Brain Disorders (Jun 2022)

Hypereosinophilic syndrome presents as multifocal acute ischemic strokes: Case series and review

  • Shamik Shah, MD, MPH,
  • Urvish Patel, MD, MPH,
  • Neev Mehta, MD, MPH,
  • Jonathan Boyd, MD

Journal volume & issue
Vol. 6
p. 100033

Abstract

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Introduction: Hypereosinophilic syndrome (HES) is a rare condition associated with hypereosinophilia and associated with multi-organ damage. HES presents with neurological manifestations such as ischemic strokes, encephalopathy, peripheral neuropathy, and/or venous sinus thrombosis. This article discusses and reviews the rare presentation of multiple acute ischemic strokes in HES. Case Series: A 51-year-old male presented with right forearm numbness for a week; two days onset of the left arm and hand weakness, gait instability, intermittent disorientation, dark spots in vision; and intermittent dysarthria. MRI showed multiple small strokes in bilateral cerebral and cerebellar areas. He had leukocytosis with 53% eosinophils. TTE and TEE were negative for thrombus. A cerebral angiogram remained negative for vasculitis. Bone marrow biopsy revealed 40% eosinophils with abnormal T-lymphocytes with phenotyping positive for CD3- CD4+. His-final diagnosis was a T-lymphocyte variant of HES. A 39-year-old right-handed male presented with changes in mental status. A day prior to the presentation, he woke up with headache, dizziness, blurry vision, right arm weakness, subsequently found to have gait imbalance, and falls. He had leukocytosis with 60% eosinophils. Brain MRI showed innumerable acute tiny strokes in bilateral cerebral hemispheres and cerebellum. Cardiac MRI showed multiple cardiac thrombi with increased left ventricular thickness. Bone marrow aspirate showed eosinophilia with minimal monoclonal B-cell population. The diagnosis was consistent with HES. Both patients responded well to steroids with a resolution of eosinophilia. Conclusion: HES can have an initial presentation of multifocal ischemic infarcts. Multiple acute ischemic strokes in the presence of hematological abnormalities should lead to suspicion for blood dyscrasia.

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