Pellagra encephalopathy as a differential diagnosis for Creutzfeldt-Jakob disease.

Abstract

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 presented with 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.

• Authors: Istvan Kapas, Katalin Majtenyi, Klara Törö, Eva Keller, Till Voigtländer, Gabor G Kovacs
• Journal: Metabolic brain disease (Metab Brain Dis) Vol. 27 Issue 2 Pg. 231-5 (Jun 2012) ISSN: 1573-7365 [Electronic] United States
• PMID: 22535301 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• 14-3-3 Proteins

Topics

• 14-3-3 Proteins (cerebrospinal fluid)
• Adult
• Aged
• Aged, 80 and over
• Brain (pathology)
• Creutzfeldt-Jakob Syndrome (diagnosis, pathology, psychology)
• Dementia (etiology, psychology)
• Diagnosis, Differential
• Disease Progression
• Electroencephalography
• Female
• Humans
• Immunohistochemistry
• Male
• Middle Aged
• Pellagra (diagnosis, pathology, psychology)
• Retrospective Studies