Patient is in chronic renal failure and on dialysis. Presented with seizures.
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- Evidence of bilateral T2/FLAIR hyperintensities involving the caudate, putamen, midbrain, and mesial temporal lobes.
- Positive lentiform fork sign is seen represented by edematous internal and external capsules delineating the putamina.
- Edematous medullary laminae are seen delineating the three nuclear components of the lentiform nucleus.
The subcortical gray matter is known to be exquisitely sensitive to a range of toxins and metabolic disturbances 1. Uremic encephalopathy (UE) is an acquired toxic syndrome related to kidney dysfunction. Whereas the literature concerning encephalopathy associated with liver disease is rich and multifaceted, there are very few studies dealing with this aspect of renal failure, thus little is known of the pathophysiology of UE. The complexity of normal kidney function however, makes it likely that renal failure leads to a variety of abnormalities that exert synergistic deleterious effects on the brain 2. Metabolic acidosis has been proposed as a possible mechanism for UE, which is associated with the lentiform fork sign on MRI 3.
- 1. Albin RL. Basal ganglia neurotoxins. Neurol Clin. 2000;18 (3): 665-80. Pubmed citation
- 2. Daroff RB, Fenichel GM, Jankovic J et-al. Bradley's Neurology in Clinical Practice: Expert Consult - Online and Print, 6e. Saunders. ISBN:1437704344. Read it at Google Books - Find it at Amazon
- 3. Kumar G, Goyal MK. Lentiform Fork sign: a unique MRI picture. Is metabolic acidosis responsible?. Clin Neurol Neurosurg. 2010;112 (9): 805-12. doi:10.1016/j.clineuro.2010.06.006 - Pubmed citation