- Email: [email protected]
Cerebral Proliferative Angiopathy
Accepted Manuscript Visual Diagnosis: Cerebral Proliferative Angiopathy Victoria Karian, RN, MSN, CPNP, Jonathan Rabner, MA, Alyssa Lebel, MD PII:
S0887-8994(16)30582-3
DOI:
10.1016/j.pediatrneurol.2016.09.012
Reference:
PNU 8996
To appear in:
Pediatric Neurology
Received Date: 5 August 2016 Revised Date:
13 September 2016
Accepted Date: 17 September 2016
Please cite this article as: Karian V, Rabner J, Lebel A, Visual Diagnosis: Cerebral Proliferative Angiopathy, Pediatric Neurology (2016), doi: 10.1016/j.pediatrneurol.2016.09.012. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Visual Diagnosis: Cerebral Proliferative Angiopathy
Jonathan Rabner, MA1 Alyssa Lebel, MD1,2
SC
1
RI PT
Victoria Karian, RN, MSN, CPNP1
Division of Pain Medicine, Department of Anesthesiology, Perioperative and Pain Medicine,
M AN U
Boston Children’s Hospital, Boston, MA 2
Department of Anesthesia, Harvard Medical School, Boston, MA
Correspondence to: Victoria Karian, RN, MSN, CPNP
Tel: (781) 216-1960 Fax: (781) 216-2540.
TE D
Pediatric Headache Program, Boston Children’s Hospital, 9 Hope Ave, Waltham MA 02453
AC C
Running head: CPA
EP
email: [email protected]
Word Count: 342 , not including figure legend
ACCEPTED MANUSCRIPT
Cerebral Proliferative Angiopathy
RI PT
Seven year-old boy presented with severe, right-sided headache pain, with left-sided progressive weakness and mental status changes, initially diagnosed with arteriovenous malformation (AVM). MRI showed large right parietal occipital vascular malformation. In October 2014, he had episodic left-sided weakness and numbness, increasing in frequency and duration. Cerebral angiography in December 2014 revealed cerebral proliferative angiopathy (CPA), with focal arteriovenous shunting. He was started on aspirin and followed for progression of symptoms.
M AN U
SC
CPA is a vascular anomaly of the brain, which differs from a classic brain AVM in blood vessel architecture, natural history, clinical presentation, and treatment [1,2,3,4]. Clinical presentation is characterized by seizures (45%), headaches (41%) and progressive neurologic deficits (12%), with strokelike symptoms and transient ischemic attacks. Imaging shows a diffuse network of densely enhancing vascular spaces with intermingled normal and abnormal brain parenchyma, often involving an entire hemisphere or multiple lobes of the brain [1,2].The pathology is characterized by cortical ischemic leading to endothelial proliferation and angiogenesis [2,3]. Treatment options are conservative and symptom-based with anti-epileptic and headache medications. Interventional treatments, such as targeted endovascular embolization, surgical calvarial burrhole formation, and encephalo-duro-arterio-synangiosis (EDAS) revascularization surgery have been used with some benefit [1,4].
TE D
Careful monitoring with developmental screening and neuropsychological evaluation can identify any early or progressive learning deficits as a result of tissue ischemia during pediatric brain development.
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1. Lasjaunais, PL, Landrieu P, Rodesh G, Alvarez H, Ozanne A, Holmin S, Zhao WY, Geibprasert S, Ducreux D, Krings T. Cerebral Proliferative Angiopathy: Clinical and angiographic description of an entity different from cerebral AVMs. Stroke, 2008;38:878-885. 2. Gold JJ, Crawford JR. Acute hemiparesis in a child as presenting symptoms of hemispheric cerebral proliferative angiopathy. Case reports in Neurological Medicine, 2013, article ID 920859 3. Fierstra J, Spieth S, Tran L, Conklin J, Tymianski M, Rer Brugge KG, Fisher J, Mikulis D, Krings T. Severely impaired cerebrovascular reserve in patients with cerebral proliferative angiopathy. Journal of Neurosurgical Pediatrics, 2011;8:310-315. 4. Liu P, Ly X, Ly M, Li Y. Cerebral proliferative angiopathy: Clinical angiographic features and literature review. Interventional Neuroradiology, 2016;22:101-7.
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S0887-8994(16)30582-3
DOI:
10.1016/j.pediatrneurol.2016.09.012
Reference:
PNU 8996
To appear in:
Pediatric Neurology
Received Date: 5 August 2016 Revised Date:
13 September 2016
Accepted Date: 17 September 2016
Please cite this article as: Karian V, Rabner J, Lebel A, Visual Diagnosis: Cerebral Proliferative Angiopathy, Pediatric Neurology (2016), doi: 10.1016/j.pediatrneurol.2016.09.012. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Visual Diagnosis: Cerebral Proliferative Angiopathy
Jonathan Rabner, MA1 Alyssa Lebel, MD1,2
SC
1
RI PT
Victoria Karian, RN, MSN, CPNP1
Division of Pain Medicine, Department of Anesthesiology, Perioperative and Pain Medicine,
M AN U
Boston Children’s Hospital, Boston, MA 2
Department of Anesthesia, Harvard Medical School, Boston, MA
Correspondence to: Victoria Karian, RN, MSN, CPNP
Tel: (781) 216-1960 Fax: (781) 216-2540.
TE D
Pediatric Headache Program, Boston Children’s Hospital, 9 Hope Ave, Waltham MA 02453
AC C
Running head: CPA
EP
email: [email protected]
Word Count: 342 , not including figure legend
ACCEPTED MANUSCRIPT
Cerebral Proliferative Angiopathy
RI PT
Seven year-old boy presented with severe, right-sided headache pain, with left-sided progressive weakness and mental status changes, initially diagnosed with arteriovenous malformation (AVM). MRI showed large right parietal occipital vascular malformation. In October 2014, he had episodic left-sided weakness and numbness, increasing in frequency and duration. Cerebral angiography in December 2014 revealed cerebral proliferative angiopathy (CPA), with focal arteriovenous shunting. He was started on aspirin and followed for progression of symptoms.
M AN U
SC
CPA is a vascular anomaly of the brain, which differs from a classic brain AVM in blood vessel architecture, natural history, clinical presentation, and treatment [1,2,3,4]. Clinical presentation is characterized by seizures (45%), headaches (41%) and progressive neurologic deficits (12%), with strokelike symptoms and transient ischemic attacks. Imaging shows a diffuse network of densely enhancing vascular spaces with intermingled normal and abnormal brain parenchyma, often involving an entire hemisphere or multiple lobes of the brain [1,2].The pathology is characterized by cortical ischemic leading to endothelial proliferation and angiogenesis [2,3]. Treatment options are conservative and symptom-based with anti-epileptic and headache medications. Interventional treatments, such as targeted endovascular embolization, surgical calvarial burrhole formation, and encephalo-duro-arterio-synangiosis (EDAS) revascularization surgery have been used with some benefit [1,4].
TE D
Careful monitoring with developmental screening and neuropsychological evaluation can identify any early or progressive learning deficits as a result of tissue ischemia during pediatric brain development.
AC C
EP
1. Lasjaunais, PL, Landrieu P, Rodesh G, Alvarez H, Ozanne A, Holmin S, Zhao WY, Geibprasert S, Ducreux D, Krings T. Cerebral Proliferative Angiopathy: Clinical and angiographic description of an entity different from cerebral AVMs. Stroke, 2008;38:878-885. 2. Gold JJ, Crawford JR. Acute hemiparesis in a child as presenting symptoms of hemispheric cerebral proliferative angiopathy. Case reports in Neurological Medicine, 2013, article ID 920859 3. Fierstra J, Spieth S, Tran L, Conklin J, Tymianski M, Rer Brugge KG, Fisher J, Mikulis D, Krings T. Severely impaired cerebrovascular reserve in patients with cerebral proliferative angiopathy. Journal of Neurosurgical Pediatrics, 2011;8:310-315. 4. Liu P, Ly X, Ly M, Li Y. Cerebral proliferative angiopathy: Clinical angiographic features and literature review. Interventional Neuroradiology, 2016;22:101-7.
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