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Abbiategrasso Brain Bank Protocol for Collecting, Processing and Characterizing Aging Brains

Abbiategrasso Brain Bank Protocol for Collecting, Processing and Characterizing Aging Brains

Published on: June 3, 2020

Rapidly progressive young-onset dementia.

Brendan J Kelley¹, Bradley F Boeve, Keith A Josephs

¹Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.

Cognitive and Behavioral Neurology : Official Journal of the Society for Behavioral and Cognitive Neurology

|April 18, 2009

Summary

This summary is machine-generated.

Rapidly progressive young-onset dementia (RP-YOD) presents with diverse causes and overlapping symptoms, often mimicking Creutzfeldt-Jakob disease. Diagnostic evaluations are crucial for identifying the etiology in these challenging cases.

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Published on: November 21, 2013

Area of Science:

Neurology
Neuroscience
Dementia Research

Background:

Limited data exists on rapidly progressive young-onset dementia (RP-YOD).
Existing literature primarily comprises case reports or small series.
Characterizing RP-YOD is essential for understanding its clinical spectrum.

Purpose of the Study:

To characterize a cohort of patients with dementia onset before age 45.
To identify common clinical features and potential etiologies of RP-YOD.
To evaluate the diagnostic utility of various investigations in RP-YOD.

Main Methods:

Retrospective review of Mayo Clinic medical records.
Inclusion criteria: dementia onset before age 45 with rapid progression.
Comprehensive review of clinical data, laboratory results, neuroimaging, and pathology.

Main Results:

Twenty-two patients met the study criteria.
Common early features included behavioral/affective disorders, cerebellar, and visual/oculomotor dysfunction.
Many patients exhibited features suggestive of Creutzfeldt-Jakob disease.
Diagnostic testing identified the etiology in most cases.

Conclusions:

RP-YOD encompasses various etiologies with overlapping clinical presentations.
Creutzfeldt-Jakob disease-like features are prevalent in RP-YOD.
While diagnostic studies are helpful, some cases remain challenging to diagnose definitively.