Eduardo de Novaes Costa Bergamaschi, Adaucto Wanderley da Nóbrega Junior, Edson Pillotto Duarte, Matheus Souza Steglich, Manuela Aparecida Kloeppel, Gabriel Martins Rodrigues, Iara de Sousa Coelho, Myllan Eiroa Feitosa, Katia Lin
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Etiologies of Rapidly Progressive Dementia: A Cross-Sectional Multicentric Tertiary Hospital Study
Background: Rapidly progressive dementia (RPD) is linked to several disorders. Reference centers for Creutzfeldt–Jakob disease have reported prion diseases as the most frequent cause of RPD. Alternatively, tertiary centers found high frequencies of nonprion diseases causing RPD, including a significant number of potentially treatable disorders. This study is aimed at describing the etiologies of RPD among inpatients from four tertiary centers in Florianópolis, Brazil.
Methods: We reviewed medical records of patients hospitalized from 2001 to 2020 under codes of the 10th revision of the International Classification of Diseases potentially related to RPD. Patients who fulfilled the criteria for RPD were selected. RPD was defined when (1) the criteria of the National Institute on Aging–Alzheimer’s Association for dementia were met and (2) the disease duration was 730 days or less. We revised all etiological diagnoses according to established clinical criteria.
Results: One hundred four patients with RPD were identified. The most frequent diagnostic groups were dementias of vascular origin (N = 27, 26.0%), nonprion degenerative diseases (N = 24, 23.0%), and mixed dementias (N = 9, 8.7%). All 11 patients with disease duration between 1 and 2 years had either dementia of vascular origin or nonprion degenerative dementia. Ten patients (9.6%)—diagnosed with autoimmune disorders, infectious disorders, neoplasia, or posterior reversible encephalopathy syndrome—showed improvement.
Conclusions: Dementias of vascular origin were the most frequent cause of RPD in our sample, which differs from what has been previously reported by the literature. Patients with dementia duration between 1 and 2 years seem to have disorders usually associated with slow progression, while all potentially reversible cases had a duration of 1 year or less. Thorough investigation of RPD is paramount for the identification of potentially reversible etiologies.
期刊介绍：
Acta Neurologica Scandinavica aims to publish manuscripts of a high scientific quality representing original clinical, diagnostic or experimental work in neuroscience. The journal''s scope is to act as an international forum for the dissemination of information advancing the science or practice of this subject area. Papers in English will be welcomed, especially those which bring new knowledge and observations from the application of therapies or techniques in the combating of a broad spectrum of neurological disease and neurodegenerative disorders. Relevant articles on the basic neurosciences will be published where they extend present understanding of such disorders. Priority will be given to review of topical subjects. Papers requiring rapid publication because of their significance and timeliness will be included as ''Clinical commentaries'' not exceeding two printed pages, as will ''Clinical commentaries'' of sufficient general interest. Debate within the speciality is encouraged in the form of ''Letters to the editor''. All submitted manuscripts falling within the overall scope of the journal will be assessed by suitably qualified referees.
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