy ML , Stanley P , Nelson M , Giannotta SL , McComb JG : Intraoperative angiography in the management of pediatric vascular disorders . Pediatr Neurosurg 30 : 16 – 22 , 1999 10.1159/000028754 15 Grossart KW , Turner JW : Operative angiography in

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Päivi Koroknay-Pál, Hanna Lehto, Mika Niemelä, Riku Kivisaari, and Juha Hernesniemi

classic connective tissue disorders were not diagnosed in our series led us to suspect these patients had some vascular disorders that would predispose them in the future to cardiac diseases or other cerebrovascular events. However, this was not the case, with the exception of de novo aneurysms. The incidence of hypertension, hypercholesterolemia, stroke, and other conditions was no higher than in an age-matched Finnish population. Also, there was no increase in the incidence of autoimmune disorders. The mean proportion of smokers in Finland has been reported to be 22

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Arya N. Bagherpour, Gustavo J. Rodriguez, Chetan Moorthy, Todd T. Trier, and Alberto Maud

. Workup and Treatment Considerations The yield of spinal axis imaging in nonaneurysmal SAH is low, particularly in perimesencephalic SAH. 3 However, imaging of the spine should be considered in certain patients. At our institution, we perform MRI of the spine particularly when cerebral digital subtraction angiography (DSA) is nonrevealing (especially after 2 or more consecutive negative cerebral DSA studies) and in the following cases: posterior fossa–predominant SAH, certain predisposing vascular disorders, and pediatric patients because AVMs are more commonly a

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Andrew S. Jack, Michael M. Chow, Loretta Fiorillo, Thea Chibuk, Jerome Y. Yager, and Vivek Mehta

disorder and may represent the most common neurocutaneous vascular disorder. 19 , 21 , 22 Several large registries and cohort studies have been reported outlining the prevalence and incidence of the disease’s different abnormalities. However, little has been reported regarding the natural history of the syndrome. More specifically, few have reported on the natural history of the intracranial arteri-opathy, which affects approximately 90% of PHACE patients. 13 Although rates of intracranial structural and cerebrovascular anomalies vary among PHACE patients, these

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Pankaj K. Agarwalla, Christopher J. Stapleton, Michael T. Phillips, Brian P. Walcott, Andrew S. Venteicher, and Christopher S. Ogilvy

natural history. Finally, associated conditions were studied in our patient population, and outside of the risk factors documented in Table 2 , there were no associated conditions such as anemia, radiation exposure, vasculitis, hereditary phakomatoses, associated vascular disorders, trauma, or a positive family history. One patient had Graves' disease, which has been associated with MMS. 13 Interestingly, there were a number of patients who had headache disorders (32%) or psychiatric conditions (24%) associated with moyamoya. Discussion Moyamoya disease and

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Ayako Ro and Norimasa Kageyama

, Masawa N , Suzuki K , : A case of segmental mediolytic arteriopathy involving both intracranial and intraabdominal arteries . Pathol Res Pract 198 : 493 – 497 , 2002 10.1078/0344-0338-00290 14 Slavin RE , Inada K : Segmental arterial mediolysis with accompanying venous angiopathy: a clinical pathologic review, report of 3 new cases, and comments on the role of endothelin-1 in its pathogenesis . Int J Surg Pathol 15 : 121 – 134 , 2007 10.1177/1066896906297684 15 Slavin RE , Yaeger MJ : Segmental arterial mediolysis—an iatrogenic vascular