RESUMEN
Creutzfeldt-Jakob disease (CJD) is the most common prion disease. It is characterized by neuronal loss, glial cell proliferation, and inflammatory reaction absence. It typically involves deep grey structures, including the caudate nucleus, putamen, and thalamus, with sparing of the hippocampus. Death usually occurs within one year of the onset of symptoms. A 59-year-old male presented to the outpatient department (OPD) with involuntary jerky movements of his right arm, progressive stiffness of the right half of his body, and slurring of speech for two months. His stiffness had led him to be completely bed-bound. He was admitted and during the hospital stay, he started showing cognitive decline. His MRI brain revealed a bright FLAIR signal in the left basal ganglia, claustrum, sub-, and peri-insular cortex extending into the left parietal parasagittal cortex. He was discharged with a probable diagnosis of CJD with advice to undergo a follow-up MRI brain after one month. He presented again to the hospital after four months with sepsis secondary to urinary tract infection, bedsores, and infected percutaneous endoscopic gastrostomy (PEG) site. His Glasgow Coma Scale (GCS) score on presentation was 8/15, with a fixed gaze and tonic posturing of upper and lower limbs. A follow-up MRI brain showed rapidly progressive cortical atrophy and communicating hydrocephalus consistent with CJD. The diagnosis of CJD requires the presence of clinical findings with a positive electroencephalogram (EEG), cerebrospinal fluid (CSF) findings, and neuroimaging, or pathological findings. In our patient, a diagnosis of probable CJD was made based on clinical symptoms and positive cortical ribboning on the MRI brain using the World Health Organization (WHO) criteria. EEG was nonspecific, and CSF tau proteins and brain biopsy could not be done.