SHM Converge Scientific Abstract ePoster Gallery-Creutzfeldt-Jakob Disease for the Hospitalist: A Multidisciplinary Approach to a Rare Presentation

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This case describes a 67-year-old man with a one-month history of rapidly worsening ataxia, visual changes, myoclonus, confusion, and encephalopathy. His symptoms evolved quickly from subtle imbalance and diplopia to word-finding difficulty, facial recognition problems, agitation, reduced responsiveness, and loss of oral intake. After multiple outpatient and emergency evaluations, neurology became concerned for rapidly progressive dementia, and the work-up included MRI brain, lumbar puncture, CT chest/abdomen/pelvis, and EEG. MRI showed cortical ribboning, EEG showed periodic discharges and slowing, and CSF RT-QuIC confirmed Creutzfeldt-Jakob Disease (CJD). He was discharged to skilled nursing with hospice.<br /><br />The presentation highlights that CJD is a prototypical rapidly progressive dementia, but clinicians must first consider reversible mimics such as autoimmune, infectious, metabolic, and neoplastic causes, as well as delirium. Early neurology consultation is recommended when symptoms suggest RPD/CJD.<br /><br />The case also emphasizes the importance of early interdisciplinary care. Palliative care was consulted during hospitalization, underscoring that supportive care is appropriate nearly universally in CJD but is often underused. Early involvement can help with symptom support, anticipatory guidance, disclosure of diagnosis and prognosis, and caregiver self-care.<br /><br />From an infection control standpoint, the CDC recommends standard precautions for routine care, with special handling needed for tissue, autopsies, and surgical instrument reprocessing.<br /><br />Overall, clinicians should recognize that patients with CJD often present with a combination of cognitive decline, gait disturbance, behavioral changes, and visual symptoms, and that obtaining a detailed history is essential to identify rapidly progressive dementia early and connect patients and families with appropriate resources.

Asset Subtitle

Sydney Erickson

Meta Tag

Author List Bonnie Cruser MD, Elio Souheil Regheb, Kyle James, Michael Wayne McGrath, Olivia Groover, Stephen Haff, Sydney Erickson

Category Clinical Vignettes

Concept Sporadic Creutzfeldt-Jakob Disease

Concept Rapidly Progressive Dementia

Concept Cognitive Decline

Concept RT-QuIC

Concept Ataxia

Distinguished Non-Finalist

Presenter Organization Emory University School of Medicine

Presenting Author Sydney Erickson

Track Adult

Keywords

Creutzfeldt-Jakob disease

rapidly progressive dementia

ataxia

myoclonus

encephalopathy

cortical ribboning

RT-QuIC

periodic discharges

palliative care

infection control

Sporadic Creutzfeldt-Jakob Disease

Rapidly Progressive Dementia

Cognitive Decline

RT-QuIC

Ataxia