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

Abstract

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.

Keywords: Cerebral proliferative angiopathy; arteriovenous malformations; infratentorial nidus; intracerebral hemorrhage.

Figure 1

Noncontrast computed tomography scan showing intraparenchymal hemorrhage in the left cerebellar hemisphere

Figure 2

T2-weighted magnetic resonance imaging of brain (a) axial, (b) sagittal showing a diffuse network of vascular channels and dilated veins with intermingled normal brain tissues between different vascular territories on the left cerebellar hemisphere, which extend to the left para-pontine, quadrigeminal and arachnoid cistern. A small intraparenchymal hemorrhage is seen in posterior aspects of the left cerebellar hemisphere

Figure 3

Computed tomography angiography of brain vessels showing diffuse network of densely enhancing vascular channels in the left posterior fossa. There was no dominant arterial feeders (a) axial, (b) sagittal

Figure 4

(a-c) Cerebral digital subtraction angiography reveal large, ill-defined, diffuse, nidus in the left posterior fossa. Diffuse nidus involved the left posterior inferior cerebellar, anterior inferior cerebellar and posterior cerebral arterial territories. There was no dominant arterial feeder identified. It had scattered “puddling” of contrast material in the widespread nidus and drained into the ill-defined, multiple posterior fossa veins to straight and left transverse sinus