Benbrahim Fatima Zohra, Belkouchi Lina, Allali Nazik, El Haddad Siham, Chat Latifa
Department of Radiology, Children hospital of Rabat, Ibn Sina University Hospital, Mohammed V University, Rabat, Morocco.
Radiol Case Rep. 2024 Aug 24;19(11):5191-5195. doi: 10.1016/j.radcr.2024.07.151. eCollection 2024 Nov.
Susac syndrome is a rare microangiopathy of unclear etiology, likely autoimmune, characterized by a characteristic clinical triad of encephalopathy, retinopathy, and hypoacusis. The majority of cases reported in the literature involve adult patients, with its occurrence in the pediatric population being extremely rare. Magnetic resonance imaging (MRI) is essential for diagnosis and patient monitoring, revealing nearly pathognomonic features, particularly valuable given the typically incomplete clinical triad and the consistent presence of encephalopathy, often as the initial symptom. We report the case of an 11-year-old child diagnosed with Susac syndrome, highlighting the importance of considering this diagnosis in cases of childhood encephalopathy and initiating treatment as early as possible to prevent irreversible sequelae.
Susac综合征是一种病因不明的罕见微血管病,可能为自身免疫性疾病,其特征为脑病、视网膜病和听力减退这一典型临床三联征。文献报道的大多数病例为成年患者,在儿科人群中极为罕见。磁共振成像(MRI)对于诊断和患者监测至关重要,可显示几乎具有诊断意义的特征,鉴于典型的临床三联征往往不完整且脑病通常作为首发症状持续存在,这一特征尤为有价值。我们报告一例11岁儿童被诊断为Susac综合征的病例,强调在儿童脑病病例中考虑这一诊断并尽早开始治疗以预防不可逆后遗症的重要性。