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Images in Neurology |

Multiple Intra-abdominal and Cerebral Aneurysms Several Years After Resection of Bilateral Atrial Myxomas in a Patient With Carney Syndrome

Jean-Luc Blanc, MD; Gérard Guillet, MD; Pierre Vandermarq, MD; Jean-Philippe Neau, MD
Arch Neurol. 2008;65(11):1536-1537. doi:10.1001/archneur.65.11.1536.
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A 38-year-old man had a 2-day history of headache, with intermittent blurred vision followed by an acute episode of aphasia with bilateral upper numbness for a few minutes. Five years before presentation, the patient reported a history of bilateral atrial myxomas, which were revealed by paroxysmal atrial fibrillation and subsequently resected. Drug treatment included oral anticoagulant and a β-blocker. There was no peculiar familial history. Findings from the clinical examination were normal, except for lentigines on his back and face associated with epithelioid-type blue nevi of his left shoulder, leading to the diagnosis of Carney syndrome (Figure 1) (mutation of the PRKAR1A gene). Cerebral magnetic resonance imaging revealed multiple small enhancing lesions on gadolinium-enhanced T1-weighted sequences corresponding to dilated blood vessels (Figure 2). There was neither sign of intracranial bleeding nor cerebral infarction. Cerebral angiography showed multiple fusiform aneurysms in peripheral branches of middle, anterior cerebral, and vertebrobasilar arteries (Figure 2). An abdominal computed tomographic scan revealed other arterial fusiform superior mesenteric aneurysms (Figure 3).

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Figure 1.

Lentiginosis of the back.

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Figure 2.

Multiple distal fusiform cerebral aneurysms.

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Figure 3.

Superior mesenteric artery aneurysms (arrows).

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