Case series of probable sporadic Creutzfeldt-Jakob disease from Eastern India

Background: Creutzfeldt-Jakob disease is a rapidly progressive, fatal, transmissible neurodegenerative disorder caused by prion protein. It is still considered rare in countries like India. This is probably due to nonavailability of autopsy studies in majority of the center. The recent European diag...

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Main Authors: Atanu Biswas, Namrata Khandelwal, Alak Pandit, Arijit Roy, Deb Sankar Guin, Goutam Gangopadhyay, Asit Senapati
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2013-01-01
Series:Annals of Indian Academy of Neurology
Subjects:
Online Access:http://www.annalsofian.org/article.asp?issn=0972-2327;year=2013;volume=16;issue=4;spage=659;epage=663;aulast=Biswas
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author Atanu Biswas
Namrata Khandelwal
Alak Pandit
Arijit Roy
Deb Sankar Guin
Goutam Gangopadhyay
Asit Senapati
author_facet Atanu Biswas
Namrata Khandelwal
Alak Pandit
Arijit Roy
Deb Sankar Guin
Goutam Gangopadhyay
Asit Senapati
author_sort Atanu Biswas
collection DOAJ
description Background: Creutzfeldt-Jakob disease is a rapidly progressive, fatal, transmissible neurodegenerative disorder caused by prion protein. It is still considered rare in countries like India. This is probably due to nonavailability of autopsy studies in majority of the center. The recent European diagnostic criterion for sporadic CJD (sCJD) is useful for making an early diagnosis. Objective: To report a series of patients of probable sCJD from a neurology institute of eastern India. Materials and Methods: Patients of rapidly developing dementia fulfilling the diagnostic criteria for sCJD were included. All were investigated in detail to find out any possible treatable cause including electroencephalography (EEG), magnetic resonance imaging (MRI) of brain, and cerebrospinal fluid analysis. Results: A total 10 patients of probable sCJD diagnosed using the European diagnostic criterion between December 2011 and January 2013. The clinical features are consistent with other reported series. While 60% of patients had the classical EEG findings, 100% had typical MRI features. Eight patients died within a mean duration of 4.56 months from the disease onset. Conclusions: The clinical features are similar to other reported series. Our observation raises question about the prevalence of this disease in India which needs more elaborate studies.
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spelling doaj.art-360fbcf9c68f49f1836e3879357cefcf2022-12-21T18:50:13ZengWolters Kluwer Medknow PublicationsAnnals of Indian Academy of Neurology0972-23271998-35492013-01-0116465966310.4103/0972-2327.120486Case series of probable sporadic Creutzfeldt-Jakob disease from Eastern IndiaAtanu BiswasNamrata KhandelwalAlak PanditArijit RoyDeb Sankar GuinGoutam GangopadhyayAsit SenapatiBackground: Creutzfeldt-Jakob disease is a rapidly progressive, fatal, transmissible neurodegenerative disorder caused by prion protein. It is still considered rare in countries like India. This is probably due to nonavailability of autopsy studies in majority of the center. The recent European diagnostic criterion for sporadic CJD (sCJD) is useful for making an early diagnosis. Objective: To report a series of patients of probable sCJD from a neurology institute of eastern India. Materials and Methods: Patients of rapidly developing dementia fulfilling the diagnostic criteria for sCJD were included. All were investigated in detail to find out any possible treatable cause including electroencephalography (EEG), magnetic resonance imaging (MRI) of brain, and cerebrospinal fluid analysis. Results: A total 10 patients of probable sCJD diagnosed using the European diagnostic criterion between December 2011 and January 2013. The clinical features are consistent with other reported series. While 60% of patients had the classical EEG findings, 100% had typical MRI features. Eight patients died within a mean duration of 4.56 months from the disease onset. Conclusions: The clinical features are similar to other reported series. Our observation raises question about the prevalence of this disease in India which needs more elaborate studies.http://www.annalsofian.org/article.asp?issn=0972-2327;year=2013;volume=16;issue=4;spage=659;epage=663;aulast=BiswasCreutzfeldt-Jakob diseaseelectroencephalographymagnetic resonance imaging
spellingShingle Atanu Biswas
Namrata Khandelwal
Alak Pandit
Arijit Roy
Deb Sankar Guin
Goutam Gangopadhyay
Asit Senapati
Case series of probable sporadic Creutzfeldt-Jakob disease from Eastern India
Annals of Indian Academy of Neurology
Creutzfeldt-Jakob disease
electroencephalography
magnetic resonance imaging
title Case series of probable sporadic Creutzfeldt-Jakob disease from Eastern India
title_full Case series of probable sporadic Creutzfeldt-Jakob disease from Eastern India
title_fullStr Case series of probable sporadic Creutzfeldt-Jakob disease from Eastern India
title_full_unstemmed Case series of probable sporadic Creutzfeldt-Jakob disease from Eastern India
title_short Case series of probable sporadic Creutzfeldt-Jakob disease from Eastern India
title_sort case series of probable sporadic creutzfeldt jakob disease from eastern india
topic Creutzfeldt-Jakob disease
electroencephalography
magnetic resonance imaging
url http://www.annalsofian.org/article.asp?issn=0972-2327;year=2013;volume=16;issue=4;spage=659;epage=663;aulast=Biswas
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