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Abstract Details

Cryoglobulinemic Vasculitis as a Cause of Recurrent Small Vessel Strokes
Cerebrovascular Disease and Interventional Neurology
P1 - Poster Session 1 (9:00 AM-5:00 PM)
520

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Cryoglobulinemic vasculitis is a systemic vasculitis and involvement of the cerebral vasculature is uncommon. In the rare occurrence of central nervous system manifestations, it is typically in association with Hepatitis C infection and involves small cerebral arterial vessels. We report a case of CNS cryoglobulinemic vasculitis in the absence of hepatitis C successfully treated with immunosuppression.

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A 75 year old man with hypertension presented with headaches and right-sided weakness. Brain MRI identified strokes in the right pons and left internal capsule, initially thought to be due to hypertensive small vessel disease. While on dual antiplatelet therapy and an optimal anti-hypertensive regimen, he returned one month later with new right sided weakness and left sided numbness and was found to have subacute infarcts in the left lentiform nucleus, right thalamus, and right paramedian pons, and a progressive purpuric rash of the extremities. Intracranial MRA demonstrated no significant stenosis, but MRI vessel wall imaging identified multifocal regions of circumferential vessel wall enhancement.  Skin biopsy of his purpuric rash suggested a leukocytoclastic vasculitis prompting further work up which revealed glomerulonephritis with hypocomplimentemia, positive SSA/SSB antibodies, severe cryoglobulinemia (3840 ug/mL, ref range <50ug/ml) with normal blood viscosity. Parotid gland enlargement was identified on CT imaging and a biopsy was consistent with MALT lymphoma potentially caused by Sjogren's syndrome. His hepatitis panel was negative. CSF showed lymphocytic pleocytosis (7 lymphocytes) and elevated protein. Given concern for life-threatening cryoglobulinemic vasculitis with renal and CNS involvement, high dose steroids and plasma exchange were initiated followed by 6 monthly rituximab infusions leading to improvement in renal function and no further strokes seen on imaging at 6, 12, and 18 months.

Cerebral vasculitis is a rare but treatable manifestation of life-threatening systemic cryoglobulinemia and can occur in the absence of hepatitis C infection. 

Authors/Disclosures
Aaron Shoskes, DO (University of Utah)
PRESENTER
Dr. Shoskes has nothing to disclose.
No disclosure on file