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Year : 2010  |  Volume : 58  |  Issue : 5  |  Page : 793-794

Multiple bilateral cerebral infarcts in a patient with idiopathic hypereosinophilic syndrome

1 Department of Neurology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea
2 Department of Neurology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Bundang, Gyeonggi-do, Korea

Date of Acceptance07-Aug-2010
Date of Web Publication28-Oct-2010

Correspondence Address:
Moon-Ku Han
Department of Neurology, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Bundang, Gyeonggi-do
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.72180

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How to cite this article:
Ahn SW, Han MK. Multiple bilateral cerebral infarcts in a patient with idiopathic hypereosinophilic syndrome. Neurol India 2010;58:793-4

How to cite this URL:
Ahn SW, Han MK. Multiple bilateral cerebral infarcts in a patient with idiopathic hypereosinophilic syndrome. Neurol India [serial online] 2010 [cited 2022 Aug 15];58:793-4. Available from: https://www.neurologyindia.com/text.asp?2010/58/5/793/72180


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.
Figure 1 :MRI of brain: Diffusion-weighted image (a) and FLAIR image (b) showing multiple bilateral cerebral infarcts and essentially normal MR angiography (c)

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Figure 2 :Microscopic images of bone marrow smear showed hypocellular marrow with a slight increase of eosinophils with hypersegmented nuclei (arrow), which is compatible with a diagnosis of idiopathic hypereosinophilic syndrome

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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 Top

1.Roufosse F, Cogan E, Goldman M. Recent advances in pathogenesis and management of hypereosinophilic syndrome. Allergy 2004;59:673-89.  Back to cited text no. 1
2.Brito-Babapulle F. The eosinophilias including the idiopathic hypereosinophilic syndrome. Br J Haematol 2003;121:203-23.  Back to cited text no. 2
3.Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood 1994;83:2759-79.  Back to cited text no. 3
4.Kobayashi S, Inokuma S, Setoguchi K, Kono H, Abe K. Incidence of peripheral blood eosinophilia and threshold eosinophilie count for indicating hypereosinophilia-associated disease. Allergy 2003;57:950-6.  Back to cited text no. 4
5.Sarazin M, Caumes E, Cohen A. Multiple microembolic borderzone brain infarction and endomyocardial fibrosis in idiopathic hypereosinophilic syndrome and in Schistosoma mansoni infestation. J Neurol Neurosurg Psychiatry 2004;75:305-7.  Back to cited text no. 5
6.Takeuchi S, Takasato Y, Masaoka H, Hayakawa T, Otani N, Yoshino Y, et al. Middle cerebral artery occlusion resulting from hypereosinophilic syndrome. J Clin Neurosci 2010;17:377-8.  Back to cited text no. 6
7.Grigoryan M, Geisler SD, St Louis EK, Baumbach GL, Davis PH. Cerebral arteriolar thromboembolism in idiopathic hypereosinophilic syndrome. Arch Neurol 2009;66:528-31.  Back to cited text no. 7
8.Briani C, Baracchini C, Zanette G, Zanusso G, Carollo C, Monaco S. Rapidly progressive dementia in hypereosinophilic syndrome. Eur J Neurol 2001;8:279-80.  Back to cited text no. 8


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