Rare Presentation of Churg-Strauss Syndrome: Intracerebral Hemorrhage, Subdural Hemorrhage and an Intracranial Aneurysm (P2.103)

2014 
OBJECTIVE: To report a rare case of Churg-Strauss syndrome (CSS) who presented with right visual field cut and found to have an intracerebral and subdural hemorrhage along with an aneurysm of the distal parieto-occipital branch of the left MCA. BACKGROUND: CSS is a systemic, necrotizing small to medium-vessel vasculitis that is characterized by asthma, hyper-eosinophilia, paranasal sinus abnormalities, extra-pulmonary vasculitis with rare neurological complications. CASE SUMMARY: 48 year old man with PMH significant for adult-onset asthma diagnosed 2 years prior to admission and peripheral neuropathy diagnosed 1 year ago, presented to an outside hospital with a 2 day history of headache and visual disturbance. CTH revealed a left occipital hemorrhage. Neurological exam revealed a right homonymous hemianopia, normal motor exam, sensory deficits to all modalities in lower extremities along with decreased ankle jerk and patellar reflexes. RESULTS: Labs on admission revealed leukocytosis of 32.2 with marked eosinophilia of 77 %, elevated ESR at 47 and elevated CRP of 83.5. Autoimmune, ID and malignancy work-up was unremarkable. MRI of the brain showed left temporal-occipital embolic ischemic infarcts, left occipital intracerebral hemorrhage and left temporal-occipital subdural hemorrhage. Cerebral angiogram revealed a microaneurysm arising from the distal m4 segment of the parieto-occipital branch of the left MCA. EKG showed left bundle branch block. TEE showed multiple masses measuring 0.5-1cm attached to the left ventricular wall and depressed EF of 30-35%. CT of chest, abdomen and pelvis revealed opacities in lung, liver, kidney and spleen along with bilateral hilar lymphadenopathy. DISCUSSION: CSS can present with rare neurologic complications namely peripheral neuropathy, ischemic and hemorrhagic stroke and much less commonly - intracranial aneurysms. Our patient also had cardiac masses that resolved after treatment with high dose steroids. Subdural hemorrhage along with a left MCA aneurysm related to CSS has not been previously reported in literature. Disclosure: Dr. Chahil has nothing to disclose. Dr. Morgan has nothing to disclose. Dr. Gonzales has nothing to disclose. Dr. Wu has nothing to disclose.
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