Abstract
Background and purposeRapidly progressive dementia (RPD) is an emergency in cognitive neurology, defined as cognitive impairment affecting the daily living activities developed over less than 1 year. This study investigated the profile of patients with rapidly progressive dementia at first presentation.MethodsRetrospective case analysis was done in 187 patients with rapidly progressive dementia who presented to the Postgraduate Institute of Medical Education and Research, Chandigarh, India from January 2008 to August 2016. Patients were divided into three groups: (1) Reversible (treatable) secondary dementia group, (2) Prion dementia group (sporadic Creutzfeldt-Jakob disease), (3) Non-prion Neurodegenerative and vascular dementias (primary neurodegenerative and vascular dementia). Cases presenting with delirium secondary to metabolic, drug induced or septic causes and those with signs of meningitis were excluded.ResultsSecondary reversible causes formed the most common cause for RPD with immune mediated encephalitides, neoplastic and infectious disorders as the leading causes. The patients in this series had an younger onset of RPD. Infections presenting with RPD accounted for the most common cause in our series (39%) with SSPE (41%) as the leading cause followed by neurosyphilis (17.9%) and progressive multifocal leukoencephalopathy (15.3%). Immune mediated dementias formed the second most common (18.1%) etiologic cause for RPD. The neurodegenerative dementias were third common cause for RPD in our series. Neoplastic disorders and immune mediated presented early (< 6 months) while neurodegenerative disorders presented later (> 6 months).ConclusionsRapidly progressive dementia is an emergency in cognitive neurology with potentially treatable or reversible causes that should be sought for diligently.
Highlights
Degenerative dementias develop insidiously over years and are often diagnosed in late stages of the illness
Retrospective case analysis was done in 187 patients with rapidly progressive dementia who presented to the Postgraduate Institute of Medical Education and Research, Chandigarh, India from January 2008 to August 2016
Infections presenting with Rapidly progressive dementias (RPD) accounted for the most common cause in our series (39%) with sclerosing panencephalitis (SSPE) (41%) as the leading cause followed by neurosyphilis (17.9%) and progressive multifocal leukoencephalopathy (15.3%)
Summary
Degenerative dementias develop insidiously over years and are often diagnosed in late stages of the illness. Progressive dementias (RPD) on the other hand include a myriad of conditions that functionally disable an individual within a span of few days to years.[1,2,3] These are recognized earlier and provide an opportunity for intervention. With the advent of newer serological and imaging aids, a genre of immune mediated dementias is evolving as a treatable entity, even though prion diseases and neurodegenerative disorders have been implicated as the common etiologies presenting with RPD in most of the series published. Dementia demands exhaustive work up to determine the cause which may span across the whole spectrum of diagnostic modalities ranging from routine serological investigations and imaging to invasive tests involving brain biopsies. Progressive dementia (RPD) is an emergency in cognitive neurology, defined as cognitive impairment affecting the daily living activities developed over less than 1 year. This study investigated the profile of patients with rapidly progressive dementia at first presentation
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
