A Case Report of Probable Creutzfeldt-Jakob Disease Based on Positive MRI Findings and the World Health Organization Criteria.

Syed Asad Ali,Muhammad Tayyab Ilyas,Waseem T Malik,Aushna Rasool

doi:10.7759/cureus.10907

Syed Asad Ali, Muhammad Tayyab Ilyas + Show 2 more

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https://doi.org/10.7759/cureus.10907

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Journal: Cureus	Publication Date: Oct 12, 2020
License type: cc-by

Affiliation: Shifa International Hospital

Abstract

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.

Highlights

Creutzfeldt-Jakob disease (CJD) is the most common prion disease [1]; it is a fatal neurodegenerative disorder
Glial cell proliferation, and inflammatory response absence are some of the pathological features associated with the condition
We present a case of a probable CJD diagnosed on the basis of patient history, physical findings, and typical MRI brain findings, keeping in view the World Health Organization (WHO) diagnostic criteria for CJD

Summary

Introduction

Creutzfeldt-Jakob disease (CJD) is the most common prion disease [1]; it is a fatal neurodegenerative disorder. Its frequency had not improved despite treatment with multiple medications He had started to experience stiffness in his right upper and lower limb one month ago; initially, he had been up and about, but later, as the stiffness progressed, he had started using support and had gone on to become completely bed-bound. The patient was admitted with suspicion of vascular parkinsonism vs drug-induced extrapyramidal symptoms; despite stopping haloperidol, his stiffness did not improve, and he was given a trial of carbidopa/levodopa but continued having myoclonic jerks on the right side of the body, decreased comprehension, disturbed sleep pattern, and agitation He continued to have cognitive decline and decreased communication with other family members. His MRI brain showed an interval increase in previously noted T2 hyperintense signal foci in bilateral periventricular and subcortical white matter, communicating hydrocephalus, and rapidly progressive cortical atrophy consistent with CJD (Figure 3)

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