| Summary: | Abstract Background Lentiform fork sign is a neuroradiological abnormality which is encountered in the clinical practice associated with uremic encephalopathy, dialysis disequilibrium syndrome and metabolic acidosis. Case presentation We describe here a case of this neuro-radiological abnormality which was encountered in a patient with uraemia and high anion gap metabolic acidosis who presented with generalised convulsion and later had some tremor in her hands. In our patient, there were few predisposing factors which might have possibly resulted in this abnormality chronic kidney disease, diabetes mellitus, and metabolic acidosis. Conclusion The Lentiform fork sign is a rare occurrence which can be related to a long list of toxic and metabolic causes but in conjunction with metabolic acidosis in chronic kidney disease patients, it can narrow down this list of alternate diagnosis.
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