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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 3  |  Page : 314-315

Spinal cord involvement of Churg-Strauss syndrome with multi-organ disorders


Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University; Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing, China

Date of Web Publication18-Jul-2014

Correspondence Address:
Chenlong Yang
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University; Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.137001

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How to cite this article:
Yang C, Wu L, Yang T, Deng X, Xu Y. Spinal cord involvement of Churg-Strauss syndrome with multi-organ disorders. Neurol India 2014;62:314-5

How to cite this URL:
Yang C, Wu L, Yang T, Deng X, Xu Y. Spinal cord involvement of Churg-Strauss syndrome with multi-organ disorders. Neurol India [serial online] 2014 [cited 2019 Aug 19];62:314-5. Available from: http://www.neurologyindia.com/text.asp?2014/62/3/314/137001


Sir,

Allergic granulomatous angiitis, known as Churg-strauss syndrome (CSS), is a rare systemic disease characterized by asthma, eosinophilia, and necrotizing vasculitis. Central nervous system (CNS) involvement is extremely rare and occurs in 6-7% of cases. The most frequent complications include cerebral infarctions, subarachnoid hemorrhage (SAH), and intracerebral hemorrhage (ICH). [1] Spinal cord involvement has been described rarely. This report presents a case of CSS with radiologic spinal involvement.

A 59-year-old man presented with a 2-month history of progressive lower extremities pain, weakness, and numbness and was wheel-chair bound since 1 month. He had undergone cholecystectomy 3 months prior to this admission. He gave a 25-year history of asthma. Neurological examination revealed distal motor weakness (grade 3/5) in lower extremities and decreased sensation to pinprick and light touch in hands and lower extremities. Spinal magnetic resonance imaging (MRI) revealed an intradural extramedullary nodule at T12 with intense and homogeneous gadolinium-enhancement [Figure 1]. The mass was isointense on both T1- and T2-weighted images. Cranial MRI revealed ethmoid and maxillary sinusitis, right-sided inferior turbinate hypertrophy [Figure 2], and multiple foci of expanded perivascular space in the thalamic and periventricular areas. Plain X-ray revealed increased lung markings and nodular calcification [Figure 3]a. Computed tomography (CT) scan chest showed nodules and fibrous stripes and mild pericardial effusion [Figure 3]b and c Electrodiagnostic tests revealed sensorimotor neuropathy. Laboratory evaluation revealed elevated leukocyte (16,230/μL) count with eosinophilia (6,530/μL, 40.2%) and thrombocytosis (475,000/μL); elevated IgE (3.2 mg/L, normal value 0.1-0.9) and IgG (21 g/L, normal value 7-16) levels, elevated C-reactive protein (CRP, 127 mg/L; normal value <10 mg/L) level, and erythrocyte sedimentation rate of 36 mmHg during the first hour. Based on the American College of Rheumatology diagnostic criteria, a diagnosis of presumed CSS was considered. Biopsy of skin, superficial peroneal nerve, and muscle revealed vascular intimal thickening and perivascular eosinophil infiltrates. He was given 3-day pulse intravenous methylprednisolone (1000 mg/day) followed by oral prednisone (55 mg/day). For neuropathic pains, he was given pregabalin (300 mg/day). With this regimen, he showed significant improvement in pain and neurological symptoms. One week later, the prednisone dosage was adjusted to 30 mg/day. At 1-month follow-up, his blood counts were normal.

CSS is a rare systemic disease characterized by allergic granulomatous angiitis, often in association with asthma and eosinophilia. Neurological involvement is common and occurs in about 65-75% of cases, and the most common manifestation is peripheral neuropathy. [1] CNS involvement is extremely rare. The American College of Rheumatology diagnostic criteria includes: (a) Asthma; (b) eosinophilia; (c) mononeuropathy or polyneuropathy; (d) non-fixed pulmonary infiltrates; (e) paranasal sinus abnormality; and (f) extravascular eosinophils. The presence of any 4 or more of these 6 criteria should suggest the diagnosis as CSS. [2] This patient had all the six criteria. Pulmonary infiltrates are present in most patients as bilateral multifocal consolidation. Cardiac involvement includes congestive heart failure, hydropericardium, arrhythmia, etc., Concomitant pancreatitis and cholecystitis have been reported, and this patient was operated for cholecystitis prior to admission.
Figure 1: Gd-enhanced sagittal (a), coronal (b) and axial (c) T1-weighted MRI of the spine reveal an enhanced nodule at T12 (white arrows)

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Figure 2: Axial T2-weighted cranial MRI demonstrates ethmoid sinusitis (a) and multiple foci of expanded perivascular space in the thalamic and periventricular areas (b, arrowheads)

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Figure 3: Plain X-ray film revealed increased lung markings and nodules of calcification (a). Computed tomography scan of the chest shows a pericardial effusion on mediastinal window (b) and nodules and fibrous stripes on lung windows (c)

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The neurologic features in this patient are consistent with peripheral neuropathy, which was corroborated by electrodiagnostic tests. The radiological spinal involvement was asymptomatic. In addition, he also had gastrointestinal involvement in the form of cholecystitis. In patients with multi-organ involvement, 1000 mg intravenous methylprednisolone for 3 days, followed by 40-60 mg of prednisone daily can induce remission. [3] Some authors advocate the use of both corticosteroid and immunosuppressors. [4] Prognosis of CSS is generally satisfactory, and most patients respond well to corticosteroids alone. [1],[5] About 20% patients experience remissions and relapses, and in such patients, continued treatment with low-dose glucocorticoids and close observation are needed. Multi-organ involvements were reported as risk factors for adverse clinical outcomes. [1] Thus, early diagnosis based on systematic assessment and timely treatment should be particularly emphasized in order to prevent further progression and even life-threatening organ involvement.

 
  References Top

1.Noth I, Strek ME, Leff AR. Churg-Strauss syndrome. Lancet 2003;361:587-94.  Back to cited text no. 1
    
2.Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094-100.  Back to cited text no. 2
    
3.Quax RA, Swaak AJ, Baggen MG. Churg-Strauss Syndrome following PTU treatment. Int J Rheumatol 2009;2009:504105.  Back to cited text no. 3
    
4.Nakamura M, Yabe I, Yaguchi H, Kishimoto R, Mito Y, Fujiki N, et al. Clinical characterization and successful treatment of 6 patients with Churg-Strauss syndrome-associated neuropathy. Clin Neurol Neurosurg 2009;111:683-7.  Back to cited text no. 4
    
5.Solans R, Bosch JA, Pérez-Bocanegra C, Selva A, Huguet P, Alijotas J, et al. Churg-Strauss syndrome: Outcome and long-term follow-up of 32 patients. Rheumatology (Oxford) 2001;40:763-71.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]

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