approach to the evolving concept of rapidly progressive dementias.

1. RAPIDLY PROGRESSIVE DEMENTIAS
MOHAMED E. ABD-ELHADY, MSC

2. DEFINITION OF RPD
• A cognitive disorder with fast progression leading to the clinical syndrome of
dementia, within a relatively brief time period, which is commonly considered to
be less than either 1 or 2 years.
• Despite that the general definitions usually consider RPD of less than 1 or 2 years
as the time span from the first disease-related symptom to the development of
the dementia syndrome, some causes of RPD, such as encephalitis or metabolic
encephalopathies, can lead to dementia within weeks.
• Another approach is the more objective measures for the speed of cognitive
decline such as changes in Mini-Mental State Examination (MMSE) scores in
rapidly progressive AD (rpAD).

4. DEFINITION OF RPD
• RPD is one of the typical clinical characteristics of Creutzfeldt–Jakob disease (CJD)
and has long been part of the diagnostic criteria for this condition.
• Prion diseases have been considered to be prototypical RPDs.

5. DDX

9. APPROACH TO A PATIENT WITH RPD

17. THERAPEUTIC OPTIONS
• RPDs caused by inflammatory, metabolic, or neoplastic diseases can be reversible
with appropriate treatment, and many aggravating factors that contribute to
cognitive deficits, such as seizures or affective symptoms, might also respond to
therapeutic intervention.
• Nonetheless, clinical management might reach a point where a palliative
therapeutic regimen has to be considered.
• In patients with an irreversible condition, the treatment of secondary
complications and application of life-extending procedures such as percutaneous
endoscopic gastrostomy tubes determine the survival time.