• Hui Li and Wenbiao Chen.
• Department of Nephrology, Shenzhen Baoxing Hospital, Shenzhen, China.
• Medicine (Baltimore). 2023 Aug 25; 102 (34): e34819e34819.

IntroductionThe 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.Patient ConcernsWe 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.DiagnosisBased on the clinical manifestations, past medical history and MRI imaging changes of the 3 cases reported here, the diagnosis of LFS was established.InterventionsOur experience from these 3 cases suggests that hormone supplementation and gamma globulin therapy may be indicated for treating LFS.LessonsOur 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.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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