INTRODUCTION: Prion diseases are protein conformation disorders and neither 
caused by viroid or virus but is a transmissible particle labeled a prion by 
Pruisner. Normal prion protein becomes infectious by a different folding, but 
the triggers are not known. Based on the characteristic brain pathology, they 
are grouped under spongiform encephalopathy affecting both man and animals. 
Estimated prevalence is one per million. Creutzfeldt-Jakob disease (CJD) 
registry from National Institute and Neurosciences (NIMHANS), Bengaluru, 
reported 69 cases in 30 years.
PATIENT AND METHODS: Patients seen by our team from December 2011 to October 
2015 who satisfied criteria for probable CJD were evaluated for clinical, 
electrophysiological, radiological, and demographic factors. None of them 
underwent histopathological examination of brain tissue or tonsils. 
Cerebrospinal fluid protein 14-3-3 was not done. All of them were followed up by 
telephonic inquiry for the course of the illness. All of them received 
symptomatic medications with anticonvulsants, flupirtine 200 mg orally daily, 
and other symptomatic medications.
RESULTS: Sporadic CJD is the most common form seen in India and is probably 
under reported. males seem to be more affected, and the mean duration for the 
bed bound state is 12 months. Drugs were only effective for a very brief period 
in controlling myoclonus and behavior.
DISCUSSION: Sporadic CJD is one of the most common and rapidly fatal forms of 
dementia in India. Cortical ribboning and periodic complexes are the most common 
laboratory findings. Familial CJD is a very rare occurrence and variant CJD is 
probably not prevalent.
CONCLUSION: All patients with rapidly progressive dementia should be handled 
with biohazard precautions unless proved otherwise. Role of alcohol and smoking 
in the transformation of PrPc to PrPsc needs to be evaluated.