SHM Converge Scientific Abstract ePoster Gallery-Moyamoya Angiopathy Masquerading as Lupus Cerebral Vasculitis: A Diagnostic Challenge

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This case describes a 51-year-old woman with hypertension and systemic lupus erythematosus (SLE) who presented after a recent lupus flare with headaches, bilateral hand numbness, and sudden right-sided tremor, facial droop, and weakness, along with mild speech hesitation. Her neurologic exam showed a subtle right upper extremity drift and an NIHSS score of 2.<br /><br />Initial laboratory testing, including electrolytes and kidney/liver function, was normal. Cerebrospinal fluid studies were not suggestive of infection or paraneoplastic disease. Brain MRI and CT raised concern for moyamoya angiopathy versus vasculitis. Digital subtraction angiography later confirmed findings compatible with moyamoya disease, and there was no vessel wall enhancement to indicate active vasculitis.<br /><br />Moyamoya is a rare progressive cerebrovascular disorder in which the vessels at the base of the brain narrow over time, reducing blood flow and prompting formation of fragile collateral vessels. The name means “puff of smoke” in Japanese, referring to the characteristic appearance of these small vessels on angiography.<br /><br />The patient was managed medically with aspirin and atorvastatin, while continuing her home prednisone and mycophenolate mofetil for SLE. A SPECT study showed good collateral circulation, so bypass surgery was not needed. Her right arm strength improved, and she was discharged home.<br /><br />This case highlights the importance of considering moyamoya angiopathy in patients with SLE who develop acute focal neurologic deficits, especially when imaging may initially suggest vasculitis or stroke.

Asset Subtitle

Hehui Quan

Meta Tag

Author List Emily Rodriguez, Hehui Quan, Joseph McClanaghan, Liam Cotter, Rebecca Zeidman, Saul Kruger, Tina Kana

Category Clinical Vignettes

Concept Moyamoya Angiopathy

Concept Intracranial Vessel Narrowing

Concept Portosystemic Collateral Veins

Concept Neurologic Deficit

Concept Vasculitis

Distinguished Non-Finalist

Presenter Organization Lenox Hill Hospital

Presenting Author Hehui Quan

Track Adult

Keywords

moyamoya disease

systemic lupus erythematosus

stroke

vasculitis

angiography

neurologic deficits

cerebrovascular disorder

SPECT

aspirin

mycophenolate mofetil

Moyamoya Angiopathy

Intracranial Vessel Narrowing

Portosystemic Collateral Veins

Neurologic Deficit

Vasculitis