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Lentiform fork sign in uraemic striatopallidal syndrome
  1. Marta Sequeira1,
  2. Gonçalo Gama Lobo2,
  3. Margarida Ferro1,
  4. Marisa Brum1
  1. 1 Neurology Department, Centro Hospitalar de Lisboa Central EPE, Lisboa, Portugal
  2. 2 Neuroradiology Department, Centro Hospitalar de Lisboa Central EPE, Lisboa, Portugal
  1. Correspondence to Dr Marta Sequeira, Neurology Department, Centro Hospitalar de Lisboa Central EPE, Lisboa, Portugal; martasequeira117{at}

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A 77-year-old man developed sudden onset of gait impairment and slowness. He had type 2 diabetes mellitus and terminal chronic kidney disease, treated with haemodialysis; the symptoms had begun 1 week after a routine haemodialysis session. On examination, he had moderate symmetric akinetic–rigid parkinsonism with troublesome gait freezing; he could not walk unaided and required a wheelchair. Blood investigations were normal except for serum urea of 18 mmol/L and creatinine of 0.58 mmol/L. MR scan of brain showed bilateral, symmetrical hyperintensities in the lentiform nucleus on fluid attenuated inversion recovery and T2 sequences, outlined by a brighter hyperintense rim, suggesting the lentiform fork sign (figure 1). Given the absence of encephalopathy or other features suggesting uraemic encephalopathy, we diagnosed uraemic striatopallidal syndrome. After intensification of haemodialysis sessions and therapy with levodopa/carbidopa 100/25 …

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  • Contributors All the coauthors revised equally this paper and have participated in the diagnosis and treatment of the mentioned patient.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned. Externally peer reviewed by Simon Cronin, Cork, Republic of Ireland, and Paresh Malhotra, London, UK.

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