Cerebral proliferative angiopathy

Dória-Netto, Hugo Leonardo; Souza-Filho, Antônio Marcos de; Dória-Netto, Ricardo Henrique; Marques, Rômulo Alberto Silva; Oliveira, Denise Aparecida de; Chaddad-Neto, Feres; Campos, Christiane Monteiro de Siqueira

doi:10.1590/s0004-282x2010000200027

Cited by 18 publications

(20 citation statements)

References 10 publications

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“…There have been several case reports on patients with CPA, [1][2][3][4][5][6][7][8][9]11,12,15 and one series of 49 patients with this disorder. 10 In that study of 49 patients (mean age 22 years), 45% presented with seizures, 41% with severe and often disabling headaches, 12% with hemorrhage, and 16% with nonhemorrhagic neurological deficits.…”

Section: Discussionmentioning

confidence: 99%

Encephaloduroarteriosynangiosis for cerebral proliferative angiopathy with cerebral ischemia

Kono¹,

Terada²

2014

JNS

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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 ( 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.

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Section: Discussionmentioning

confidence: 99%

Encephaloduroarteriosynangiosis for cerebral proliferative angiopathy with cerebral ischemia

Kono¹,

Terada²

2014

JNS

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“…There have been relatively few case reports of CPA [2–7]. CPA is considered to have a low risk for hemorrhage at initial presentation, although a case of fatal hemorrhage has been described in an elderly woman [6].…”

Section: Discussionmentioning

confidence: 99%

Acute Hemiparesis in a Child as a Presenting Symptom of Hemispheric Cerebral Proliferative Angiopathy

Gold

Crawford

2013

Case Reports in Neurological Medicine

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A 9-year-old girl with a several-month history of unilateral intermittent headaches presented to the hospital with worsening headaches and unsteadiness. Neurologic exam was positive for a mild right hemiparesis and right homonymous hemianopsia. Noncontrast computed tomography revealed an engorged sagittal and straight sinus with prominent cortical veins concerning an arteriovenous malformation and the patient was admitted to the pediatric intensive care unit. Computed tomography angiogram demonstrated a left hemispheric vascular malformation, without evidence of dural arteriovenous fistula on conventional angiogram consistent with a diagnosis of cerebral proliferative angiopathy. There was no evidence of infarct on magnetic resonance imaging, and the patient's symptoms were completely resolved within 24 hours. Cerebral proliferative angiopathy is a rare but important vascular malformation distinct from classic arteriovenous malformations that may present with stroke-like symptoms in childhood.

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“…A patient presents with characteristics such as large dimensions, multiple feeding vessels, small veins, and normal brain tissue warehouse. [1][2][3] It is classified as a disease itself within the group of arteriovenous malformations (AVMs). 4 Alternatives for curative treatment imply significant neurologic deficits and accept more options aimed at easing the symptoms.…”

Section: Introductionmentioning

confidence: 99%

Cerebral Proliferative Angiopathy—Description of a Rare Clinical Entity

et al. 2015

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The cerebral proliferative angiopathy (CPA) is an uncommon vascular malformation of the brain, representing approximately 3.4% of arteriovenous malformation (AVM) cases, with complex morphology and poorly understood origin. Thus, we present the case of a woman patient aged 27 years, who performed magnetic resonance imaging (MRI) of the brain because of a headache. Extensive malformation was identified vascular right frontoparietal, with the presence of the brain tissue mixed with the vessel. CPA identified multiple feeder arteries, with the absence of nidus and slowing the flow into the venous system, confirming that it was CPA. During evaluation at another hospital she underwent partial embolization of the lesion, without success. Currently, the patient is progressing with left hemiparesis. CPA has distinct characteristics, such as large dimensions, presence of functional brain tissue intermingled with the vessels, no nidus, multiple nourishing vessels, and small veins. It does not present well-defined nidus or flow-related aneurysms. These characteristics classify it as a disease itself within the group of AVMs. This case clinically presents with seizures, refractory headaches, focal neurologic deficits, and low risk of bleeding. Alternatives for curative treatment imply significant neurologic deficits and the most accepted options aimed at easing the symptoms and the partial embolization alternative that is best associated with risks and benefits. ResumoA angiopatia cerebral progressiva (ACP) é uma malformação vascular encefálica incomum, representando cerca de 3,4% dos casos de malformações arteriovenosas (MAV's), com morfologia complexa e origem pouco compreendida. Assim, apresentamos o caso de uma paciente de 27 anos, que realizou RNM de crânio para investigação etiológica de cefaleia, tendo sido identificado extensa malformação vascular frontoparietal direita, com a presença de tecido cerebral entremeado aos vasos. Exame de

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Cerebral proliferative angiopathy

Cited by 18 publications

References 10 publications

Encephaloduroarteriosynangiosis for cerebral proliferative angiopathy with cerebral ischemia

Encephaloduroarteriosynangiosis for cerebral proliferative angiopathy with cerebral ischemia

Acute Hemiparesis in a Child as a Presenting Symptom of Hemispheric Cerebral Proliferative Angiopathy

Cerebral Proliferative Angiopathy—Description of a Rare Clinical Entity

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