Cognitive Impairments and Subjective Cognitive Complaints in Fabry Disease: A Nationwide Study and Review of the Literature

Publikation: Bidrag til bog/antologi/rapportBidrag til bog/antologiForskningfagfællebedømt

Standard

Cognitive Impairments and Subjective Cognitive Complaints in Fabry Disease : A Nationwide Study and Review of the Literature. / Loeb, Josefine; Feldt-Rasmussen, Ulla; Madsen, Christoffer Valdorff; Vogel, Asmus Mejling.

JIMD Reports. Bind 41 Springer, 2018. s. 73-80 (JIMD Reports).

Publikation: Bidrag til bog/antologi/rapportBidrag til bog/antologiForskningfagfællebedømt

Harvard

Loeb, J, Feldt-Rasmussen, U, Madsen, CV & Vogel, AM 2018, Cognitive Impairments and Subjective Cognitive Complaints in Fabry Disease: A Nationwide Study and Review of the Literature. i JIMD Reports. bind 41, Springer, JIMD Reports, s. 73-80. https://doi.org/10.1007/8904_2018_103

APA

Loeb, J., Feldt-Rasmussen, U., Madsen, C. V., & Vogel, A. M. (2018). Cognitive Impairments and Subjective Cognitive Complaints in Fabry Disease: A Nationwide Study and Review of the Literature. I JIMD Reports (Bind 41, s. 73-80). Springer. JIMD Reports https://doi.org/10.1007/8904_2018_103

Vancouver

Loeb J, Feldt-Rasmussen U, Madsen CV, Vogel AM. Cognitive Impairments and Subjective Cognitive Complaints in Fabry Disease: A Nationwide Study and Review of the Literature. I JIMD Reports. Bind 41. Springer. 2018. s. 73-80. (JIMD Reports). https://doi.org/10.1007/8904_2018_103

Author

Loeb, Josefine ; Feldt-Rasmussen, Ulla ; Madsen, Christoffer Valdorff ; Vogel, Asmus Mejling. / Cognitive Impairments and Subjective Cognitive Complaints in Fabry Disease : A Nationwide Study and Review of the Literature. JIMD Reports. Bind 41 Springer, 2018. s. 73-80 (JIMD Reports).

Bibtex

@inbook{a750bc81ce6b420fa984baf3a582cf89,
title = "Cognitive Impairments and Subjective Cognitive Complaints in Fabry Disease: A Nationwide Study and Review of the Literature",
abstract = "Fabry disease is a rare progressive X-linked lysosomal storage disorder which leads to neuropathic pain, organ dysfunction and cerebral pathology. Few studies have investigated cognitive impairment in Fabry disease and these previous studies are difficult to compare due to heterogeneous methodological designs and small cohorts. The objective was to investigate the frequency of cognitive impairment in the Danish nationwide cohort of Fabry patients. Further, we examined if subjective cognitive complaints were associated with objective cognitive performances in this patient group. Neuropsychological tests (17 measures) and evaluation of subjective complaints with the Perceived Deficits Questionnaire (PDQ) were applied in 41 of 63 patients. According to an a priori definition, 12 patients (29.3%) were cognitively impaired. Tests tapping psychomotor speed, attention and executive functions had the highest frequency of impairment. In general, disease related variables as Mainz Severity Score Index, enzyme activity and years since onset and depression did not have a significant impact on the categorisation of patients as being cognitively impaired or non-impaired. Thus, cognitive impairment in Fabry disease does not seem to occur solely by having symptoms for many years or by having high disease burden. However, impaired neuropsychological test results were significantly more common in patients with cerebrovascular disease. Only three patients had scores in the abnormal range of the PDQ scale and subjective perceptions of cognition were not associated with cognitive performances. The levels of subjective cognitive complaints were generally very low in the studied patients demonstrating that the absence of subjective cognitive complaints does not exclude the presence of objective cognitive problems.",
keywords = "Faculty of Social Sciences, Cognition, Fabry disease, Memory, Neuropsychology, Subjective symptoms",
author = "Josefine Loeb and Ulla Feldt-Rasmussen and Madsen, {Christoffer Valdorff} and Vogel, {Asmus Mejling}",
year = "2018",
doi = "10.1007/8904_2018_103",
language = "English",
volume = "41",
series = "JIMD Reports",
publisher = "Springer",
pages = "73--80",
booktitle = "JIMD Reports",
address = "Switzerland",

}

RIS

TY - CHAP

T1 - Cognitive Impairments and Subjective Cognitive Complaints in Fabry Disease

T2 - A Nationwide Study and Review of the Literature

AU - Loeb, Josefine

AU - Feldt-Rasmussen, Ulla

AU - Madsen, Christoffer Valdorff

AU - Vogel, Asmus Mejling

PY - 2018

Y1 - 2018

N2 - Fabry disease is a rare progressive X-linked lysosomal storage disorder which leads to neuropathic pain, organ dysfunction and cerebral pathology. Few studies have investigated cognitive impairment in Fabry disease and these previous studies are difficult to compare due to heterogeneous methodological designs and small cohorts. The objective was to investigate the frequency of cognitive impairment in the Danish nationwide cohort of Fabry patients. Further, we examined if subjective cognitive complaints were associated with objective cognitive performances in this patient group. Neuropsychological tests (17 measures) and evaluation of subjective complaints with the Perceived Deficits Questionnaire (PDQ) were applied in 41 of 63 patients. According to an a priori definition, 12 patients (29.3%) were cognitively impaired. Tests tapping psychomotor speed, attention and executive functions had the highest frequency of impairment. In general, disease related variables as Mainz Severity Score Index, enzyme activity and years since onset and depression did not have a significant impact on the categorisation of patients as being cognitively impaired or non-impaired. Thus, cognitive impairment in Fabry disease does not seem to occur solely by having symptoms for many years or by having high disease burden. However, impaired neuropsychological test results were significantly more common in patients with cerebrovascular disease. Only three patients had scores in the abnormal range of the PDQ scale and subjective perceptions of cognition were not associated with cognitive performances. The levels of subjective cognitive complaints were generally very low in the studied patients demonstrating that the absence of subjective cognitive complaints does not exclude the presence of objective cognitive problems.

AB - Fabry disease is a rare progressive X-linked lysosomal storage disorder which leads to neuropathic pain, organ dysfunction and cerebral pathology. Few studies have investigated cognitive impairment in Fabry disease and these previous studies are difficult to compare due to heterogeneous methodological designs and small cohorts. The objective was to investigate the frequency of cognitive impairment in the Danish nationwide cohort of Fabry patients. Further, we examined if subjective cognitive complaints were associated with objective cognitive performances in this patient group. Neuropsychological tests (17 measures) and evaluation of subjective complaints with the Perceived Deficits Questionnaire (PDQ) were applied in 41 of 63 patients. According to an a priori definition, 12 patients (29.3%) were cognitively impaired. Tests tapping psychomotor speed, attention and executive functions had the highest frequency of impairment. In general, disease related variables as Mainz Severity Score Index, enzyme activity and years since onset and depression did not have a significant impact on the categorisation of patients as being cognitively impaired or non-impaired. Thus, cognitive impairment in Fabry disease does not seem to occur solely by having symptoms for many years or by having high disease burden. However, impaired neuropsychological test results were significantly more common in patients with cerebrovascular disease. Only three patients had scores in the abnormal range of the PDQ scale and subjective perceptions of cognition were not associated with cognitive performances. The levels of subjective cognitive complaints were generally very low in the studied patients demonstrating that the absence of subjective cognitive complaints does not exclude the presence of objective cognitive problems.

KW - Faculty of Social Sciences

KW - Cognition

KW - Fabry disease

KW - Memory

KW - Neuropsychology

KW - Subjective symptoms

U2 - 10.1007/8904_2018_103

DO - 10.1007/8904_2018_103

M3 - Book chapter

C2 - 29654545

VL - 41

T3 - JIMD Reports

SP - 73

EP - 80

BT - JIMD Reports

PB - Springer

ER -

ID: 215360418