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|Year : 2016 | Volume
| Issue : 4 | Page : 827-830
Churg–Strauss syndrome in a pediatric patient presenting with recurrent hemorrhagic stroke and mitral regurgitation due to a prolapsed mitral valve
Shri Ram Sharma, Hibu Habung, Masaraf Hussain
Department of Neurology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya, India
|Date of Web Publication||5-Jul-2016|
Dr. Shri Ram Sharma
Department of Neurology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Mawdiangdiang, Shillong - 789 018, Meghalaya
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sharma SR, Habung H, Hussain M. Churg–Strauss syndrome in a pediatric patient presenting with recurrent hemorrhagic stroke and mitral regurgitation due to a prolapsed mitral valve. Neurol India 2016;64:827-30
|How to cite this URL:|
Sharma SR, Habung H, Hussain M. Churg–Strauss syndrome in a pediatric patient presenting with recurrent hemorrhagic stroke and mitral regurgitation due to a prolapsed mitral valve. Neurol India [serial online] 2016 [cited 2019 Nov 17];64:827-30. Available from: http://www.neurologyindia.com/text.asp?2016/64/4/827/185374
A 6-year-old Nepali girl presented to us with recurrent intracranial hemorrhage. She had a normal birth history and developmental milestones. She has been asthmatic since the age of 3 years. She also had a history of frequent rhinitis. She had a sudden-onset right-sided hemiparesis in April 2013 due to the left parietal lobe bleed [Figure 1]. She recovered completely from her neurological deficits over 5 months. In August 2014, she again developed a sudden-onset left hemiparesis due to the right parietal lobe bleed [Figure 2]. She improved with minimal residual weakness. Since 1 month, she had developed multiple, tender subcutaneous nodules over her left cheek, abdomen, and back.
On physical examination, her general condition was fair. Nervous system examination showed minimal left-sided motor weakness. There were occasional rhonchi heard bilaterally on respiratory examination. Cardiovascular examination revealed a holosystolic murmur, best heard over the apical region of the heart and radiating to the aortic area. A late systolic click was also heard over the apex. She had multiple firm, tender nodules over her left cheek, abdomen, and back. She also had tenderness in the maxillary sinus region. Rest of the examination was within normal limits.
On investigation, the complete blood count showed a total leukocyte count of 19,800 with 30% eosinophilic count, the hemoglobin level of 9.9 g%, the erythrocyte sedimentation rate of 75 mm/1st h, and a normal platelet count. Renal and liver function tests, coagulation profile, serum lipid profile, and electrolyte levels were normal. C-reactive protein was positive (33.4 mg/l) with a negative rheumatoid factor. Viral markers (for hepatitis B surface antigen, anti-hepatitis C virus, venereal disease research laboratory [VDRL] and human immunodeficiency virus [HIV]) were nonreactive. The routine urine examination was normal. Electrocardiogram showed a sinus tachycardia with nonspecific ST-T wave changes. Chest X-ray showed bilateral perihilar and left lower zone infiltrates with minimal right-sided effusion [Figure 3]. Computed tomography (CT) of the brain showed a resolving hematoma in the right parietal lobe with gliosis within the left parietal lobe.
|Figure 3: Chest X-ray showing bilateral perihilar and left lower zone infiltrates with minimal right pleural effusion|
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Her previous medical records showed a persistent hyper-eosinophilic leukocytosis since 2012 (eosinophil > 20% on five occasions). Chest X-rays showed bilateral perihilar infiltrates [Figure 3]. Computed tomographic (CT) angiography of the brain showed extensive leptomeningeal vessels with a normal caliber of the main arteries [Figure 4]. On further investigations, her serum IgE level was found to be very high (1605.5). However, her anti-nuclear antibody, perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) and cytoplasmic ANCA were negative. X-ray of the paranasal sinuses (PNS) showed bilateral maxillary sinus haziness [Figure 5]. Her nerve conduction study was suggestive of mononeuritis multiplex. The two-dimensional echocardiography showed a posterior mitral leaflet prolapse with moderate mitral regurgitation and normal biventricular function [Figure 6]. Fine-needle aspiration cytology of the subcutaneous nodules showed a hemorrhagic smear with fat and a few scattered clusters of histiocytes and foamy macrophages with eosinophils in a hemorrhagic background suggestive of inflammatory lesions. She fulfilled five of the six criteria for Churg–Strauss syndrome [CSS] (American College of Rheumatology criteria), i.e., asthma, persistent hypereosinophilia (>10%), paranasal sinusitis, lung infiltrates, and mononeuritis multiplex. She was started on steroids and pulse therapy of cyclophosphamide and is currently doing well with a normal eosinophil count and subsidence of subcutaneous nodules.
|Figure 4: Computed tomography angiogram shows an extensive presence of leptomeningeal vessels with the normal calibre of main arteries|
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|Figure 5: X-ray of paranasal sinuses shows bilateral maxillary sinus haziness|
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|Figure 6: Two-dimensional echocardiogram showing posterior mitral leaflet prolapse|
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Churg Strauss syndrome (CSS) is a rare syndrome characterized by systemic necrotizing vasculitis of small and medium-sized vessels, associated with the presence of extravascular eosinophilic granulomas, peripheral eosinophilia, and asthma. Due to its rarity, the incidence of the disease in the pediatric age group is unknown. Before 1999, only ten cases of childhood CSS had been reported. The youngest reported patient with CSS was 2 years old.
Three stages have been described in the clinical evolution of CSS. The typical prodromal phase involves allergic rhinitis and asthma. The second stage includes peripheral eosinophilia and an eosinophilic tissue infiltration. The hallmark of the final phase is systemic vasculitis, which if left untreated, can be fatal. It may involve the skin, lungs, musculoskeletal structures, gastrointestinal tract, kidneys, heart, and the paranasal sinuses.
Pulmonary disease is a central feature of CSS. It may take the form of transitory patchy or diffuse pulmonary infiltrates, nodular infiltrates, or pleural effusion, often with a high fever and severe systemic manifestations. The reported patient was asthmatic with the chest X-ray showing bilateral perihilar and left lower zone infiltrates with minimal right-sided effusion [Figure 3]. Her previous chest X-rays also showed transitory patchy infiltrates.
Cardiovascular disease occurs in approximately half of the patients with CSS, irrespective of the age. The specific cardiac involvement in CSS includes pericarditis and less commonly, tamponade and myocarditis. Congestive heart failure/myocardial infarction accounted for 48% of all deaths in one series. Severe cardiomyopathy, occasionally with pericardial effusion, has been reported in children and was fatal in a case. Morgan et al., found that mitral regurgitation is common in CSS. They suggested that mitral regurgitation is caused by diffuse myocardial fibrosis. The patient clinically had a mitral regurgitation, which was later on confirmed by echocardiography. Mitral regurgitation occurred due to the prolapsed mitral valve.
The most common neurological manifestation of CSS is mononeuritis multiplex. Childhood-onset CSS is less likely to be complicated by central nervous system vasculitis than occurs in adults. The patient had two episodes of intracranial hemorrhage that resulted in hemiparesis. She also had mononeuritis multiplex on nerve conduction studies.
Skin manifestations are commonly present in CSS in childhood. They range from discrete to diffuse, petechial, or purpuric lesions that may be itchy, edematous, or tender. The patient had multiple, firm, tender subcutaneous nodules over the cheek, abdomen and back which regressed after administration of steroids.
Stroke in CSS has been reported in adults, but recurrent hemorrhagic stroke in children has not been reported so far. Although mitral regurgitation is common in CSS due to myocardial fibrosis, the association of the prolapsed mitral valve with CSS needs further evaluation as it has not been described nor reported in the literature so far. A high index of suspicion is the key to the diagnosis of CSS in childhood.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]