Disclosures: Anita M. Lowe Taylor, MD: Nothing to disclose

Case Description: The patient initially presented with one month of disorientation, confusion, and poor attention, prompting admission to a medicine service. Brain MRI showed right parietal cortical ribboning. Lumbar puncture was bland. The patient’s immunosuppressant was discontinued out of suspicion for encephalitis. She was started on antiepileptics due to concern for seizures on EEG. The patient was discharged home and re-admitted twelve days later with worsening symptoms. Repeat MRI showed rapid progression of cortical ribboning and she was treated with empiric intravenous immunoglobulin for possible autoimmune encephalitis without clinical improvement. Five days later, she was transferred to an acute rehabilitation unit with a preliminary diagnosis of autoimmune encephalitis versus Creutzfeldt Jacob Disease (CJD). On admission to rehab, her primary impairments were in the domains of visuospatial, executive function, language, short-term memory, and attention. She continued to decline in these domains, which limited her functional progress in therapies. Toxic metabolic etiologies were ruled out. Eventually her cerebrospinal fluid labs, including 14-3-3 protein and real-time quaking-induced conversion (a test for sporadic CJD), resulted and were consistent with CJD.

Setting: Acute inpatient rehabilitation center

Patient: A 65-year-old woman with history of rheumatoid arthritis previously on immunosuppression.

Assessment/Results: Given the poor prognosis, palliative care was involved and goals of care were discussed with the patient’s family. She was discharged home with hospice and died two weeks later.

Discussion: CJD is a rapidly progressive neurodegenerative disease with a 100% mortality rate. To our knowledge, there are only two other case reports of CJD diagnosed in the rehab setting, both of which had a distinctly different presentation than the patient presented here.

Conclusion: Physiatrists must consider a diagnosis of CJD when presented with rapidly progressive cognitive deterioration in the rehab setting. The physiatrist should address goals of care and involve palliative care services.

Level of Evidence: Level IV

« Back to AAPM&R Annual Assembly 2019

PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/rapidly-progressive-creutzfeldt-jacob-disease-in-the-acute-rehab-setting-a-case-report/