title: 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.affiliation: Neuropathology and Prion Disease Reference Center, Semmelweis University, Budapest, Hungary.
authors.forename: Istvan
authors.initials: I
authors.lastname: Kapas
authors.forename: Katalin
authors.initials: K
authors.lastname: Majtenyi
authors.forename: Klara
authors.initials: K
authors.lastname: Törö
authors.forename: Eva
authors.initials: E
authors.lastname: Keller
authors.forename: Till
authors.initials: T
authors.lastname: Voigtländer
authors.forename: Gabor G
authors.initials: GG
authors.lastname: Kovacs
mesh_terms: D048948:14-3-3 Proteins / Q000134:cerebrospinal fluid; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000473:pathology / Q000523:psychology; D003704:Dementia / Q000209:etiology / Q000523:psychology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010383:Pellagra / Q000175:diagnosis* / Q000473:pathology / Q000523:psychology; D012189:Retrospective Studies
chemical_list: D048948:14-3-3 Proteins