Article

The Evaluation of Rapidly Progressive Dementia

Bedford VA Hospital, Geriatric Research and Education Clinical Center (GRECC), Bedford.
The Neurologist (Impact Factor: 1.08). 03/2011; 17(2):67-74. DOI: 10.1097/NRL.0b013e31820ba5e3
Source: PubMed

ABSTRACT Rapidly progressive dementia (RPD) is a unique set of disorders resulting in cognitive, behavioral, and motor decline within 2 years. A variety of etiologies may contribute to RPD including neurodegenerative, inflammatory, infectious, and toxic-metabolic conditions. Jakob-Creutzfeldt disease (CJD) is frequently the most concerning diagnosis on the differential. The challenge for the neurologist is distinguishing prion disease from reversible processes that result in dementia.
This review discusses the clinical aspects and the diagnostic workup of RPD. Particular focus is given to both CJD and the potentially treatable inflammatory conditions that may cause a similar presentation. Furthermore, a standardized stepwise approach is outlined for patients presenting with RPD.
Neurologists should adopt a standardized approach to the rapidly presenting disease processes that may mimic CJD in their clinical and radiologic features.

0 Followers
 · 
202 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Sporadic Creutzfeldt-Jakob disease is a rare disorder that typically presents as rapidly progressive dementia. We present a case of a highly functioning 41-year-old Navy Commander with sporadic Creutzfeldt-Jakob disease, to include the diagnostic challenges, rapid clinical deterioration, and limited treatment options. Reprint & Copyright © 2015 Association of Military Surgeons of the U.S.
    Military medicine 01/2015; 180(1):e174-6. DOI:10.7205/MILMED-D-14-00163 · 0.77 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Transcranial magnetic stimulation (TMS) is emerging as a promising tool to non-invasively assess specific cortical circuits in neurological diseases. A number of studies have reported the abnormalities in TMS assays of cortical function in dementias. A PubMed-based literature review on TMS studies targeting primary and secondary dementia has been conducted using the key words "transcranial magnetic stimulation" or "motor cortex excitability" and "dementia" or "cognitive impairment" or "memory impairment" or "memory decline". Cortical excitability is increased in Alzheimer's disease (AD) and in vascular dementia (VaD), generally reduced in secondary dementias. Short-latency afferent inhibition (SAI), a measure of central cholinergic circuitry, is normal in VaD and in frontotemporal dementia (FTD), but suppressed in AD. In mild cognitive impairment, abnormal SAI may predict the progression to AD. No change in cortical excitability has been observed in FTD, in Parkinson's dementia and in dementia with Lewy bodies. Short-interval intracortical inhibition and controlateral silent period (cSP), two measures of gabaergic cortical inhibition, are abnormal in most dementias associated with parkinsonian symptoms. Ipsilateral silent period (iSP), which is dependent on integrity of the corpus callosum is abnormal in AD. While single TMS measure owns low specificity, a panel of measures can support the clinical diagnosis, predict progression and possibly identify earlier the "brain at risk". In dementias, TMS can be also exploited to select and evaluate the responders to specific drugs and, it might become a rehabilitative tool, in the attempt to restore impaired brain plasticity.
    08/2014; 125(8). DOI:10.1016/j.clinph.2014.04.010
  • [Show abstract] [Hide abstract]
    ABSTRACT: While the most common dementia is Alzheimer disease (AD), a detailed history is needed to rule out rapidly progressive dementias (RPDs). RPDs are less than two years in duration and have a rate of progression faster typical neurodegenerative diseases. Identification of RPDs is important as some are treatable. This review focuses on the spectrum of RPDs, with special emphasis on paraneoplastic disorders and Creutzfeldt-Jakob disease (CJD).
    Missouri medicine 110(5):422-8.

Preview

Download
4 Downloads
Available from