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Images in Neurology |

Dural Arteriovenous Fistula of the Medulla Initially Mimicking Guillain-Barré Syndrome

Camilla Neergaard Clark, MRCP; Tabish A. Saifee, MRCP; Peter O. Cowley, FRCR; Lionel Ginsberg, PhD, FRCP
Arch Neurol. 2012;69(6):786-787. doi:10.1001/archneurol.2011.2934.
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A 65-year-old woman who was previously well presented with 2 days of ascending flaccid quadriparesis. This had been preceded by 2 weeks of gastroenteritis, with an episode of urinary incontinence at onset. On admission, she was catheterized for urinary retention. She was areflexic in her lower limbs, and power was markedly reduced proximally (1/5) more than distally (4/5). In the upper limbs, her reflexes were present with reinforcement, with global reduction of power (4/5). Her presentation was felt to be consistent with Guillain-Barré syndrome. However, her weakness and areflexia improved 12 hours after admission, returning to full power with brisk reflexes in the upper limbs, reduced knee jerks, and absent ankle jerks. Her power continued to fluctuate while on the ward. These atypical aspects pointed away from a diagnosis of Guillain-Barré syndrome1 and prompted magnetic resonance imaging of the brain and cervical spine, the results of which showed features consistent with a medullary dural arteriovenous fistula, later confirmed by the results of angiography (Figure).

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Figure. A, T2-weighted axial magnetic resonance imaging scan of the head of a 65-year-old woman that shows a diffuse symmetrical signal abnormality within the medulla with extension inferiorly into the cervical cord, suggestive of venous congestion. There were a few prominent perimedullary flow voids. B, Catheter angiogram (sagittal plane; contrast injected into the right external carotid artery) confirmed the presence of a dural arteriovenous fistula, the fistulous point being located in the region of the right superior petrosal vein with (presumed) venous occlusions leading to congestion in the perimedullary and upper cervical venous channels. The dural supply was from small and tortuous branches of the middle meningeal artery and the meningohypophyseal trunk. The fistulous point was occluded using endovascular embolization with onyx and subsequently surgically. After an initial postoperative episode of quadriparesis and respiratory muscle weakness, the patient is now undergoing neurorehabilitation.




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