Unintended findings: Therapeutic effects of hormones or gamma globulins on Lentiform Fork sign in 3 diabetic uremic patients: Case report/case series.

Abstract

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.