Age, specific pathology tied to shorter symptom duration in rapidly progressive dementia

CHICAGO — Older age at symptom onset and select pathologies were associated with shorter symptom duration in patients with rapidly progressive dementia, according to a poster at ANA 2022.

“We tried to find out why certain neurodegenerative diseases progress more rapidly compared with typical progression,” Evelyn Lazar, MD, a resident at JFK University Medical Center – Hackensack Meridian Health in New Jersey, said.

Lazar and colleagues sought to determine the neuropathological causes of rapidly progressive dementia within the Mayo Clinic neurodegenerative brain bank and identify clinically relevant factors that influence duration of disease.

They evaluated 8,586 brain samples provided from 1998 to 2020, as well as questionnaire results and relevant clinical records submitted by decedent families and health care providers. Researchers defined rapidly progressive dementia (RPD) as disease duration less than 4 years from symptom onset to death.

According to results, 306 samples met the criteria for RPD (median age at symptom onset, 70.8 years; median disease duration, 3.1 years; 39% women).

Further, corticobasal degeneration and progressive supranuclear palsy accounted for 142 cases, followed by Lewy body disease (n = 49), Alzheimer’s disease (n = 48), frontotemporal lobar degeneration (n = 32) and Creutzfeldt-Jakob disease (n = 30). Nearly 75% of patients with Creutzfeldt-Jakob disease presented with RPD (32 of 43).

Those with Creutzfeldt-Jakob disease and other causes had the shortest symptom duration, while no differences in duration were observed across other neurodegenerative diseases.

Data also showed that delusions and older age ( 75 years) at symptom onset were associated with shorter symptom duration, but co-pathologies — including vascular disease and other patient-specific factors — did not lead to shorter symptom duration.

“We still need to know what the cause of some neurodegenerative diseases is and how they progress,” Lazar said. “We need to look into the molecular pathology.”