rapidly progressive dementia
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Introduction
- 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)*
- hereditary diffuse leukoencephalopathy with spheroids
- adult-polyglucosan body disease
- Marchiafava-Bignami disease
- superficial siderosis of the central nervous system
- Huntington disease
- autoimmune encephalitis**
- inflammatory vasculitis*
- Wernicke encephalopathy*
- familial Alzheimer's disease
- glioblastoma multiforme
* potentially treatable ** most common treatable disorder
Laboratory
- complete blood count
- comprehensive metabolic panel;
- serum TSH
- serum vitamin B12
- serum 25-hydroxyvitamin D levels
- inflammatory markers
- autoimmune panel
- urine drug screen.
- CSF 14-3-3
Diagnostic procedures
- a STAM3P score of >=3 with positive predictive value of 100% for potentially treatable disorder[4]
Radiology
More general terms
References
- ↑ Chitravas N et al. Treatable neurological disorders misdiagnosed as Creutzfeldt- Jakob disease. Ann Neurology 2011 14 Jun PMID: https://pubmed.ncbi.nlm.nih.gov/21674591
- ↑ Rosenbloom MH, Atri A. The evaluation of rapidly progressive dementia. Neurologist. 2011 Mar;17(2):67-74. PMID: https://pubmed.ncbi.nlm.nih.gov/21364356
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 16 American College of Physicians, Philadelphia 2012
Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025 - ↑ 4.0 4.1 4.2 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: https://pubmed.ncbi.nlm.nih.gov/37782554 https://onlinelibrary.wiley.com/doi/10.1002/ana.26812
- ↑ Day GS. Rapidly progressive dementia. Continuum (Minneap Minn). 2022;28:901-936. PMID: https://pubmed.ncbi.nlm.nih.gov/35678409