Abstract

Most dementias begin insidiously, developing slowly and generally occurring in the elderly age group. The so-called rapidly progressive dementias constitute a different, diverse collection of conditions, many of which are reversible or treatable. For this reason, prompt identification and assessment of acute and subacute forms of dementia are critical to effective treatment. Numerous other entities within this category of presenile rapid-onset dementias are untreatable such as the prion-related diseases. Neuroimaging aids in the diagnosis and evaluation of many of these rapidly progressive dementias, which include myriad conditions ranging from variations of more common neurodegenerative dementias, such as Alzheimer disease, dementia with Lewy bodies, and frontotemporal dementia; infectious-related dementias such as acquired immune deficiency syndrome dementia; autoimmune and malignancy-related conditions; to toxic and metabolic forms of encephalopathy. This first of a 2-part review will specifically address the ability of MR imaging and ancillary neuroimaging strategies to support the diagnostic evaluation of rapidly progressive dementias due to neurodegenerative causes.

Highlights

  • Serial MR imaging has been used to characterize the atrophy noted within the pons, being good at distinguishing MSA and CBD with a 3-fold greater atrophy rate, both of which can be differentiated on this basis from Parkinson dementia and healthy controls.[29]

  • From the wealth of clinical studies performed to date, MR imaging has a powerful role in the diagnosis and prognostication of HD. This first installment of a 2-part review of MR imaging findings in the diverse clinical manifestation of rapidly progressive dementia discussed the use of neuroimaging in diagnosing neurodegenerative conditions, ranging from the common aging-associated dementias to uncommon presentations of less prevalent conditions such as multiple-system atrophy

  • MR imaging with selective use of DWI, DTI, and MR spectroscopy is essential to narrow the differential diagnosis on the basis of the subtle differences between neurodegenerative dementias, and it plays a key role in establishing the diagnosis

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