An 88-year-old diabetic man, with end-stage chronic kidney disease, on dialysis, presented
with nausea and diarrhea for 2 days. After this period, the patient's family sought
emergency care due to a significant and acute reduction in movement, dysphonia, and
dysphagia. His physical examination was consistent with akinetic-rigid parkinsonian
syndrome. A brain magnetic resonance imaging scan was performed, and the lentiform
fork sign was identified ([Figure 1 ]), which, associated with metabolic acidosis, corroborated the diagnosis of uremic
striatopallidal syndrome.
Figure 1 Head computed tomography (CT) scan showing symmetrical hypodensity in the lentiform
nuclei bilaterally (A ). Brain magnetic resonance imaging (MRI) scan in fluid-attenuated inversion recovery
(FLAIR) (B ) and T2-weighted (C ) sequences demonstrateing hyperintensity in the lentiform nuclei bilaterally. The
white arrows show T2 and FLAIR hyperintensity in the white matter surrounding the
lentiform, representing edema in the internal and external capsules. This finding
forms the basis of the fork of the “lentiform fork sign”. Facilitated diffusion can
be seen on the diffusion-weighted imaging (DWI) (D ) and apparent diffusion coefficient (ADC) (E ) sequences. Illustrative representation of a fork (F ).
Uremic striatopallidal syndrome is a rare cerebral complication of metabolic acidosis.
It may present in the form of chorea or, more commonly, acute parkinsonism.[1 ]
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