Rapidly progressive dementias - aetiologies, diagnosis and management

被引:67
作者
Hermann, Peter [1 ,2 ]
Zerr, Inga [1 ,2 ,3 ]
机构
[1] Univ Med Ctr, Clin Dementia Ctr, Dept Neurol, Gottingen, Germany
[2] Univ Med Ctr, Natl Reference Ctr CJD Surveillance, Gottingen, Germany
[3] German Ctr Neurodegenerat Dis DZNE, Gottingen, Germany
关键词
CREUTZFELDT-JAKOB-DISEASE; FRONTOTEMPORAL LOBAR DEGENERATION; NMDA-RECEPTOR ENCEPHALITIS; DIFFUSION-WEIGHTED MRI; PRIMARY CNS LYMPHOMA; CEREBROSPINAL-FLUID; ALZHEIMERS-DISEASE; CLINICAL-FEATURES; SPORADIC CJD; CASE SERIES;
D O I
10.1038/s41582-022-00659-0
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
The term 'rapidly progressive dementia' (RPD) describes a cognitive disorder with fast progression, leading to dementia within a relatively short time. This Review discusses the wide range of RPD aetiologies, as well as the diagnostic approach and treatment options. Rapidly progressive dementias (RPDs) are a group of heterogeneous disorders that include immune-mediated, infectious and metabolic encephalopathies, as well as prion diseases and atypically rapid presentations of more common neurodegenerative diseases. Some of these conditions are treatable, and some must be diagnosed promptly because of their potential infectivity. Prion disease is considered to be the prototypical RPD, but over the past two decades, epidemiological reports and the identification of various encephalitis-mediating antibodies have led to a growing recognition of other encephalopathies as potential causes of rapid cognitive decline. Knowledge of RPD aetiologies, syndromes and diagnostic work-up protocols will help clinicians to establish an early, accurate diagnosis, thereby reducing morbidity and mortality, especially in immune-mediated and other potentially reversible dementias. In this Review, we define the syndrome of RPD and shed light on its different aetiologies and on secondary factors that might contribute to rapid cognitive decline. We describe an extended diagnostic procedure in the context of important differential diagnoses, discuss the utility of biomarkers and summarize potential treatment options. In addition, we discuss treatment options such as high-dose steroid therapy in the context of therapy and diagnosis in clinically ambiguous cases.
引用
收藏
页码:363 / 376
页数:14
相关论文
共 149 条
[141]   The importance of ongoing international surveillance for Creutzfeldt-Jakob disease [J].
Watson, Neil ;
Brandel, Jean-Philippe ;
Green, Alison ;
Hermann, Peter ;
Ladogana, Anna ;
Lindsay, Terri ;
Mackenzie, Janet ;
Pocchiari, Maurizio ;
Smith, Colin ;
Zerr, Inga ;
Pal, Suvankar .
NATURE REVIEWS NEUROLOGY, 2021, 17 (06) :362-379
[142]   Application of telehealth for comprehensive Creutzfeldt-Jakob disease surveillance in the United Kingdom [J].
Watson, Neil ;
Kurudzhu, Hatice ;
Green, Alison ;
Summers, David ;
Smith, Colin ;
Pal, Suvankar .
JOURNAL OF THE NEUROLOGICAL SCIENCES, 2021, 420
[143]   Evaluation of rapidly progressive dementia [J].
Woodruff, Bryan Keith .
SEMINARS IN NEUROLOGY, 2007, 27 (04) :363-375
[144]   SFPQ and Tau: critical factors contributing to rapid progression of Alzheimer's disease [J].
Younas, Neelam ;
Zafar, Saima ;
Shafiq, Mohsin ;
Noor, Aneeqa ;
Siegert, Anna ;
Arora, Amandeep Singh ;
Galkin, Alexey ;
Zafar, Ayesha ;
Schmitz, Mathias ;
Stadelmann, Christine ;
Andreoletti, Olivier ;
Ferrer, Isidre ;
Zerr, Inga .
ACTA NEUROPATHOLOGICA, 2020, 140 (03) :317-339
[145]   Creutzfeldt-Jakob disease in a man with COVID-19: SARS-CoV-2-accelerated neurodegeneration? [J].
Young, Michael J. ;
O'Hare, Meabh ;
Matiello, Marcelo ;
Schmahmann, Jeremy D. .
BRAIN BEHAVIOR AND IMMUNITY, 2020, 89 :601-603
[146]   Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease [J].
Zerr, I. ;
Kallenberg, K. ;
Summers, D. M. ;
Romero, C. ;
Taratuto, A. ;
Heinemann, U. ;
Breithaupt, M. ;
Varges, D. ;
Meissner, B. ;
Ladogana, A. ;
Schuur, M. ;
Haik, S. ;
Collins, S. J. ;
Jansen, Gerard H. ;
Stokin, G. B. ;
Pimentel, J. ;
Hewer, E. ;
Collie, D. ;
Smith, P. ;
Roberts, H. ;
Brandel, J. P. ;
van Duijn, C. ;
Pocchiari, M. ;
Begue, C. ;
Cras, P. ;
Will, R. G. ;
Sanchez-Juan, P. .
BRAIN, 2009, 132 :2659-2668
[147]   Diagnostic challenges in rapidly progressive dementia [J].
Zerr, Inga ;
Hermann, Peter .
EXPERT REVIEW OF NEUROTHERAPEUTICS, 2018, 18 (10) :761-772
[148]   Biomarkers for Alzheimer's disease-preparing for a new era of disease-modifying therapies [J].
Zetterberg, Henrik ;
Bendlin, Barbara B. .
MOLECULAR PSYCHIATRY, 2021, 26 (01) :296-308
[149]   Spectrum of noncerebrovascular rapidly progressive cognitive deterioration: a 2-year retrospective study [J].
Zhang, Yun ;
Gao, Ting ;
Tao, Qing-Qing .
CLINICAL INTERVENTIONS IN AGING, 2017, 12 :1655-1659