Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm

Kasra Khatibi; Jeremy J Heit; Nicholas A Telischak; Jorina M Elbers; Huy M Do

doi:10.1136/bcr-2015-011753

Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm

BMJ Case Rep. 2015 Jun 24:2015:bcr2015011753. doi: 10.1136/bcr-2015-011753.

Authors

Kasra Khatibi¹, Jeremy J Heit², Nicholas A Telischak², Jorina M Elbers¹, Huy M Do³

Affiliations

¹ Department of Neurology, Stanford University, Stanford, California, USA.
² Department of Radiology, Stanford University, Stanford, California, USA.
³ Departments of Radiology and Neurosurgery, Stanford University, Stanford, California, USA.

Abstract

A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit.

Keywords: Aneurysm; Angiography; Brain; Coil; Vasculitis.

2015 BMJ Publishing Group Ltd.

Publication types

Case Reports

MeSH terms

Acne Vulgaris / complications
Acne Vulgaris / pathology*
Aneurysm, Ruptured / pathology*
Aneurysm, Ruptured / surgery
Anti-Bacterial Agents / therapeutic use
Aortic Dissection / diagnostic imaging
Aortic Dissection / pathology*
Arthritis, Infectious / complications
Arthritis, Infectious / pathology*
Cefazolin / therapeutic use
Cerebral Angiography
Humans
Intracranial Aneurysm / diagnostic imaging
Intracranial Aneurysm / pathology*
Methicillin-Resistant Staphylococcus aureus / isolation & purification*
Methylprednisolone / therapeutic use
Pyoderma Gangrenosum / complications
Pyoderma Gangrenosum / pathology*
Staphylococcal Infections / complications
Staphylococcal Infections / diagnostic imaging
Staphylococcal Infections / pathology*
Treatment Outcome
Vasculitis / etiology*
Vasculitis / microbiology

Substances

Anti-Bacterial Agents
Cefazolin
Methylprednisolone

Supplementary concepts

Pyogenic arthritis, pyoderma gangrenosum, and acne