Polinöropatinin Nadir Bir Nedeni: Churg Strauss Sendromu
Küçük Resim Yok
Tarih
2016
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Churg Strauss Sendromu, küçük ve orta boy vasküler yapıları etkileyen sistemik- nekrotizan bir vaskülittir. Olguların %60-70inde nöropati gelişmektedir. Bu çalışmada klasik immünsupresanlara dirençli 60 yaşındaki bir bayan olgu sunulmaktadır. .Son bir yıldır astım bronciale tanısıyla izlenmekteydi ve her iki bacakta purpurik lezyonları vardı. Tam kan incelemesinde hipereozinofilisi, paranasal sinüs bilgisayarlı tomografide kronik sinüzit lehine mukozal kalınlaşmaları, cilt biyopsisinde perivasküler eozinofili infiltrasyonu saptandı. IgE düzeyi yüksekti. İlk elektrofizyolojik değerlendirmede aksonal sensorimotor polinöropati saptandı. Pulse prednizolon 1gr/gün ve siklofosfamid 750mg olarak uygulanan kombine tedaviye rağmen ağrılarda azalmayla birlikte sağ el ve sol bacakta kas güçsüzlüğünde ilerleme ve sağ el interosseoz kaslarda belirgin atrofi gözlenmesi üzerine yapılan kontrol sinir iletim çalışmaları asimetrik aksonal etkilenmenin olduğu mononöritis mültipleks ile uyumlu olarak değerlendirildi. Siklofosfamid tedavisine dirençli olduğu düşünülen olguya rituksimab başlandı. İki ay sonra yapılan iğne EMG'de rejenerasyon gözlendi. Bu CSS olgusunu geleneksel tedavilerden yanıt alınamadıktan sonra alternatif olarak denenen rituksimab tedavisine iyi yanıt vermesi nedeniyle sunmayı amaçladık.
Churg Strauss Syndrome is a systemic necrotising vasculitis affecting small and medium sized vessels. The neuropathy may occur in 60-70% of cases. In this study, we present a 60year-old female patient resistant to conventional immunosuppressants. Over the past year she has been followed with the diagnosis of astma bronchiale and she had purpuric lesions in both legs. Her test results showed eosinoplilia, Paranasal sinüs BT showed mucosal thickening in favor of chronic sinusitis, perivascular infiltration of eosinophils were present in the skin biopsy. Axonal sensorymotor polyneuropathy was determined in first electrophysiologic evaluation. Levels of total serum IgE were high. Although pulse of prednisolone 1gr/day treatment combined with 750 mg cyclophosphamide was given, muscle weakness progressed on right hand and left leg and she had remarkable atrophy of interosseous muscle of right hand. Nerve conduction studies revealed mononeuropathy multiplex ( asymmetric axonal sensorimotor neuropathy). The patient, who was considered refractory to cyclophosfamide therapy, has started using rituximab. Two months later, regeneration was observed in needle electromyography.In this case, we aim to provide the clinical , laboratory and electrophysiological findings with CSS who has responsed well to treatment with rituximab after conventional medical treatment has failed.
Churg Strauss Syndrome is a systemic necrotising vasculitis affecting small and medium sized vessels. The neuropathy may occur in 60-70% of cases. In this study, we present a 60year-old female patient resistant to conventional immunosuppressants. Over the past year she has been followed with the diagnosis of astma bronchiale and she had purpuric lesions in both legs. Her test results showed eosinoplilia, Paranasal sinüs BT showed mucosal thickening in favor of chronic sinusitis, perivascular infiltration of eosinophils were present in the skin biopsy. Axonal sensorymotor polyneuropathy was determined in first electrophysiologic evaluation. Levels of total serum IgE were high. Although pulse of prednisolone 1gr/day treatment combined with 750 mg cyclophosphamide was given, muscle weakness progressed on right hand and left leg and she had remarkable atrophy of interosseous muscle of right hand. Nerve conduction studies revealed mononeuropathy multiplex ( asymmetric axonal sensorimotor neuropathy). The patient, who was considered refractory to cyclophosfamide therapy, has started using rituximab. Two months later, regeneration was observed in needle electromyography.In this case, we aim to provide the clinical , laboratory and electrophysiological findings with CSS who has responsed well to treatment with rituximab after conventional medical treatment has failed.
Açıklama
Anahtar Kelimeler
Nörolojik Bilimler
Kaynak
Journal of Neurological Sciences (Turkish)
WoS Q Değeri
Scopus Q Değeri
Cilt
33
Sayı
2
