Lentiform fork sign in a uremic patient with a high anion gap metabolic acidosis with seizures: a case report from North West of Ireland

Salman Mansoor,Barry Coffey,James Lineen,Lize De Klerk,Imran Ali,Kevin Murphy,Muhammad Fahad,Monica Ann Mulry,Shoab Saadat,Siobhan Kelly,Mohammad Hijaz Adenan

doi:10.1186/s41983-020-00234-8

Abstract

BackgroundLentiform fork sign is a neuroradiological abnormality which is encountered in the clinical practice associated with uremic encephalopathy, dialysis disequilibrium syndrome and metabolic acidosis.Case presentationWe 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.ConclusionThe 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.

Highlights

Lentiform fork sign is a neuroradiological abnormality which is encountered in the clinical practice associated with uremic encephalopathy, dialysis disequilibrium syndrome and metabolic acidosis
Lentiform fork sign is a neuroradiological abnormality which can be associated with uremic encephalopathy, dialysis disequilibrium syndrome and metabolic acidosis [3,4,5]
We describe here a case of this neuro-radiological abnormality which was encountered in our patient with Uraemia and high anion gap metabolic acidosis who presented with a generalised convulsion and later had some tremor in her hands

Summary

Background

Basal ganglia are metabolically highly active deep grey matter structures. There are various toxic and metabolic insults (uraemia, hypoxia, drug overdose, infections) that result in abnormalities in basal ganglia on neuroimaging [1,2,3]. We describe here a case of this neuro-radiological abnormality which was encountered in our patient with Uraemia and high anion gap metabolic acidosis who presented with a generalised convulsion and later had some tremor in her hands. Case presentation A 74-year-old lady with known CKD stage 3b was brought by ambulance to the emergency department after a first prolonged generalised tonic clonic seizure that lasted 90 minutes On arrival she had a low Glasgow Coma Score (GCS) of 3/15. Her family reported her being fatigued over the previous week Her past medical history included hypertension, type 2 diabetes mellitus, osteoporosis, chronic obstructive pulmonary disease, anxiety disorder and benign paroxysmal positional vertigo. Investigations Laboratory investigations on admission (Table 1) showed an acute kidney injury, with associated high anion gap metabolic acidosis.

Result

Discussion