Encephaloduroarteriosynangiosis for cerebral proliferative angiopathy with cerebral ischemia

Case report

Kenichi Kono M.D. and Tomoaki Terada M.D., Ph.D.
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  • Department of Neurosurgery, Wakayama Rosai Hospital, Wakayama, Japan
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Cerebral proliferative angiopathy (CPA) is a rare clinical entity. This disorder is characterized by diffuse vascular abnormalities with intermingled normal brain parenchyma, and is differentiated from classic arteriovenous malformations. The management of CPA in patients presenting with nonhemorrhagic neurological deficits due to cerebral ischemia is challenging and controversial. The authors report a case of adult CPA with cerebral ischemia in which neurological deficits were improved after encephaloduroarteriosynangiosis (EDAS).

A 28-year-old man presented with epilepsy. Magnetic resonance imaging and angiography showed a diffuse vascular network (CPA) in the right hemisphere. Antiepileptic medications were administered. Four years after the initial onset of epilepsy, the patient's left-hand grip strength gradually decreased over the course of 1 year. The MRI studies showed no infarcts, but technetium-99m–labeled ethyl cysteinate dimer (99mTc-ECD) SPECT studies obtained with acetazolamide challenge demonstrated hypoperfusion and severely impaired cerebrovascular reactivity over the affected hemisphere. This suggested that the patient's neurological deficits were associated with cerebral ischemia. The authors performed EDAS for cerebral ischemia, and the patient's hand grip strength gradually improved after the operation. Follow-up angiography studies obtained 7 months after the operation showed profound neovascularization through the superficial temporal artery and the middle meningeal artery. A SPECT study showed slight improvement of hypoperfusion at the focal region around the right motor area, indicating clinical improvement from the operation. The authors conclude that EDAS may be a treatment option for CPA-related hypoperfusion.

Abbreviations used in this paper:AVM = arteriovenous malformation; CPA = cerebral proliferative angiopathy; ECA = external carotid artery; EDAS = encephaloduroarteriosynangiosis; ICA = internal cerebral artery; STA = superficial temporal artery; 99mTc-ECD = technetium-99m–labeled ethyl cysteinate dimer; VEGF = vascular endothelial growth factor.

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Contributor Notes

Address correspondence to: Kenichi Kono, M.D., Department of Neurosurgery, Wakayama Rosai Hospital, 93-1 Kinomoto, Wakayama 640-8505, Japan. email: vyr01450@gmail.com.

Please include this information when citing this paper: published online August 8, 2014; DOI: 10.3171/2014.7.JNS132793.

  • 1

    Dória-Netto HL, , Souza-Filho AM, , Dória-Netto RH, , Marques RA, , Oliveira DA, & Chaddad-Neto F, : Cerebral proliferative angiopathy. Arq Neuropsiquiatr 68:300302, 2010

    • Search Google Scholar
    • Export Citation
  • 2

    Ducreux D, , Petit-Lacour MC, , Marsot-Dupuch K, , Bittoun J, & Lasjaunias P: MR perfusion imaging in a case of cerebral proliferative angiopathy. Eur Radiol 12:27172722, 2002

    • Search Google Scholar
    • Export Citation
  • 3

    Eesa M, , Sharma P, & Goyal M: Cerebral proliferative angiopathy. Can J Neurol Sci 36:242243, 135:2009

  • 4

    Ellis MJ, , Armstrong D, & Dirks PB: Large vascular malformation in a child presenting with vascular steal phenomenon managed with pial synangiosis. Case report. J Neurosurg Pediatr 7:1521, 2011

    • Search Google Scholar
    • Export Citation
  • 5

    Fierstra J, , Spieth S, , Tran L, , Conklin J, , Tymianski M, & ter Brugge KG, : Severely impaired cerebrovascular reserve in patients with cerebral proliferative angiopathy. Clinical article. J Neurosurg Pediatr 8:310315, 2011

    • Search Google Scholar
    • Export Citation
  • 6

    Gold JJ, & Crawford JR: Acute hemiparesis in a child as a presenting symptom of hemispheric cerebral proliferative angiopathy. Case Rep Neurol Med 2013:920859, 2013

    • Search Google Scholar
    • Export Citation
  • 7

    Hong KS, , Lee JI, & Hong SC: Neurological picture. Cerebral proliferative angiopathy associated with haemangioma of the face and tongue. J Neurol Neurosurg Psychiatry 81:3637, 2010

    • Search Google Scholar
    • Export Citation
  • 8

    Khan A, , Rattihalli R, , Beri S, , Dickinson F, , Hussain N, & Gosalakkal J: Cerebral proliferative angiopathy: a rare form of vascular malformation. J Paediatr Child Health 49:504505, 2013

    • Search Google Scholar
    • Export Citation
  • 9

    Kimiwada T, , Hayashi T, , Shirane R, & Tominaga T: 123I-IMPSPECT in a patient with cerebral proliferative angiopathy: a case report. J Stroke Cerebrovasc Dis 22:14321435, 2013

    • Search Google Scholar
    • Export Citation
  • 10

    Lasjaunias PL, , Landrieu P, , Rodesch G, , Alvarez H, , Ozanne A, & Holmin S, : Cerebral proliferative angiopathy: clinical and angiographic description of an entity different from cerebral AVMs. Stroke 39:878885, 2008

    • Search Google Scholar
    • Export Citation
  • 11

    Maekawa H, , Tanaka M, & Hadeishi H: Fatal hemorrhage in cerebral proliferative angiopathy. Interv Neuroradiol 18:309313, 2012

  • 12

    Marks MP, & Steinberg GK: Cerebral proliferative angiopathy. J Neurointerv Surg 4:e25, 2012

  • 13

    Smith ER, & Scott RM: Surgical management of moyamoya syndrome. Skull Base 15:1526, 2005

  • 14

    Suzuki J, & Kodama N: Moyamoya disease—a review. Stroke 14:104109, 1983

  • 15

    Vargas MC, & Castillo M: Magnetic resonance perfusion imaging in proliferative cerebral angiopathy. J Comput Assist Tomogr 35:3338, 2011

    • Search Google Scholar
    • Export Citation

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