Rapidly Progressive Dementia

Day, Gregory S.

doi:10.1212/con.0000000000001089

Cited by 13 publications

(35 citation statements)

References 116 publications

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“…It becomes noticeable to them only when it severely affects functionality. 1 The following clinical vignettes illustrate our point. All patients had a history of what could initially be classified as rapidly progressive cognitive impairment.…”

Section: Dear Editormentioning

confidence: 80%

Slowly diagnosed dementia

Jiménez‐Ruiz,

Sotelo‐Ramirez,

Aguilar‐Fuentes

et al. 2023

Psychogeriatrics

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Section: Dear Editormentioning

confidence: 80%

Slowly diagnosed dementia

Jiménez‐Ruiz,

Sotelo‐Ramirez,

Aguilar‐Fuentes

et al. 2023

Psychogeriatrics

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“…A monoclonal serum component is present in only 14% of cases [4, 13, 17]. In our patients, as recommended by the guidelines for RPD diagnosis [7, 8, 12], routine blood tests, including blood cell count, electrolytes, kidney and liver function markers, thyroid hormones, and B vitamins, were repeatedly performed to exclude metabolic causes of RPD. Specific tests for syphilis, borreliosis, HIV, viral encephalitis, and autoimmune encephalitis were variably used to exclude inflammatory causes of RPD.…”

Section: Discussionmentioning

confidence: 99%

“…The term RPD is commonly used to indicate a heterogeneous set of diseases leading to a dementia syndrome (multi-domain cognitive decline with functional impairment) within a variable timeframe, generally considered less than 1 or 2 years [7]. Despite being a rare condition (3%-4% of dementia cases in clinical practice), early etiological diagnosis is critical due to the high frequency of, at least partially, treatable causes [9,12]. The IVLBCL classical variant is increasingly recognized as a rare and potentially treatable cause of RPD [6,8] whose diagnosis is often missed or delayed, especially if the clinical and instrumental findings meet the diagnostic criteria for more common causes of RPD, such as sCJD, encephalitis or vascular dementia [7][8][9].…”

Section: Discussionmentioning

confidence: 99%

Rapidly progressive dementia due to intravascular lymphoma: A prion disease reference center experience

Bentivenga,

Baiardi,

Righini

et al. 2023

Euro J of Neurology

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BackgroundIntravascular large B‐cell lymphoma (IVLBCL) is a rare extranodal lymphoma that is characterized by the selective growth of neoplastic cells in blood vessels, representing a potentially treatable cause of rapidly progressive dementia (RPD). Given its diverse clinical and instrumental presentation, it is often misdiagnosed with more common RPD causes, for example, Creutzfeldt‐Jakob disease (CJD) or vascular dementia.MethodsThis study presents the clinical and histopathological characteristics of four IVLBCL cases that we diagnosed post‐mortem over 20 years among over 600 brain samples received as suspected CJD cases at our prion disease reference center.ResultsOur patients exhibited various presenting symptoms, including behavioral disturbances, disorientation, and alertness fluctuations. The diagnostic tests performed at the time, including blood work, cerebrospinal fluid (CSF) analyses, electroencephalography, and neuroimaging, yielded nonspecific and occasionally misleading results. Consequently, the patients were repeatedly diagnosed as variably having CJD, epilepsy, vascular dementia, and encephalitis. The stored CSF samples of two patients tested negative at prion real‐time quaking‐induced conversion (RT‐QuIC), which we performed afterwards for research purposes. Neuropathological analysis revealed a differential involvement of various brain areas, with frontotemporal neocortices being the most affected.ConclusionsOur results confirm the significant clinical and instrumental heterogeneity of IVLBCL. Neuropathological evidence of the preferential involvement of frontotemporal neocortices, potentially conditioning the clinical phenotype, could be relevant to reach an early diagnosis. Finally, given the therapeutic implications of its misdiagnosis with CJD, we emphasize the utility of prion RT‐QuIC as a test for ruling out CJD in these patients.

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“…For example, rapidly progressive dementia may be caused by Creutzfeldt-Jakob disease (CJD), and highly accurate CSF tests for CJD are available. For more information on CJD and available testing, refer to the article “Rapidly Progressive Dementia” by Gregory S. Day, MD, MSc, MSCI, FAAN, 11 in this issue of Continuum .…”

Section: Indications For Advanced Diagnostic Testingmentioning

confidence: 99%

Fluid Biomarkers in Dementia Diagnosis

Schindler¹

2022

CONTINUUM: Lifelong Learning in Neurology

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PURPOSE OF REVIEWThis article discusses how fluid biomarkers can augment the routine dementia evaluation and improve diagnostic accuracy. The tests that are currently available and the indications for their use are described. Further, tests that are under development and likely to be used in the future are identified.RECENT FINDINGSTechnical improvements in assay sensitivity and precision have led to the rapid development of blood-based biomarkers for Alzheimer disease (AD) over the past several years. Studies have found that the ratio of amyloid-β (Aβ) peptides (Aβ42/Aβ40) and concentrations of phosphorylated tau isoforms in plasma can identify individuals with AD brain pathology. Blood-based tests may enable much broader use of AD biomarkers in the evaluation of patients with cognitive impairment.SUMMARYEven after a detailed history, examination, routine laboratory testing, and brain imaging, the cause of dementia sometimes remains unclear. CSF and blood-based biomarkers can evaluate for a range of neurologic disorders that are associated with dementia, including AD. Integrating data from fluid biomarker tests and the routine dementia evaluation may improve the accuracy of dementia diagnosis.

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Rapidly Progressive Dementia

Cited by 13 publications

References 116 publications

Slowly diagnosed dementia

Slowly diagnosed dementia

Rapidly progressive dementia due to intravascular lymphoma: A prion disease reference center experience

Fluid Biomarkers in Dementia Diagnosis

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