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E-045 cerebral vascular findings in papa syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm
  1. K Khatibi1,
  2. J Heit2,
  3. N Telischak2,
  4. J Elbers1,
  5. H Do2
  1. 1Neurology, Stanford University, Stanford, CA, USA
  2. 2Interventional Neuroradiology, Stanford University, Stanford, CA, USA


PAPA (Pyogenic Arthritis, Pyoderma ganngrenosum, and Acne) syndrome is a rare autosomal-dominant auto-inflammatory disorder that affects joints and the skin and manifests as pyogenic arthritis, pyoderma gangrenosum and acne. We describe the first report of cerebral arterial vasculitis with rupture of an associated dissecting aneurysm in PAPA syndrome.

A patient with PAPA syndrome developed thunderclap headaches, and non-invasive imaging demonstrated diffuse cerebral arterial irregularity that was consistent with a cerebral arterial vasculitis or vasculopathy. The patient was treated with steroids, but there was no improvement in the patient’s symptoms or cerebral arterial irregularity.

The patient then developed subarachnoid hemorrhage secondary to rupture of a dissecting aneurysm of the right posterior cerebral artery. This aneurysm was treated by endovascular coil sacrifice of the dissecting aneurysm, and the patient made an excellent recovery.

Disclosures K. Khatibi: None. J. Heit: None. N. Telischak: None. J. Elbers: None. H. Do: None.

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