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.
This is a preview of subscription content, log in via an institution to check access.
References
Brown TM (2010) Pellagra: an old enemy of timeless importance. Psychosomatics 51:93–97
Chitravas N, Jung RS, Kofskey DM, Blevins JE, Gambetti P, Leigh RJ, Cohen ML (2011) Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease. Ann Neurol 70:437–444
Gelpi E, Heinzl H, Hoftberger R, Unterberger U, Strobel T, Voigtlander T, Drobna E, Jarius C, Lang S, Waldhor T, Bernheimer H, Budka H (2008) Creutzfeldt-Jakob disease in Austria: an autopsy-controlled study. Neuroepidemiology 30:215–221
Hauw JJ, De Baecque C, Hausser-Hauw C, Serdaru M (1988) Chromatolysis in alcoholic encephalopathies. Pellagra-like changes in 22 cases. Brain 111(Pt 4):843–857
Heinemann U, Krasnianski A, Meissner B, Varges D, Kallenberg K, Schulz-Schaeffer WJ, Steinhoff BJ, Grasbon-Frodl EM, Kretzschmar HA, Zerr I (2007) Creutzfeldt-Jakob disease in Germany: a prospective 12-year surveillance. Brain 130:1350–1359
Ishii N, Nishihara Y (1981) Pellagra among chronic alcoholics: clinical and pathological study of 20 necropsy cases. J Neurol Neurosurg Psychiatry 44:209–215
Ishii N, Nishihara Y (1985) Pellagra encephalopathy among tuberculous patients: its relation to isoniazid therapy. J Neurol Neurosurg Psychiatry 48:628–634
Josephs KA, Ahlskog JE, Parisi JE, Boeve BF, Crum BA, Giannini C, Petersen RC (2009) Rapidly progressive neurodegenerative dementias. Arch Neurol 66:201–207
Kovacs GG, Budka H (2009) Molecular pathology of human prion diseases. Int J Mol Sci 10:976–999
Kovacs GG, Budka H (2010) Current concepts of neuropathological diagnostics in practice: neurodegenerative diseases. Clin Neuropathol 29:271–288
Pfefferbaum A, Adalsteinsson E, Sullivan EV (2006) Dysmorphology and microstructural degradation of the corpus callosum: Interaction of age and alcoholism. Neurobiol Aging 27:994–1009
Quadrio I, Perret-Liaudet A, Kovacs GG (2011) Molecular diagnosis of human prion disease. Exp Op Med Diag 5:291–306
Serdaru M, Hausser-Hauw C, Laplane D, Buge A, Castaigne P, Goulon M, Lhermitte F, Hauw JJ (1988) The clinical spectrum of alcoholic pellagra encephalopathy. A retrospective analysis of 22 cases studied pathologically. Brain 111(Pt 4):829–842
Van Everbroeck B, Dobbeleir I, De Waele M, De Deyn P, Martin JJ, Cras P (2004) Differential diagnosis of 201 possible Creutzfeldt-Jakob disease patients. J Neurol 251:298–304
Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemann U, Breithaupt M, Varges D, Meissner B, Ladogana A, Schuur M, Haik S, Collins SJ, Jansen GH, Stokin GB, Pimentel J, Hewer E, Collie D, Smith P, Roberts H, Brandel JP, van Duijn C, Pocchiari M, Begue C, Cras P, Will RG, Sanchez-Juan P (2009) Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 132:2659–2668
Acknowledgments
This study was performed in the frame of the Hungarian CJD Surveillance and supported partly by the Project TÁMOP-4-2-1/B-03/1/KMR-2010-001. Assistance of Marianna Lenkeine and Katalin Ress is also acknowledged. We are grateful for the collegues for referring the cases and for the relatives of the patients.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kapas, I., Majtenyi, K., Törö, K. et al. Pellagra encephalopathy as a differential diagnosis for Creutzfeldt-Jakob disease. Metab Brain Dis 27, 231–235 (2012). https://doi.org/10.1007/s11011-012-9308-8
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11011-012-9308-8