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22.05.2021 | Review Article

Neuropsychological assessment in Niemann-Pick disease type C: a systematic review

verfasst von: Giuseppa Maresca, Caterina Formica, Vanessa Nocito, Desiree Latella, Simona Leonardi, Maria Cristina De Cola, Giuseppe Triglia, Placido Bramanti, Francesco Corallo

Erschienen in: Neurological Sciences | Ausgabe 8/2021

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Abstract

Background

The neuropsychological profile of Niemann-Pick type C (NP-C) patients is characterized by an early deterioration in executive functions and attention. There are few studies on cognitive impairment and on neuropsychological assessment of NP-C disease. The purpose of this review is to analyze the studies on a psychological assessment for NP-C patients.

Method

This review aims to identify a neuropsychological assessment to evaluate cognitive domains and neuropsychological changes in these patients. There were a total of 73 articles. The search terms were identified as titles and abstracts. All articles were evaluated by title, abstract, and text.

Results

Only four of the 73 articles were included because they met the criteria of our review. Furthermore, in these studies, possible diagnostic protocols are proposed on NP-C subjects.

Discussion and conclusion

The cognitive impairment in NP-C has a negative impact on daily functioning and quality of life. Early diagnosis could identify cognitive deficits and promote cognitive interventions to improve the neuropsychological profile. The management of NP-C disease should be based on a multidisciplinary approach, to treating symptoms, preserving neurological functions, and guaranteeing the best possible quality of life. Early identification of neurological and psychological symptoms of the disease is necessary in order to decrease the progression of neurological disease and improve patient care and treatment outcomes. Furthermore, research should focus more on cognitive aspects, not only in the diagnostic process but also in the rehabilitation process.

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Titel: Neuropsychological assessment in Niemann-Pick disease type C: a systematic review
verfasst von: Giuseppa Maresca
Caterina Formica
Vanessa Nocito
Desiree Latella
Simona Leonardi
Maria Cristina De Cola
Giuseppe Triglia
Placido Bramanti
Francesco Corallo
Publikationsdatum: 22.05.2021
Verlag: Springer International Publishing
Erschienen in: Neurological Sciences / Ausgabe 8/2021
Print ISSN: 1590-1874
Elektronische ISSN: 1590-3478
DOI: https://doi.org/10.1007/s10072-021-05337-5

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Neuropsychological assessment in Niemann-Pick disease type C: a systematic review

Abstract

Background

Method

Results

Discussion and conclusion

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Abstract

Background

Method

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