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Rapidly progressive neurodegenerative dementias
Keith A Josephs1, J Eric Ahlskog, Joseph E Parisi
1Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA. josephs.keith@mayo.edu
Archives of Neurology
|February 11, 2009
Summary
Rapidly progressive dementia is uncommon but can be caused by various neurodegenerative diseases. Creutzfeldt-Jakob disease (CJD) is a common cause, but other conditions like frontotemporal lobar degeneration and diffuse Lewy body disease also present rapidly.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Neurodegenerative dementias typically present with slow progression.
- Rapidly progressive dementia is a less common but critical clinical presentation.
Purpose of the Study:
- To characterize patients with neurodegenerative diseases exhibiting a rapidly progressive course.
- To identify the underlying pathologies and clinical trajectories of rapidly progressive dementia.
Main Methods:
- Retrospective review of medical records using keyword searches for "rapid" and "dementia".
- Inclusion criteria: patients with brain autopsy, neurodegenerative disease diagnosis, and disease duration under 4 years.
- Analysis of 22 identified cases from a tertiary care medical center.
Main Results:
- Creutzfeldt-Jakob disease (CJD) accounted for 36% of cases, with rapid progression (≤12 months).
- Other diagnoses included frontotemporal lobar degeneration with motor neuron degeneration (23%), tauopathies (18%), diffuse Lewy body disease (14%), and Alzheimer disease (9%).
- Diffuse Lewy body disease cases showed a distinct pattern of initial encephalopathy followed by rapid decline.
Conclusions:
- While CJD is a frequent cause of rapidly progressive dementia, other neurodegenerative diseases can also present this way.
- A disease duration exceeding 12 months may suggest a non-CJD diagnosis.
- Accurate diagnosis is crucial for understanding and managing rapidly progressive neurodegenerative conditions.
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