Zagar Marija, Vranjes Davorka, Sostarko Marija, Vogrinc Zeljka, Bilić Ervina, Trbojević Cepe Milica
Department of Neurology, University Hospital Center Zagreb, Zagreb, Croatia.
Coll Antropol. 2009 Dec;33(4):1151-4.
In myasthenia gravis (MG) patients without detectable anti-acetylcholine receptor (anti-AChR) antibody, referred to as seronegative myasthenia gravis patients, there is a variable proportion of patients with antibodies against the muscle-specific kinase (MuSK). MuSK antibodies were found in 8 (29.6%) of our 27 patients with generalized MG without anti-AChR antibodies. All these patients were female. The age at the onset ranged from 22 to 38 years. All patients had ocular and bulbar symptoms, and two patients also had generalized limb weakness. Two patients had pure ocular symptoms for 7 or 8 years before the development of bulbar symptoms. All anti-MuSK positive patients were treated with immunosuppressive drugs, three received plasmapheresis and one patient required mechanical ventilation. Our results are consistent with other literature reports.
在重症肌无力(MG)患者中,若检测不到抗乙酰胆碱受体(anti-AChR)抗体,这类患者被称为血清阴性重症肌无力患者,其中有一定比例的患者存在抗肌肉特异性激酶(MuSK)抗体。在我们27例无anti-AChR抗体的全身型MG患者中,有8例(29.6%)检测到MuSK抗体。所有这些患者均为女性。发病年龄在22岁至38岁之间。所有患者均有眼肌和延髓症状,2例患者还伴有全身肢体无力。2例患者在出现延髓症状前有7年或8年的单纯眼肌症状。所有抗MuSK阳性患者均接受免疫抑制药物治疗,3例接受了血浆置换,1例患者需要机械通气。我们的结果与其他文献报道一致。