Multiple cerebral infarctions in a patient with Churg-Strauss syndrome

Ghaeni, Leyli; Siebert, Eberhard; Ostendorf, Florian; Endres, Matthias; Reuter, Uwe

doi:10.1007/s00415-009-5439-1

Cited by 17 publications

(10 citation statements)

References 14 publications

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“…In EGPA, neurological manifestation is common, usually consisting of peripheral neuropathy due to small-vessel vasculitis, whereas cerebral manifestation is less frequent, especially when due to brain embolism [ 17 ]. The source of brain embolism includes the left ventricle and even the ascending aorta [ 18 ].…”

Section: Discussionmentioning

confidence: 99%

“…The source of brain embolism includes the left ventricle and even the ascending aorta [ 18 ]. However, according to one series of cases, none of the EGPA patients had any known cause in the heart that could account for cerebral/cerebellar infarcts [ 17 , 19 ]. Psychogios et al reported a case of EGPA in which multiple cerebral infarcts, along with purpuric skin rash, was the first manifestation of EGPA, although in the case of a 63-year-old female, no apparent findings suggestive of cardioembolism were detected on echocardiography [ 20 ].…”

Section: Discussionmentioning

confidence: 99%

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Eosinophilic Granulomatosis with Polyangiitis (EGPA) with an Unusual Manifestation of Mid-Ventricular Obstruction Caused by Endocardial Thrombus

et al. 2018

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Patient: Male, 46Final Diagnosis: Eosinophilic granulomatosis with polyangiitisSymptoms: Chest painMedication: —Clinical Procedure: Skin biopsySpecialty: CardiologyObjective:Rare co-existance of disease or pathologyBackground:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause accompanied by prominent eosinophilia. Intracardiac thrombosis is one of the major cardiac complications in EGPA that may cause thromboembolism.Case Report:A 46-year-old male presenting with intermittent chest pain and numbness of the lower extremities was admitted to our center. His case was complicated by multiple brain infarcts and endocardial thrombosis in the left ventricle. A condition of restrictive cardiomyopathy was also found. After a thorough workup, he was diagnosed with antineutrophil cytoplasmic antibody (ANCA) positive EGPA. Interestingly, the thrombus was accompanied by a pressure gradient producing mid-ventricular obstruction. The patient improved reasonably with immunosuppression and anticoagulation treatment, in addition to heart failure treatment, and had a concomitant regression of the thrombus and reduction of the pressure gradient.Conclusions:For an EGPA patient complicated by intraventricular obstruction caused by endocardial thrombosis, which could accelerate the release of the thrombus into the systemic circulation resulting in a life-threating condition, timely and aggressive measures against cardioembolic complications should be considered.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Eosinophilic Granulomatosis with Polyangiitis (EGPA) with an Unusual Manifestation of Mid-Ventricular Obstruction Caused by Endocardial Thrombus

et al. 2018

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“…A previous case series included brain infarcts as a manifestation or complication of Churg-Strauss syndrome in 3 patients [10]. One case report described intracerebral haemorrhage as a rare sequel of Churg-Strauss syndrome [11], and another reported an elderly woman with multiple cerebral infarctions and hypereosinophilia with assumed cardiac involvement and multiple cerebral infarctions due to cardiac embolism [12]. …”

Section: Discussionmentioning

confidence: 99%

Churg-Strauss Syndrome as an Unusual Aetiology of Stroke with Haemorrhagic Transformation in a Patient with No Cardiovascular Risk Factors

Sairanen¹,

Kanerva

Valanne

et al. 2011

Case Rep Neurol

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Background: We present here a case of haemorrhagic brain infarction in a middle-aged and physically active male, who had never smoked. This case report aims to remind the internist and neurologist to bear in mind unusual aetiologies of brain infarcts in patients without classical cardiovascular risk factors. Case Description: A 49-year-old male with pulmonary asthma and a prior history of nasal polyps had a wake-up stroke with left-sided symptoms and speech disturbance. A head MRI and MR angiography revealed a recent haemorrhagic infarct in the right putamen and corona radiata. The left hemiparesis progressed to sensory-motor hemiplegia on the 4th day. In the head CT, it was shown that the haemorrhagic infarct had progressed to a large haematoma. A pansinusitis was also diagnosed. The aetiological investigations revealed a minor atrial septal defect (ASD) with shunting and a heterozygotic clotting factor V R506Q mutation. A remarkable blood eosinophilia of 9.80 E9/l (42%) together with fever, sinusitis, wide-spread bilateral nodular pulmonary infiltrates that did not respond to wide-spectrum antimicrobial treatment, positive anti-neutrophilic cytoplasmic antibodies, a high myeloperoxidase antibody level and slightly positive anti-proteinase 3 antibodies suggested the diagnosis of Churg-Strauss syndrome. These inflammatory symptoms and findings promptly responded to treatment with corticosteroids and cyclophosphamide. Conclusions: Even after the concomitant findings of the low risk factors, i.e. small ASD and heterozygotic clotting factor mutation, continued search for the final aetiology of stroke revealed Churg-Strauss syndrome, which was the key to the treatment.

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“…Eosinophilic granulomatosis with polyangiitis (EGPA) causes bronchial asthma and allergic rhinitis as a preceding symptom and results in vasculitis accompanied with eosinophilia [1, 2]. In rare cases, EGPA is thought to exhibit multiple cerebral infarction in cortical, subcortical, and watershed areas, and mechanisms of vasculitis and special granules secreted by eosinophils have been considered [3]. Furthermore, because of the presence of left ventricular thrombus due to eosinophilic myocarditis, embolism is considered a cause, but the details are unknown [3].…”

Section: Introductionmentioning

confidence: 99%

“…In rare cases, EGPA is thought to exhibit multiple cerebral infarction in cortical, subcortical, and watershed areas, and mechanisms of vasculitis and special granules secreted by eosinophils have been considered [3]. Furthermore, because of the presence of left ventricular thrombus due to eosinophilic myocarditis, embolism is considered a cause, but the details are unknown [3].…”

Section: Introductionmentioning

confidence: 99%

Multiple cerebral infarction diagnosed as Eosinophilic Granulomatosis with Polyangiitis by autopsy

et al. 2019

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BackgroundEosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause involving the brain and accompanied by prominent eosinophilia. Intracardiac thrombosis is a major cardiac complication of EGPA that may cause thromboembolism.Case presentationA 53-year-old man presenting with abulia (consciousness disturbance) and left upper limb paralysis was admitted to our hospital. His case was complicated by penetrating branches, small vessel infarcts, and endocardial thrombosis in the right and left ventricle. Cardiomyopathy was also observed. Sixteen days after admission, the patient died from intracranial hemorrhage. Brain autopsy revealed two major findings: 1) large hemorrhagic infarction caused by cardiac embolism; and 2) granuloma and eosinophil infiltration. Vasculitis was accompanied by eosinophil infiltration in the cortical blood vessels and granuloma.ConclusionsIn this case study, we report autopsy findings of brain infarction in a patient with EGPA and endocardial thrombosis. The brain infarction was caused by the cardiac embolisms and vasculitis.

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Multiple cerebral infarctions in a patient with Churg-Strauss syndrome

Cited by 17 publications

References 14 publications

Eosinophilic Granulomatosis with Polyangiitis (EGPA) with an Unusual Manifestation of Mid-Ventricular Obstruction Caused by Endocardial Thrombus

Eosinophilic Granulomatosis with Polyangiitis (EGPA) with an Unusual Manifestation of Mid-Ventricular Obstruction Caused by Endocardial Thrombus

Churg-Strauss Syndrome as an Unusual Aetiology of Stroke with Haemorrhagic Transformation in a Patient with No Cardiovascular Risk Factors

Multiple cerebral infarction diagnosed as Eosinophilic Granulomatosis with Polyangiitis by autopsy

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