Churg-Strauss syndrome mimicking myocardial infarction with cerebral vascular involvement

Abstract

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 monoor 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 (absolute value 2.7 9 10/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