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.

Recurrent periventricular hemorrhage in cerebral proliferative angiopathy: Case report

“…1 On the other hand, hemorrhage is rare and only 10 cases have been reported. 1,[4][5][6][7] In the largest case series including 49 patients, 6 had hemorrhage, 4 experienced recurrence, and 1 died of a rebleeding. 1 Unfortunately, the authors described none of these cases in detail.…”

Cerebral revascularization in cerebral proliferative angiopathy: a systematic review

“…2,9,[19][20][21] The remaining 4 studies reported favorable clinical outcomes, including 2 patients treated with antiepileptic drugs and 1 patient receiving acetazolamide. 13,16,17,28 Two of 4 studies describing radiological outcomes following conservative management reported adverse radiological progression, including increased size of the malformation and progressive angiogenesis. 19,21 The remaining studies reported no progressive changes; 9 however, 1 patient suffered a fatal intracranial hemorrhage after a 9-year period of radiographic quiescence.…”

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

“…Other findings are the paucity of draining vessels compared with the nidus size, proliferation of pial vessels, and presence of flow void -indicating vigorously flowing blood. The absence of acute infarcts or intracranial hemorrhages also supports this diagnosis, even though they can be present (2,7,10,14,19) .…”

“…A diffuse network of densely enhancing vascular spaces with intermingled normal brain parenchyma might be considered an essential finding across series of cases and case reports to emit a diagnosis of CPA (Figure 1). Lesions are usually large and profound, compromising multiple lobes, whole hemispheres or even both sides of the brain, with extension to basal ganglia and thalamus in 85% of cases, and with possible infratentorial impairment (2,7,10,14,19) .…”