Pellagra encephalopathy as a differential diagnosis for Creutzfeldt-Jakob disease

Istvan Kapas, Katalin Majtenyi, Klara Törö, Eva Keller, Till Voigtländer, Gabor G. Kovacs

Research output: Contribution to journalArticle

5 Citations (Scopus)


In the present study we evaluated cases referred as suspected Creutzfeldt-Jakob disease (CJD). Five out of 59 without prion disease showed neuropathological features of pellagra encephalopathy with widespread chromatolytic neurons (age range 40-48 years at death; one woman). These patients presentedwith a progressive neuropsychiatric disorder lasting for 2 to 24 months. Common symptoms included gait disorder, para- or tetraspasticity, extrapyramidal symptoms, incontinence, and myoclonus. Protein 14-3-3 in the cerebrospinal fluid was examined in a single patient and was positive, allowing the clinical classification as probable sporadic CJD. Pellagra encephalopathy may be considered as a differential diagnosis of CJD including detection of protein 14-3-3.

Original languageEnglish
Pages (from-to)231-235
Number of pages5
JournalMetabolic Brain Disease
Issue number2
Publication statusPublished - Jun 1 2012


  • Chromatolysis
  • Creutzfeldt-Jakob disease
  • Dementia
  • Pellagra
  • Prion
  • Surveillance

ASJC Scopus subject areas

  • Biochemistry
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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