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A Case of Creutzfeldt-Jakob Disease Suspected to be Acquired from Gelatin Capsules (P1-9.006)
Gaurav Kathuria, Shimron Brown, Sean Kenniff
Neurology May 2022, 98 (18 Supplement) 3654;
Abstract
Objective: Present a unique case of Creutzfeldt-Jakob Disease (CJD) suspected to be acquired from gelatin capsules.
Background: CJD is a rare human prion neurodegenerative disease. Variant CJD, an acquired subtype, is even less common. Variant CJD has been linked to bovine spongiform encephalopathy (BSE), a prion disease in cattle. Although most cases have been linked to the ingestion of contaminated beef, bovine products are commonly consumed in other ways. Gelatin is often derived from bovine collagen. Gelatin is commonly used as a food ingredient, as well as in capsules for medications and supplements.
Design/Methods: N/A
Results: A 62 year old male with past medical history of paroxysmal atrial fibrillation and hypertension presented with dysarthria and right-sided ataxia. His ataxia rapidly worsened and soon involved all extremities. The MRI brain showed diffusion-weighted imaging (DWI) hyperintensities in the cortical ribbon of the parietal lobes, which was suggestive of CJD. Real-time quaking-induced conversion (RT-QuIC) in the cerebral spinal fluid (CSF) was positive, which suggested an over 98% likelihood of prion disease. During an extensive work up to rule out differential diagnoses, it was found he was ingesting large quantities of vitamins and supplements daily for decades, many of which were encased in gelatin capsules. He rapidly declined and became globally aphasic, eventually passing shortly after.
Conclusions: The link between variant CJD and BSE has been documented multiple times in the past. Although BSE contamination in gelatin is theoretical, there is a high likelihood in this case. The large amounts of gelatin consumed by this patient on a chronic daily basis does not appear to be a coincidence. There are numerous documented cases of gelatin being suspected as the source of BSE contamination. There is a need for more organized data collection, as well as testing of suspected gelatin products.
Disclosure: Dr. Kathuria has nothing to disclose. Mr. Brown has nothing to disclose. Dr. Kenniff has nothing to disclose.