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RAPIDLY PROGRESSIVE DEMENTIAS
MOHAMED E. ABD-ELHADY, MSC
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).
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.
DDX
APPROACH TO A PATIENT WITH RPD
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.
Approach to patients with Rapidly Progressive Dementias

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Approach to patients with Rapidly Progressive Dementias

  • 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).
  • 3.
  • 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
  • 6.
  • 7.
  • 8.
  • 9. APPROACH TO A PATIENT WITH RPD
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  • 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.