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Bilateral Medial Medullary Infarction in a Patient with Basilar Artery Fenestration – Cause or Coincidence? Raja Godasi MBBS 1 , Rashid Ahmed MD 1 , Hesham Masoud MD 1 SUNY Upstate Medical University Department of Neurology Introduction Case presentation Conclusion Fenestration refers to localized duplication of a vessel . Basilar artery fenestration is a congenital variant with an incidence of 5 % . Medial medullary infarction (MMI) is a rare stroke syndrome accounting for 0 . 5 - 1 . 5 % of all strokes and bilateral involvement is uncommon . The most common presentation of bilateral MMI is the sudden onset of a variable constellation of findings which can include quadriparesis/quadriplegia, dysarthria, nystagmus, sensory disturbances, hypoglossal palsy and bulbar dysfunction . A 56 - year - old female with no significant cerebrovascular risk factors except for a remote history of breast cancer in remission, presented within 5 hours of sudden onset, whole body paresthesia sparing the face, oscillopsia, nausea and vomiting . Initially neurological examination was remarkable only for bilateral vertical nystagmus . MRI brain disclosed a “V” shaped diffusion restriction in the medial medulla with ADC correlation . CT angiography of the head revealed proximal basilar artery fenestration at the level of medulla . The next day, patient developed asymmetric quadriparesis, dysarthria, hypophonia and dysphagia . Repeat MRI demonstrated evolution of medial medullary infarction with FLAIR hyperintensity . An extensive stroke work - up was unremarkable, except for a patent foramen ovale without evidence of venous thrombosis . Our case, as well as several others, show association between basilar artery fenestration and strokes in the brainstem. Knowledge of this rare entity is important to consider when pursing workup for medullary lacunar strokes. Discussion Pontine and lateral medullary infarctions have been reported in patients with basilar artery fenestrations. However, to our knowledge, this is the first reported case of bilateral medial medullary infarction. Hemodynamic alterations and turbulence are thought to make fenestrated artery a more common site for thrombosis compared to a normal artery. It is also noted that partial endothelium-lined intraluminal septa appearing as spurs are seen in fenestrated arteries. These are hypothesized to cause turbulent blood flow leading to thrombosis and possible embolization from the fenestrated artery. Paradoxical embolic etiology, though a possibility in our case was thought to be unlikely in the absence of venous thrombosis or cortical infarction on DWI. A B C A- Initial MRI DWI showing “V” shaped heart sign in medulla, B- Initial MRI ADC sequence, C- Repeat MRI with subtle FLAIR hyperintensity References D E F D- CT Angiography of the head showing proximal basilar fenestration, E- 3D reconstruction of the fenestration, F- Magnified view of the fenestration -Berry 3rd, A. D., John J. Kepes, and Mark D. Wetzel. "Segmental duplication of the basilar artery with thrombosis." Stroke 19.2 (1988): 256-260. -Pongmoragot, Jitphapa, et al. "Bilateral medial medullary infarction: a systematic review." Journal of Stroke and Cerebrovascular Diseases 22.6 (2013): 775-780. -Woo, Seong-Ryong, et al. "Extreme duplication-type, nonseparated fenestration of the basilar artery in a patient with pontine infarction: confirmation with virtual arterial endoscopy." Journal of Clinical Neurology 2.1 (2006): 74-77. -Tanaka, M., Y. Kikuchi, and T. Ouchi. "Neuroradiological analysis of 23 cases of basilar artery fenestration based on 2280 cases of MR angiographies." Interventional Neuroradiology 12.1_suppl (2006): 39-44.

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Page 1: Bilateral Medial Medullary Infarction in a Patient with ... · endothelium-lined intraluminal septa appearing as spurs are seen in fenestrated arteries. These are hypothesized to

Bilateral Medial Medullary Infarction in a Patient with Basilar Artery Fenestration – Cause or Coincidence? Raja Godasi MBBS1, Rashid Ahmed MD1, Hesham Masoud MD1

SUNY Upstate Medical University Department of Neurology

Introduction

Case presentation

Conclusion

Fenestration refers to localized duplication of avessel. Basilar artery fenestration is a congenitalvariant with an incidence of 5%. Medial medullaryinfarction (MMI) is a rare stroke syndromeaccounting for 0.5-1.5% of all strokes and bilateralinvolvement is uncommon. The most commonpresentation of bilateral MMI is the sudden onset of avariable constellation of findings which can includequadriparesis/quadriplegia, dysarthria, nystagmus,sensory disturbances, hypoglossal palsy and bulbardysfunction.

A 56-year-old female with no significant

cerebrovascular risk factors except for a remote

history of breast cancer in remission, presented within

5 hours of sudden onset, whole body paresthesia

sparing the face, oscillopsia, nausea and vomiting.

Initially neurological examination was remarkable

only for bilateral vertical nystagmus. MRI brain

disclosed a “V” shaped diffusion restriction in the

medial medulla with ADC correlation. CT

angiography of the head revealed proximal basilar

artery fenestration at the level of medulla. The next

day, patient developed asymmetric quadriparesis,

dysarthria, hypophonia and dysphagia. Repeat MRI

demonstrated evolution of medial medullary

infarction with FLAIR hyperintensity. An extensive

stroke work-up was unremarkable, except for a patent

foramen ovale without evidence of venous

thrombosis.

Our case, as well as several others, show association

between basilar artery fenestration and strokes in the

brainstem. Knowledge of this rare entity is important to

consider when pursing workup for medullary lacunar

strokes.

Discussion

Pontine and lateral medullary infarctions have been

reported in patients with basilar artery fenestrations.

However, to our knowledge, this is the first reported case

of bilateral medial medullary infarction. Hemodynamic

alterations and turbulence are thought to make

fenestrated artery a more common site for thrombosis

compared to a normal artery. It is also noted that partial

endothelium-lined intraluminal septa appearing as spurs

are seen in fenestrated arteries. These are hypothesized to

cause turbulent blood flow leading to thrombosis and

possible embolization from the fenestrated artery.

Paradoxical embolic etiology, though a possibility in our

case was thought to be unlikely in the absence of venous

thrombosis or cortical infarction on DWI.

A B C

A- Initial MRI DWI showing “V” shaped heart sign in medulla, B- Initial MRI ADC sequence, C- Repeat MRI with subtle FLAIR hyperintensity

References

D E F

D- CT Angiography of the head showing proximal basilar fenestration, E- 3D reconstruction of the fenestration, F- Magnified view of the fenestration

-Berry 3rd, A. D., John J. Kepes, and Mark D. Wetzel. "Segmental duplication of the basilar artery

with thrombosis." Stroke 19.2 (1988): 256-260.

-Pongmoragot, Jitphapa, et al. "Bilateral medial medullary infarction: a systematic review." Journal

of Stroke and Cerebrovascular Diseases 22.6 (2013): 775-780.

-Woo, Seong-Ryong, et al. "Extreme duplication-type, nonseparated fenestration of the basilar artery

in a patient with pontine infarction: confirmation with virtual arterial endoscopy." Journal of Clinical

Neurology 2.1 (2006): 74-77.

-Tanaka, M., Y. Kikuchi, and T. Ouchi. "Neuroradiological analysis of 23 cases of basilar artery

fenestration based on 2280 cases of MR angiographies." Interventional Neuroradiology 12.1_suppl

(2006): 39-44.