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Rapidly progressive dementia and intractable diarrhea: a teaching case report and a systematic review of cognitive impairment in Whipple’s disease

OBJECTIVE: Whipple’s disease (WD) is a systemic, chronic, relapsing disease caused by Tropheryma whipplei, which can mimic signs and symptoms of various clinical entities. Typical manifestations are represented by gastrointestinal and systemic symptoms, among which neurological ones are frequent. We...

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Autores principales: Manini, Arianna, Querzola, Giacomo, Lovati, Carlo, Pantoni, Leonardo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer International Publishing 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8722651/
https://www.ncbi.nlm.nih.gov/pubmed/34981284
http://dx.doi.org/10.1007/s10072-021-05844-5
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author Manini, Arianna
Querzola, Giacomo
Lovati, Carlo
Pantoni, Leonardo
author_facet Manini, Arianna
Querzola, Giacomo
Lovati, Carlo
Pantoni, Leonardo
author_sort Manini, Arianna
collection PubMed
description OBJECTIVE: Whipple’s disease (WD) is a systemic, chronic, relapsing disease caused by Tropheryma whipplei, which can mimic signs and symptoms of various clinical entities. Typical manifestations are represented by gastrointestinal and systemic symptoms, among which neurological ones are frequent. We present the case of a patient with WD and rapidly progressive cognitive impairment and a review of literature aimed to report epidemiological, clinical, neuroimaging, and laboratory findings of cognitive impairment associated with WD. METHODS: A systematic review of medical literature published until November 22, 2020, was performed. Full-text, peer-reviewed case reports and series in English language presenting patients with WD and cognitive impairment were included. Data concerning demographic, clinical, neuroimaging, and laboratory characteristics were collected and synthesized qualitatively. RESULTS: The patient was a 54-year-old male who developed rapidly progressive dementia, fluctuating arousal disturbances, and supranuclear ophthalmoparesis associated with chronic diarrhea and fever spikes. T. whipplei was detected in the cerebrospinal fluid, and appropriate antimicrobial therapy was given with progressive clinical benefit. The systematic review of 114 case reports/series identified 147 patients with WD and cognitive impairment; this latter was rarely isolated. Neurological symptoms associated with cognitive decline were psychiatric disturbances, supranuclear ophthalmoplegia, hypothalamic involvement, and consciousness disorders. Brain imaging and cerebrospinal fluid findings were heterogeneous and nonspecific. CONCLUSIONS: Cognitive impairment represents one of the most common neurological features associated with WD. The clinical suspicion of this disease in patients with rapidly progressive dementia is crucial to guide diagnostic strategies and proper antimicrobial therapy, which may revert the clinical deterioration. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s10072-021-05844-5.
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spelling pubmed-87226512022-01-04 Rapidly progressive dementia and intractable diarrhea: a teaching case report and a systematic review of cognitive impairment in Whipple’s disease Manini, Arianna Querzola, Giacomo Lovati, Carlo Pantoni, Leonardo Neurol Sci Review Article OBJECTIVE: Whipple’s disease (WD) is a systemic, chronic, relapsing disease caused by Tropheryma whipplei, which can mimic signs and symptoms of various clinical entities. Typical manifestations are represented by gastrointestinal and systemic symptoms, among which neurological ones are frequent. We present the case of a patient with WD and rapidly progressive cognitive impairment and a review of literature aimed to report epidemiological, clinical, neuroimaging, and laboratory findings of cognitive impairment associated with WD. METHODS: A systematic review of medical literature published until November 22, 2020, was performed. Full-text, peer-reviewed case reports and series in English language presenting patients with WD and cognitive impairment were included. Data concerning demographic, clinical, neuroimaging, and laboratory characteristics were collected and synthesized qualitatively. RESULTS: The patient was a 54-year-old male who developed rapidly progressive dementia, fluctuating arousal disturbances, and supranuclear ophthalmoparesis associated with chronic diarrhea and fever spikes. T. whipplei was detected in the cerebrospinal fluid, and appropriate antimicrobial therapy was given with progressive clinical benefit. The systematic review of 114 case reports/series identified 147 patients with WD and cognitive impairment; this latter was rarely isolated. Neurological symptoms associated with cognitive decline were psychiatric disturbances, supranuclear ophthalmoplegia, hypothalamic involvement, and consciousness disorders. Brain imaging and cerebrospinal fluid findings were heterogeneous and nonspecific. CONCLUSIONS: Cognitive impairment represents one of the most common neurological features associated with WD. The clinical suspicion of this disease in patients with rapidly progressive dementia is crucial to guide diagnostic strategies and proper antimicrobial therapy, which may revert the clinical deterioration. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s10072-021-05844-5. Springer International Publishing 2022-01-03 2022 /pmc/articles/PMC8722651/ /pubmed/34981284 http://dx.doi.org/10.1007/s10072-021-05844-5 Text en © Fondazione Società Italiana di Neurologia 2022 This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.
spellingShingle Review Article
Manini, Arianna
Querzola, Giacomo
Lovati, Carlo
Pantoni, Leonardo
Rapidly progressive dementia and intractable diarrhea: a teaching case report and a systematic review of cognitive impairment in Whipple’s disease
title Rapidly progressive dementia and intractable diarrhea: a teaching case report and a systematic review of cognitive impairment in Whipple’s disease
title_full Rapidly progressive dementia and intractable diarrhea: a teaching case report and a systematic review of cognitive impairment in Whipple’s disease
title_fullStr Rapidly progressive dementia and intractable diarrhea: a teaching case report and a systematic review of cognitive impairment in Whipple’s disease
title_full_unstemmed Rapidly progressive dementia and intractable diarrhea: a teaching case report and a systematic review of cognitive impairment in Whipple’s disease
title_short Rapidly progressive dementia and intractable diarrhea: a teaching case report and a systematic review of cognitive impairment in Whipple’s disease
title_sort rapidly progressive dementia and intractable diarrhea: a teaching case report and a systematic review of cognitive impairment in whipple’s disease
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8722651/
https://www.ncbi.nlm.nih.gov/pubmed/34981284
http://dx.doi.org/10.1007/s10072-021-05844-5
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