Soufi Ghazaleh J, Tooyserkani Seyed H, Hekmatnia Ali, Norouzi Ali, Sadeghian Amirhossein, Riahi Farshad
Department of Radiology, Isfahan University of Medical Sciences, Isfahan, Iran.
School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
Radiol Case Rep. 2024 Jul 27;19(10):4346-4353. doi: 10.1016/j.radcr.2024.06.078. eCollection 2024 Oct.
Posterior reversible encephalopathy syndrome (PRES) is a neurological disease characterized by a variety of neurological findings, in accordance with radiological characteristics. PRES is commonly secondary to elevated BP and/or conditions such as autoimmune patients receiving immunosuppressive drugs. Our case involves a 36-year-old female with a history of autoimmune hepatitis (AIH), who presented with sudden onset headaches from 3 weeks prior, and a single episode of seizure attack the morning before admission. In the initial examination she had highly elevated blood pressure (BP) (190/116). Her neurological examination revealed decline in force of limbs in addition to mild paresthesia. After primary stabilization, she underwent brain magnetic resonance imaging. Due to the clinical and radiological findings, the patient was diagnosed with PRES. In the following work-up of BP elevation, abdominopelvic sonography and subsequent computed tomography scan, multiple lesions were observed in spleen and both kidneys consistent with infarction. In further evaluation, Lupus-like anticoagulants were found to be elevated, which, in conjunction with the confirmed antiphospholipid syndrome (APS), suggested a possible role for APS-nephropathy as the missing link between PRES and APS. However, despite the role of an autoimmune disease in increasing the risk of developing other autoimmune conditions, APS and AIH have been rarely observed together. Our study indicates that developing APS in the context of AIH is a rare occurrence. However, APS could serve as a critical intermediary, potentially facilitating the onset of PRES despite lower BP.
后部可逆性脑病综合征(PRES)是一种神经疾病,根据影像学特征,其具有多种神经学表现。PRES通常继发于血压升高和/或自身免疫性疾病患者接受免疫抑制药物等情况。我们的病例是一名36岁患有自身免疫性肝炎(AIH)的女性,她在入院前3周突然出现头痛,并在入院前一天早晨有一次癫痫发作。在初次检查时,她的血压(BP)极高(190/116)。她的神经学检查除了轻度感觉异常外,还显示肢体力量下降。在初步稳定病情后,她接受了脑部磁共振成像检查。根据临床和影像学检查结果,该患者被诊断为PRES。在随后对血压升高的检查中,进行了腹部盆腔超声检查及后续的计算机断层扫描,发现脾脏和双肾有多个符合梗死的病变。在进一步评估中,发现狼疮样抗凝物升高,结合已确诊的抗磷脂综合征(APS),提示APS肾病可能是PRES与APS之间缺失的环节。然而,尽管自身免疫性疾病在增加患其他自身免疫性疾病风险方面有作用,但APS和AIH很少同时被观察到。我们的研究表明,在AIH背景下发生APS是一种罕见情况。然而,APS可能是一个关键的中间因素,尽管血压较低,但可能促使PRES发病。