Encephaloduroarteriosynangiosis for cerebral proliferative angiopathy with cerebral ischemia

Abstract

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 ( 99m Tc-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. (http://thejns.org/doi/abs/10.3171/2014.7.JNS132793) Key WorDs • arteriovenous malformation • cerebral ischemia • cerebral proliferative angiopathy • encephaloduroarteriosynangiosis • epilepsy • vascular disordersAbbreviations 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; 99m Tc-ECD = technetium-99m-labeled ethyl cysteinate dimer; VEGF = vascular endothelial growth factor.

Encephaloduromyosynangiosis (EDMS) on a Pediatric Patient with Cerebral Proliferative Angiopathy

“…Clinical symptoms of CPA are seizures (45% patients), headaches (41%), focal neurological deficits (FNDs) not because of hemorrhage (16%), and cerebral hemorrhages (12%). Although it is rare that the onset of CPA is hemorrhagic, the chance of re-bleeding may be higher than in patients with typical AVMs [1,2] .…”

“…Furthermore, increased regional cerebral blood flow perfusion may relieve clinical symptoms and improve prognosis of patient with CPA. Surgical procedures for treating moyamoya disease which can increase regional cortex's cerebral blood flow perfusion may benefit patients with CPA, Kono et al [2]. reported a case of adult CPA with cerebral ischemia in which neurological deficits were improved after encephaloduroarteriosynangiosis (EDAS).…”

Cerebral proliferative angiopathy: a review / Angiopatia proliferativa cerebral: uma revisão

“…The clinical course is believed to be less aggressive than true AVMs. Symptoms usually relapse and remit over a few years, and might culminate with a more severe disturbance that motivates an emergency investigation or progresses to significantly impair the patients's quality of life (4,8,18) . However, Marks et al, 2011 (4) , reported a more rapid progression.…”

“…Perfusion images may play a role in the diagnosis and follow-up of these patients, to assess the extension of ischemic compromise and the efficacy of treatments (3,20) . Several methods were used across case reports and series, such as perfusion-weighted MRI (PW-MRI) (3,6,15,(20)(21)(22) , 123I-IMP-SPECT (21) , 99mTc-ECD-SPECT (18) , Positron Emission Tomography (PET) (23) , and 11C-METH PET (24) .…”

Cerebral Proliferative Angiopathy in a Child

“…Patients with CPA, typically young females, usually present with headaches, seizures, and progressive neurological deficits due to ischemia with vascular steal [4,5] . These patients have lower risk of hemorrhagic events than those with brain AVMs [1,4,6] .…”