J Neurol (2013) 260:2659–2661 DOI 10.1007/s00415-013-7088-7
LETTER TO THE EDITORS
Churg-Strauss syndrome mimicking myocardial infarction with cerebral vascular involvement C. Gandolfo • M. Balestrino • C. Finocchi E. Viani
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Received: 5 June 2013 / Revised: 20 August 2013 / Accepted: 21 August 2013 / Published online: 1 September 2013 Ó Springer-Verlag Berlin Heidelberg 2013
Dear Sirs, Churg-Strauss syndrome (CSS) is a rare multisystemic disorder defined by the American Rheumatological Society [1] as an eosinophilic vasculitis plus one or more of the following: asthmatic airway obstruction, [10 % eosinophils, migratory or transient pulmonary infiltrates, sinusitis and mono- or poly-neuropathy. In a minority of cases the process involves the central nervous system [2] or the heart [3]. We describe a very rare case of a patient with simultaneous cardiac and encephalic involvement due to ChurgStrauss syndrome. A 59 y.o. male, whose medical history included mild hypertension, asthma, chronic sinusitis, and nasal polyposis, was admitted to the emergency department because of sudden onset of mental confusion. The neurological examination showed psychomotor slowing, mild right hemiparesis, bilateral Babinski sign, increased deep tendon reflexes in the four limbs, and instability in the standing position. An encephalic MRI showed multiple bilateral cerebral and cerebellar small ischemic lesions in diffusionweighted sequences (Fig. 1). Troponin I was markedly elevated (4.380 lg/l, with a further rapid increase to 8.140 lg/l), thus, the patient was transferred to the coronary unit. Coronarography did not show any stenosis (obviously, this could not rule out small vessel cardiac ischemia). A trans-thoracic echocardiogram and several EKGs were also normal, thus, the patient was transferred to our Stroke Unit because of the MRI lesions and of the mild right hemiparesis. Marked hyper-eosinophilia was found
C. Gandolfo (&) M. Balestrino C. Finocchi E. Viani Stroke-Unit, Genoa University Hospital, Largo P. Daneo, 3, 16132 Genoa, Italy e-mail:
[email protected]
(absolute value 2.7 9 109/L, 24.3 % of total leukocytes), with a strong elevation of IgE (368 kU/L; normal range 0–25) and inflammation indexes. Parasites and bone marrow pathologic proliferation were ruled out. A total body CT scan showed pulmonary bilateral diffuse micro-nodular appearance with bilateral pleural effusions. Immunologic tests for vasculitis were normal with the exception of cANCA (positive 1:20). A tentative diagnosis of vasculitis (Churg-Strauss syndrome) was done, and methylprednisone 1 g daily i.v. was started. A dramatic improvement of
Fig. 1 Axial MR diffusion-weighted sequence showing multiple bihemispheric cortical and sub-cortical recent cerebral ischemic lesions
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Table 1 CNS involvement in CSS (single case reports) Reference
Age/sex
Clinical findings
Neuro-imaging
Laboratory and other instrumental findings
Kok et al. [5]
13/F
Choreatic movements
MRI: WML and Globus Pallidus hyperintesities
TTE negative; Skin biopsy positive.
Dinc et al. [6]
46/F
Cortical blindness
MRI: bilateral occipital infarcts
ANCA negative; TTE and TEE negative.
Kang et al. [7]
22/M
Right hemiparesis; aphasia
MRI: infarcts in the territories of MCA and ACA
MCA occlusion and ACA stenosis; Hypereosinophilia; Temporal artery biopsy positive.
Sonneville et al. [8]
42/F
Left hemiparesis; hemianopsia
MRI: bilateral borderzone infarcts
TTE: Myocarditis, ANCA positive; Skin biopsy negative
Bhagirath et al. [9]
30/M
Right hemiparesis; aphasia
MRI: MCA infarct
TTE: Multiple left intra-ventricular thrombi; Hypereosinophilia.
Ghaeni et al. [10]
77/F
Tetraparesis; global aphasia; disorientation
MRI of the brain revealed multiple small, predominantly cortical infarctions in all vascular territories
Hypereosinophilia; Pneumonic infiltration; Sinusitis; Skin biopsy positive; pANCA positive.
Gandolfo et al. 2013 (present case)
59/M
Mental confusion; right hemiparesis; static ataxia
MRI: multiple bi-hemispheric and cerebellar small infarcts
Hypereosinophilia, Pneumonic infiltration; Sinusitis; cANCA positive; TTE, TEE, and Coronarography negative.
mental status was observed, together with a significant improvement of right weakness and ataxia. Methylprednisone was continued for five days, afterwards it was reduced to 0.5 g daily for three days then replaced by oral prednisone 1 mg/Kg daily (i.e., 75 mg daily), tapered over four weeks. Afterwards, immunosuppressive therapy with azathioprine was started. The previous anti-hypertensive therapy was continued and anti-platelet therapy (ASA 100 mg/daily) was added. A second cranial MRI, one week after admission, confirmed multiple small ischemic lesions with restricted water diffusion in the left cerebellar lobe and in both cerebral hemispheres, both cortical and subcortical. Transesophageal echocardiography, tumor markers, and screening for thrombophilic disorders were normal. A third cranial MRI, two weeks after clinical onset, showed a marked reduction in number and dimension of ischemic lesions, with residual restricted water diffusion only in the white matter of semi-oval centers of both hemispheres. In conclusion, this is an unusual case of inflammatory systemic disorders that simulates a primary myocardial infarction with cerebral involvement that was initially attributed to cerebral cardio-embolism. Systemic vasculitis may be considered in patient with acute disseminated vascular encephalopathy and myocardial involvement without atherosclerosis and other common risk factors. Diagnosis should be timely, to start treatment with steroid and immunosuppressant drugs as soon as possible to prevent recurrences and clinical deterioration.
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The literature reports only a few cases with cerebral multi-infarcts due to middle size or small vessel vasculitis during acute CSS [4–10]. Even rarer is the simultaneous cardiac and encephalic involvement mimicking acute myocardial infarction complicated by multiple cardiogenic intracranial embolisms [7, 9, 10]. Our present case represents CSS with myocardial ischemia combined with encephalopathy with cerebral multifocal and bilateral signs and mental status changes. Single case reports of stroke or cerebral vascular encephalopathy during CCS are in Table 1. Acknowledgments The authors thanks Prof. Leonard H. Calabrese, Cleveland, OH 44195, USA, for helpful comments about diagnostic and therapeutic addresses. Conflicts of interests
None.
Ethical standard This study has been performed in the accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.
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J Neurol (2013) 260:2659–2661 3. Neumann T, Manger B, Schmid M et al (2009) Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine (Baltimore) 88:236 4. Cheng M-J, Huang P-H, Liao P-W, Chen J-T, Chiang T-R (2012) Multiple cerebral and cerebellar infarcts as the first clinical manifestation in a patient with Churg-Strauss syndrome: case report and literature review. Acta Neurol Taiwan 21:169–175 5. Kok J, Bosseray A, Brion JP, Micoud M, Besson G (1993) Chorea in a child with Churg-Strauss syndrome. Stroke 24:1263–1264 6. Dinc A, Soy M, Pay S, Simsek I, Erdem H, Sobaci G (2000) A case of Churg-Strauss syndrome presenting with cortical blindness. Clin Rheumatol 19:318–320
2661 7. Kang DW, Kim DE, Yoon BW, Seo JW, Roh JK (2001) Delayed diagnosis: recurrent cerebral infarction associated with ChurgStrauss syndrome. Cerebrovasc Dis 12:280–281 8. Sonneville R, Langrange M, Guidoux C, Michel M, Khellaf M, Russel S, Hosseini H (2006) The association of cardiac involvement and ischemic stroke in Churg Strauss syndrome. Rev Neurol (Paris) 162:229–232 9. Bhagirath KM, Paulson K, Ahmadie R, Bhalla RS, Robinson D, Jassal DS (2009) Clinical utility of cardiac magnetic resonance imaging in Churg-Strauss syndrome: case report and review of the literature. Rheumatol Int 29:445–449 10. Ghaeni L, Siebert E, Ostendorf F, Endres M, Reuter U (2010) Multiple cerebral infarctions in a patient with Churg-Strauss syndrome. J Neurol 257:678–680
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