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

Kumar S, Sharma M, Srivastava T, Sinha VD - Asian J Neurosurg (2015 Jul-Sep)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India.

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.

No MeSH data available.


Related in: MedlinePlus

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
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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

Mentions: Hemogram, coagulation profile (platelets count, bleeding time, coagulation time, prothrombin time, INR), serum biochemistry including thyroid function tests were normal. Noncontrast computed tomography (CT) scan showed intraparenchymal hemorrhage in the left cerebellar hemisphere [Figure 1]. MRI of brain demonstrated 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 was present in posterior aspects of the left cerebellar hemisphere [Figure 2a and b]. CT angiography of brain vessels showed diffuse network of densely enhancing vascular channels in the left posterior fossa. There were no dominant arterial feeders [Figure 3a and b]. Cerebral digital subtraction angiography (DSA) revealed 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 [Figure 4a–c]. MRI brain and DSA findings were consistent with the diagnosis of the CPA. The patient was managed with symptomatic medical therapy. He was asymptomatic at 1-year follow-up.


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

Kumar S, Sharma M, Srivastava T, Sinha VD - Asian J Neurosurg (2015 Jul-Sep)

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
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4553743&req=5

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
Mentions: Hemogram, coagulation profile (platelets count, bleeding time, coagulation time, prothrombin time, INR), serum biochemistry including thyroid function tests were normal. Noncontrast computed tomography (CT) scan showed intraparenchymal hemorrhage in the left cerebellar hemisphere [Figure 1]. MRI of brain demonstrated 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 was present in posterior aspects of the left cerebellar hemisphere [Figure 2a and b]. CT angiography of brain vessels showed diffuse network of densely enhancing vascular channels in the left posterior fossa. There were no dominant arterial feeders [Figure 3a and b]. Cerebral digital subtraction angiography (DSA) revealed 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 [Figure 4a–c]. MRI brain and DSA findings were consistent with the diagnosis of the CPA. The patient was managed with symptomatic medical therapy. He was asymptomatic at 1-year follow-up.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India.

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.

No MeSH data available.


Related in: MedlinePlus