Cerebral Proliferative Angiopathy: A Rare Case with Rare Presentation and Rarer Angiographic Features

Srivastava, Trilochan; Mathur, Tarun; Mittal, Radhey Shyam; Raghavendra, B. S.; Jain, RS; Handa, Rumiko

doi:10.1016/j.ejvs.2013.05.008

Cited by 3 publications

(8 citation statements)

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“…The exact cause of DPCA is unknown; however, its diffuse character is confirmed by the presence of a trans -dural supply in remote locations (supra and infra-tentorial) suggesting an unrepressed response to cerebral sub-ischemic manifestations [ 2 ]. DPCA is characterized by the presence of a nidus of normal brain parenchyma composed of multiple arteries as an angiogenetic response to cortical ischemia [ 1 , 2 , 4 ].…”

Section: Discussionmentioning

confidence: 99%

“…Diffuse proliferative cerebral angiopathy (DPCA) is an uncommon type of cerebral vascular malformation, mostly diagnosed in young females [ 1 , 2 ]. Patients present with headaches, epileptic features and progressive neurological deficits but rarely with a hemorrhage [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

“…It is characteristically different from other cerebral arteriovenous malformations (AVMs) and can be differentiated by its peculiar imaging findings [ 2 ]. One critical finding is a nidus of normal brain parenchyma in between the abnormal vascular channels [ 1 , 2 ]. Therefore, it is crucial to diagnose it as a separate entity because unnecessary treatment of DPCA increases the risk of damage to the normal parenchyma leading to neurological deficits [ 1 , 2 ].…”

Section: Introductionmentioning

confidence: 99%

“…One critical finding is a nidus of normal brain parenchyma in between the abnormal vascular channels [ 1 , 2 ]. Therefore, it is crucial to diagnose it as a separate entity because unnecessary treatment of DPCA increases the risk of damage to the normal parenchyma leading to neurological deficits [ 1 , 2 ].…”

Section: Introductionmentioning

confidence: 99%

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Diffuse proliferative cerebral angiopathy: a case report and literature review on a very rare and misdiagnosed entity

Panthi¹,

Khanal²,

Poudel³

et al. 2022

Journal of Surgical Case Reports

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Diffuse proliferative cerebral angiopathy (DPCA) is an uncommon type of cerebral vascular malformation, mostly diagnosed in young females. It is characteristically different from other cerebral arteriovenous malformations and can be differentiated by its peculiar imaging findings. A nidus of normal brain parenchyma is present between the abnormal vascular channels. Therefore, it is crucial to diagnose it as a separate entity because unnecessary treatment of DPCA increases the risk of damage to the normal parenchyma leading to neurological deficits. Here we describe a case of a 60-year-old male who presented with severe neurological deficits and was later diagnosed with DPCA. He was managed conservatively with antiepileptics and almost completely recovered to normal within 2 weeks. A rare case of DPCA confused with other hemorrhagic disorders is discussed here. Rare cases are often overlooked. Correct diagnosis helps to prevent tragic consequences.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Diffuse proliferative cerebral angiopathy: a case report and literature review on a very rare and misdiagnosed entity

Panthi¹,

Khanal²,

Poudel³

et al. 2022

Journal of Surgical Case Reports

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“…Therefore, treatment options for CPA are limited. [ 7 8 ] Usually, symptomatic medical therapy are offered to such patient in the form of analgesics and antiepileptic drugs. The various neuro-interventions like microsurgery,[ 6 ] arterial endovascular embolization[ 9 ] and radiosurgery[ 10 ] have also been tried with variable success rates.…”

Section: Discussionmentioning

confidence: 99%

Infratentorial hemorrhagic cerebral proliferative angiopathy: A rare presentation of a rare disease

et al. 2015

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Cerebral proliferative angiopathy (CPA) is a unique and rare vascular malformation with distinct clinico-radiological features. CPA is associated with low risk of hemorrhage as compared to classical cerebral arteriovenous malformations (AVMs). Infratentorial location of diffuse nidus is also an uncommon presentation. Infratentorial hemorrhagic presentation of CPA is a rare co-occurrence. Herein, we report a case of an elderly old male, who presented with acute onset severe headache, recurrent vomiting, vertigo and swaying toward left side while walking. Cerebellar signs in the form of scanning speech, nystagmus, impaired finger-to-nose incoordination test, dysdiadochokinesia, and limb ataxia on the left side were present. Magnetic resonance imaging (MRI) brain revealed diffuse vascular network with intermingled normal brain parenchyma and hemorrhage in the left cerebellum. Digital subtraction angiography (DSA) revealed diffuse, ill-defined, nidus in left posterior fossa involving the left posterior inferior cerebellar, anterior inferior cerebellar and posterior cerebral arterial territories. There were no definite arterial feeders. DSA showed scattered “puddling” appearance of contrast material in the widespread nidus and drained into the multiple ill-defined posterior fossa veins. The MRI brain and DSA findings were consistent with the diagnosis of the CPA. Treatment in our case was limited to supportive medical therapy because selective embolization was not feasible due to nonidentifiable arterial feeders. He was asymptomatic at 1-year follow-up. This case highlights a rare entity called CPAs, which have different clinical presentations, angiographic features, treatment options and patient outcome as compared to classical cerebral AVMs.

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Clinico-radiological features of cerebral proliferative angiopathy: A large case series from India

Srivastava

Gandhi

Kumar³

2022

Neuroradiol J

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Background Based on angiographic features, a rare subgroup, different from classical brain AVMs, called cerebral proliferative angiopathy (CPA) was suggested by Lasjaunias et al. in 1989. There are only few reports of CPA described from Asia. Liu et al. has described 5 cases of CPA from China. The objective of study was to evaluate the clinico-radiological features of patients with CPA in our cohort and to find out the differences between our study and the study by Lasjaunias and Liu et al. Methods We retrospectively analysed cases of CPA from March 2012 to April 2020. All radiological images of the patients with AVMs were studied and findings suggestive of CPA were identified. We found 10 patients with CPA out of 272 cases of AVM (10/272) and described the various demographic and clinico-radiological features. Results The age of the patients with CPA in our series varied from 14 to 53 years and the mean age was 28.6 years. There was male preponderance (60%) with male to female ratio of 3:2. The most common presentation was seizure (70%). Intracranial haemorrhage was present in five patients. One patient had cerebral infarction. Conclusions There were substantial differences in clinico-radiological profile of patients with CPA in our cohort as compared to the study by Lasjaunias et al. and Liu et al. in form of male dominance, higher rate of ICH and seizure. In our knowledge, this is the second largest series of CPA worldwide and the first largest series in Asia.

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Cerebral Proliferative Angiopathy: A Rare Case with Rare Presentation and Rarer Angiographic Features

Cited by 3 publications

References 0 publications

Diffuse proliferative cerebral angiopathy: a case report and literature review on a very rare and misdiagnosed entity

Diffuse proliferative cerebral angiopathy: a case report and literature review on a very rare and misdiagnosed entity

Infratentorial hemorrhagic cerebral proliferative angiopathy: A rare presentation of a rare disease

Clinico-radiological features of cerebral proliferative angiopathy: A large case series from India

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