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Abstract Details

PET Imaging in Rapidly Progressive Neurodegeneration – an Underutilised Resource?
General Neurology
P13 - Poster Session 13 (5:30 PM-6:30 PM)
6-008
To highlight PET imaging as a useful diagnostic aid in the evaluation of patients with rapidly progressive neurodegenerative disease.
Creutzfeldt-Jakob Disease (CJD) is the most common type of human prion disease. The majority are sporadic (sCJD). Death usually occurs within 1 year (median 6 months). Definitive diagnosis is made on neuropathological examination.

We report a case of a 77 year old man with a 2 month history of mobility decline. There were pyrimadal and cerebellar features on examination. Cognition was initially spared. His condition rapidly progressed, with the development of myoclonus and eventually cognitive decline.

Routine blood work-up, CSF analysis and MRI Brain was unremarkable. The first EEG recording showed non-specific bilateral cerebral dysfunction. Cerebral PET imaging showed hypermetabolism within the periventricular white matter, temporal lobes and brainstem. Repeat EEG showed a more pronounced bilateral cerebral dysfunction with periodic sharp wave complexes, indicating a marked encephalopathy and compatible with a form of sCJD. CSF 14-3-3, S-100b and RT-QuIC came back positive, and postmortem examination confirmed sCJD.
Currently, PET imaging is not routinely used in the diagnostic evaluation of patients with prion disease. Typically, there is hypometabolism in the parietal region which can also be seen in the temporal lobe and the cerebellum. When FDG hypometabolism is compared with neuropathological changes, it was predictive of neuropathology in 80.6% of cortical regions. This emphasises its potential as a diagnostic aid in patients with rapidly progressive neurodegeneration. However, as it is underused at present, there are limited publications on PET imaging in prion disease. This case highlights new radiological features of sCJD compared to what is reported to date.
Authors/Disclosures
Michael Doyle, MRCPI
PRESENTER 		No disclosure on file
Sean Connolly, MD, MRCPI (St. Vincent's University Hospital) Dr. Connolly has received publishing royalties from a publication relating to health care.
No disclosure on file
Niall Tubridy, MD (St Vincent's University Hospital) Dr. Tubridy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis .