Welcome to the WSC 2022 Interactive Program

Background and Aims

Case Report

Methods

64years old lady was admitted with progressive right sided weakness over few months, unable to hold things, walking very slow, holding on to things to maintain her balance. Patient’s speech was deteriorating. She had good comprehension, she used texting and writing to communicate, cognition was normal. Family noticed that patient was having repeated jerky movements in her legs. 3 months prior to the CJD diagnosis patient presented to stroke unit with expressive dysphasia and initially treated as strokediagnosed as stroke. In her past medical history patient had submandibular gland excision 2019, hypothyroidism

Patient has been vegetarian most of her life and had not consumed red meat for 30 years.

On examination patient was aphasic, withdrawn was not making any eye contact. Myoclonic jerks were present which increased with stimulus. Patient’s mobility was reduced, she remained in bed and had reduced eating and drinking.

Results

Patient had MRI head which showed cortical ribbon sign in left frontal and temporal region and left basal ganglia involvement. CSF sample was positive for 14 3 3, RT-QuIC positive

Patient was reviewed by National CJD centre and the diagnosis of sporadic CJD was confirmed. Prognosis was less than 6 months

Left temporal and insular cortical ribbon sign

Conclusions

As in patient she was deteriorating very rapidly, she was eating and drinking very minimal amounts and remained in bed most of the time. Patient was discharged home for end of life care and she passed away at home with her family around.