Department of Nephrology, Shenzhen Baoxing Hospital, Shenzhen, China.
Central laboratory, People's Hospital of Longhua, The Affiliated Hospital of Southern Medical University, Shenzhen, China.
Medicine (Baltimore). 2023 Aug 25;102(34):e34819. doi: 10.1097/MD.0000000000034819.
The lentiform fork sign (LFS) is a unique magnetic resonance imaging (MRI) finding characterized by a bright hyperintense rim delineating the lentiform nucleus as a fork associated with metabolic acidosis in end-stage renal disease.
We report 3 cases of LFS in diabetic uremic patients. In one case of uremia, intensive hemodialysis treatment was not effective. Given our poor understanding of LFS, it was regarded as bilateral basal ganglia pathology, and pulse hormone and gamma globulins therapy was initiated. The patient neurological symptoms improved, and the pathological signs on imaging subsided. Based on our experience with the first LFS case, 2 diabetic uremic cases presenting with LFS were successfully treated with hormone or gamma globulin pulse therapy in addition to intensive hemodialysis.
Based on the clinical manifestations, past medical history and MRI imaging changes of the 3 cases reported here, the diagnosis of LFS was established.
Our experience from these 3 cases suggests that hormone supplementation and gamma globulin therapy may be indicated for treating LFS.
Our findings highlight that in diabetic uremic dialysis patients with neurological symptoms, LFS should be suspected. The clinical manifestations, past medical history and MRI imaging findings are essential for diagnosing LFS. Hormone supplementation and gamma globulin therapy may be the effective treatment for LFS.
豆状核叉征(LFS)是一种独特的磁共振成像(MRI)表现,其特征为亮高信号边缘勾勒出豆状核呈叉状,与终末期肾病代谢性酸中毒有关。
我们报告了 3 例糖尿病尿毒症患者的 LFS 病例。在一例尿毒症患者中,强化血液透析治疗无效。由于我们对 LFS 的了解有限,将其视为双侧基底节病变,并开始使用激素和丙种球蛋白治疗。患者的神经症状改善,影像学上的病理征象消退。基于我们对首例 LFS 病例的经验,对 2 例糖尿病尿毒症伴 LFS 的患者除了强化血液透析外,还采用激素或丙种球蛋白脉冲治疗取得了成功。
根据这 3 例报告的临床表现、既往病史和 MRI 影像学改变,诊断为 LFS。
我们的经验表明,激素补充和丙种球蛋白治疗可能对 LFS 有治疗作用。
我们的发现强调,在有神经症状的糖尿病尿毒症透析患者中,应怀疑 LFS。临床表现、既往病史和 MRI 影像学表现对于诊断 LFS 至关重要。激素补充和丙种球蛋白治疗可能是 LFS 的有效治疗方法。