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Neurology India, Vol. 58, No. 5, September-October, 2010, pp. 793-794 Letter to Editor Multiple bilateral cerebral infarcts in a patient with idiopathic hypereosinophilic syndrome Suk-Won Ahn1, Moon-Ku Han2 1 Department of Neurology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea Correspondence Address: Date of Acceptance: 07-Aug-2010 Code Number: ni10221 PMID: 21045519 DOI: 10.4103/0028-3886.72180 Sir, Idiopathic hypereosinophilic syndrome (IHES) is a rare multisystem disorder associated with significant morbidity and mortality. The diagnosis is by exclusion of other causes of hypereosinophilia. [1],[2],[3] IHES is characterized by a persistent eosinophilic count of more than 1500/μL for a period of 6 months or more [1],[2],[3],[4] and is associated with multiple-organ involvement: skin, cardiac, respiratory, gastrointestinal and neurological systems. [4] Cerebral infarction as a complication of IHES is rarely documented. [5],[6],[7] We describe a case of a patient of IHES with multiple bilateral cerebral infarcts. A 56-year-old man was referred with 2-day history of sudden-onset left-side weakness and 1-day history of right-side weakness followed by speech disturbance and swallowing difficulty. He had no history of any vascular risk factors. Neurological examination revealed dysarthria, dysphagia and weakness of all the 4 limbs (4/5). Deep tendon reflexes were bilaterally brisk. Magnetic resonance imaging (MRI) of the brain revealed acute multiple cerebral infarcts in both the hemispheres [Figure - 1]a and b. MR angiography, both intracranial and extracranial, was essentially normal [Figure - 1]c. Transthoracic and transesophageal echocardiograms, Holter monitoring and transcranial Doppler were all normal. Serum glucose, cholesterol, anti-neutrophil cytoplasmic antibody, anti-Sm (smith) antibody, anti-SSA/Ro (Sjogren syndrome A) antibody, anti-SSB/La (Sjogren syndrome B) antibody, cryoglobulin, rheumatoid factor, fluorescent anti-nuclear antibody, lupus anticoagulant, antithrombin III, proteins C and S were all normal. Eosinophil count was 3190/μL (reference range, 50-500/μL). Bone marrow biopsy showed increased hypersegmented eosinophil [Figure - 2]. Additional studies for hypereosinophilia - abdominal and chest computed tomography, cerebrospinal fluid (CSF) study, tumor screening tests, serum and CSF enzyme-linked immunosorbent assay for parasitic diseases - provided normal results. Six months later, the eosinophilic count remained high, 2460/μL. He was put on oral prednisolone, 60 mg/d; and antiplatelet agent. Our patient fulfilled the diagnostic criteria for IHES. The neurological manifestations of IHES include encephalopathy, sensory polyneuropathy and cerebral infarction. The possible mechanisms of cerebral infarction include hypereosinophilic cardiac thromboembolism and hypereosinophilic coagulopathy. [5],[6],[7],[8] Cardiac involvement may include endocardial damage and eosinophil infiltration of the myocardium. Thrombi formation may occur on the damaged endocardium and on the valve leaflets. Progressive scarring may lead to endomyocardial fibrosis resulting in a restrictive cardiomyopathy. [1],[4] In addition, eosinophils contain neurotoxic granule proteins, eosinophil-derived neurotoxin, major basic protein, and eosinophil cationic protein, which can cause thrombosis or microthrombi. [1],[2],[3] However, the mechanism of thrombus formation in IHES and the underlying pathogenic mechanism of IHES remain uncertain. We presume that the cerebral infarcts in this patient were probably related to hypereosinophilia. However, in this patient we could not identify any coagulopathy, mural cardiac thrombus or valvular abnormalities. Probably the cerebral infarcts in this patient may be the result of microthrombi caused by various neurotoxins. To conclude, we suggest that the possibility of IHES should be considered in patients with ischemic stroke and hypereosinophilia. References
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