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rapidly progressive dementia
- rapidly progressive dementia represents a subset of neurologic disorders resulting in cognitive, behavioral, & motor decline within 2 years
- timely recognition of patients with treatment-responsive rapidly progressive dementia may minimize diagnostic delays & missed opportunities for treatment of these patients [4]
Etiology:
- Creutzfeldt-Jakob disease (CJD)
- infectious meningoencephalitides
- transmissible spongiform encephalopathy (prion disease)
- central nervous system lymphoma (CNS lymphoma)*
- primary central nervous system lymphoma*
- hereditary diffuse leukoencephalopathy with spheroids
- adult-polyglucosan body disease
- Marchiafava-Bignami disease
- superficial siderosis of the central nervous system
- Huntington disease
- autoimmune encephalitis**
- limbic encephalitis
- paraneoplastic neurologic syndrome
- LGI1 encephalitis
- inflammatory vasculitis*
- Wernicke encephalopathy*
- familial Alzheimer's disease
- glioblastoma multiforme
* potentially treatable ** most common treatable disorder
Laboratory:
- CSF 14-3-3
- positive in > 50% with treatable dementias
- not specific for CJD
Special laboratory:
- a STAM3P score of >=3 with positive predictive value of 100% for potentially treatable disorder [4]
Radiology:
- diffusion-weighted magnetic resonance imaging
General
dementia; Alzheimer's disease & related dementias (ADRD)
References
- Chitravas N et al.
Treatable neurological disorders misdiagnosed as Creutzfeldt-
Jakob disease.
Ann Neurology 2011 14 Jun
PMID: 21674591
- Rosenbloom MH, Atri A.
The evaluation of rapidly progressive dementia.
Neurologist. 2011 Mar;17(2):67-74.
PMID: 21364356
- Medical Knowledge Self Assessment Program (MKSAP) 16
American College of Physicians, Philadelphia 2012
- Satyadev N, Tipton PW, Martens Y et al.
Improving early recognition of treatment-responsive causes of rapidly progressive
dementia: The STAM3P score.
Ann Neurol 2023 Oct 2; [e-pub].
PMID: 37782554
https://onlinelibrary.wiley.com/doi/10.1002/ana.26812