Betend Romain, Humm Andrea M, Medlin Friedrich
Department of Internal Medicine, Unit of Neurology, HFR Fribourg Hopital Cantonal, Fribourg, Switzerland.
Department of Neurology, Inselspital Universitatsspital Bern, Bern, Switzerland.
BMJ Case Rep. 2017 Aug 7;2017:bcr-2017-220494. doi: 10.1136/bcr-2017-220494.
A 67-year-old patient, only known for bilateral presbycusis, presented with subacute onset of delirium. Clinical examination showed multifocal neurological deficits, all together suggesting subcortical frontal dysfunction together with cerebellar and corpus callosum involvement.Cerebral MRI demonstrated supratentorial and infratentorial subcortical and periventricular T2-hyperintense lesions with cerebellar gadolinium enhancement and multiple central lesions of the corpus callosum (snowball lesions). The diagnosis of Susac syndrome was made and the patient treated with intravenous methylprednisolone, followed by a prednisone maintenance dose over 8 weeks. After a clinical improvement, a relapse was noticed during corticosteroid tapering. The patient was again treated with intravenous methylprednisolone followed by a prednisone maintenance therapy with simultaneous introduction of mycophenolate mofetil acid and one cycle of intravenous rituximab. The patient recovered rapidly. At 11-month follow-up, only mild executive dysfunction and persistent right postural tremor was noted, MRI showed partial regression of subcortical and juxtacortical lesions.
一名67岁患者，仅患有双侧老年性耳聋，出现谵妄亚急性发作。临床检查显示多灶性神经功能缺损，所有这些提示皮质下额叶功能障碍以及小脑和胼胝体受累。脑部磁共振成像（MRI）显示幕上和幕下皮质下及脑室周围T2高信号病变，小脑钆增强，以及胼胝体多发中央病变（雪球样病变）。诊断为Susac综合征，患者接受静脉注射甲泼尼龙治疗，随后8周给予泼尼松维持剂量。临床改善后，在糖皮质激素减量过程中出现复发。患者再次接受静脉注射甲泼尼龙治疗，随后进行泼尼松维持治疗，同时引入霉酚酸酯并进行一个周期的静脉注射利妥昔单抗治疗。患者迅速康复。在11个月的随访中，仅发现轻度执行功能障碍和持续的右侧姿势性震颤，MRI显示皮质下和皮质旁病变部分消退。
