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

Abstract

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.

A study of clinical profile, radiological and electroencephalographic characteristics of suspected Creutzfeldt-Jakob disease in a tertiary care centre in South India

“…Six patients had diffusion restriction in parieto-occipital and two patients in frontal region. [ 20 ]…”

Alien hand and leg as the presenting feature of probable sporadic Creutzfeldt-Jakob disease: A rare presentation of a rare disease

“…In a series of 10 patients of probable sCJD from east India by Atanu Biswas et al .,[ 5 ] the most common presentation was psychosis, ataxia and visual complaints. None of their patients developed alien hand phenomenon as the initial presentation or any time during the course of the disease.…”

“…sCJD is a rare disease and few case series are reported from India. [ 5 ] We present a case of alien hand and leg as the presenting feature of probable sCJD. This is to our knowledge the first case report of this rare presentation of probable sCJD from our region.…”

Scientific Opinion on a request for a review of a scientific publication concerning the zoonotic potential of ovine scrapie prions

“…It is of note that Australia has never reported a case of Classical scrapie. Since 2011, case reports or case series on ssCJD have also been published from China (Gao et al, 2011), Chile (Cartier et al, 2012, Hawaii (Kojima et al, 2013), Korea , India (Biswas et al, 2013), Morocco (Hajjaj and Kissani, 2011), Pakistan (Ahmad et al, 2014), Peru (Torres-Ramirez et al, 2014), Mexico (Moreno et al, 2013), and Turkey (Atalay et al, 2013). This evidence is consistent with the observation that sCJD occurs worldwide and the consistency of mortality rates in systematic surveys suggest that, should there be an environmental risk factor for the development of disease, this must be ubiquitous and evenly distributed.…”