Cognitive and psychological functioning in Fabry disease

Linda Sigmundsdottir, Michel C. Tchan, Alex A. Knopman, Graham C. Menzies, Jennifer Batchelor, David O. Sillence

Research output: Contribution to journalArticleResearchpeer-review

Abstract

Fabry disease is an X-linked lysosomal storage disorder which can result in renal, cardiac, and cerebrovascular disease. Patients are at increased risk of stroke and neuroimaging studies note cerebrovascular pathology. This study provides a cognitive profile of a cohort of individuals with Fabry disease and investigates the impact of pain, age, renal, cardiac, and cerebrovascular functioning on cognition and psychological functioning. Seventeen Fabry patients (12 males) with ages ranging 25 to 60 years (M = 46.6+11.8), and 15 age-matched healthy controls (M = 46.2+12.7) were administered a comprehensive neuropsychological battery. Fabry males demonstrated slower speed of information processing, reduced performance on measures of executive functions (verbal generation, reasoning, problem solving, perseveration), were more likely to show clinically significant reductions, and were more likely to report symptoms of anxiety and depression. Conversely, Fabry females performed at a similar level to controls. Correlational analyses indicated a link between cognitive and clinical measures of disease severity.

LanguageEnglish
Pages642-650
Number of pages9
JournalArchives of Clinical Neuropsychology
Volume29
Issue number7
DOIs
Publication statusPublished - 1 Nov 2014

Fingerprint

Fabry Disease
Psychology
Kidney
Cerebrovascular Disorders
Executive Function
Automatic Data Processing
Neuroimaging
Cognition
Heart Diseases
Anxiety
Stroke
Depression
Pathology
Pain

Cite this

Sigmundsdottir, L., Tchan, M. C., Knopman, A. A., Menzies, G. C., Batchelor, J., & Sillence, D. O. (2014). Cognitive and psychological functioning in Fabry disease. Archives of Clinical Neuropsychology, 29(7), 642-650. https://doi.org/10.1093/arclin/acu047
Sigmundsdottir, Linda ; Tchan, Michel C. ; Knopman, Alex A. ; Menzies, Graham C. ; Batchelor, Jennifer ; Sillence, David O. / Cognitive and psychological functioning in Fabry disease. In: Archives of Clinical Neuropsychology. 2014 ; Vol. 29, No. 7. pp. 642-650.
@article{cfb2e165493842c7b7c7dade4c1b8a49,
title = "Cognitive and psychological functioning in Fabry disease",
abstract = "Fabry disease is an X-linked lysosomal storage disorder which can result in renal, cardiac, and cerebrovascular disease. Patients are at increased risk of stroke and neuroimaging studies note cerebrovascular pathology. This study provides a cognitive profile of a cohort of individuals with Fabry disease and investigates the impact of pain, age, renal, cardiac, and cerebrovascular functioning on cognition and psychological functioning. Seventeen Fabry patients (12 males) with ages ranging 25 to 60 years (M = 46.6+11.8), and 15 age-matched healthy controls (M = 46.2+12.7) were administered a comprehensive neuropsychological battery. Fabry males demonstrated slower speed of information processing, reduced performance on measures of executive functions (verbal generation, reasoning, problem solving, perseveration), were more likely to show clinically significant reductions, and were more likely to report symptoms of anxiety and depression. Conversely, Fabry females performed at a similar level to controls. Correlational analyses indicated a link between cognitive and clinical measures of disease severity.",
author = "Linda Sigmundsdottir and Tchan, {Michel C.} and Knopman, {Alex A.} and Menzies, {Graham C.} and Jennifer Batchelor and Sillence, {David O.}",
year = "2014",
month = "11",
day = "1",
doi = "10.1093/arclin/acu047",
language = "English",
volume = "29",
pages = "642--650",
journal = "Archives of Clinical Neuropsychology",
issn = "0887-6177",
publisher = "Pergamon-Elsevier Science",
number = "7",

}

Sigmundsdottir, L, Tchan, MC, Knopman, AA, Menzies, GC, Batchelor, J & Sillence, DO 2014, 'Cognitive and psychological functioning in Fabry disease', Archives of Clinical Neuropsychology, vol. 29, no. 7, pp. 642-650. https://doi.org/10.1093/arclin/acu047

Cognitive and psychological functioning in Fabry disease. / Sigmundsdottir, Linda; Tchan, Michel C.; Knopman, Alex A.; Menzies, Graham C.; Batchelor, Jennifer; Sillence, David O.

In: Archives of Clinical Neuropsychology, Vol. 29, No. 7, 01.11.2014, p. 642-650.

Research output: Contribution to journalArticleResearchpeer-review

TY - JOUR

T1 - Cognitive and psychological functioning in Fabry disease

AU - Sigmundsdottir, Linda

AU - Tchan, Michel C.

AU - Knopman, Alex A.

AU - Menzies, Graham C.

AU - Batchelor, Jennifer

AU - Sillence, David O.

PY - 2014/11/1

Y1 - 2014/11/1

N2 - Fabry disease is an X-linked lysosomal storage disorder which can result in renal, cardiac, and cerebrovascular disease. Patients are at increased risk of stroke and neuroimaging studies note cerebrovascular pathology. This study provides a cognitive profile of a cohort of individuals with Fabry disease and investigates the impact of pain, age, renal, cardiac, and cerebrovascular functioning on cognition and psychological functioning. Seventeen Fabry patients (12 males) with ages ranging 25 to 60 years (M = 46.6+11.8), and 15 age-matched healthy controls (M = 46.2+12.7) were administered a comprehensive neuropsychological battery. Fabry males demonstrated slower speed of information processing, reduced performance on measures of executive functions (verbal generation, reasoning, problem solving, perseveration), were more likely to show clinically significant reductions, and were more likely to report symptoms of anxiety and depression. Conversely, Fabry females performed at a similar level to controls. Correlational analyses indicated a link between cognitive and clinical measures of disease severity.

AB - Fabry disease is an X-linked lysosomal storage disorder which can result in renal, cardiac, and cerebrovascular disease. Patients are at increased risk of stroke and neuroimaging studies note cerebrovascular pathology. This study provides a cognitive profile of a cohort of individuals with Fabry disease and investigates the impact of pain, age, renal, cardiac, and cerebrovascular functioning on cognition and psychological functioning. Seventeen Fabry patients (12 males) with ages ranging 25 to 60 years (M = 46.6+11.8), and 15 age-matched healthy controls (M = 46.2+12.7) were administered a comprehensive neuropsychological battery. Fabry males demonstrated slower speed of information processing, reduced performance on measures of executive functions (verbal generation, reasoning, problem solving, perseveration), were more likely to show clinically significant reductions, and were more likely to report symptoms of anxiety and depression. Conversely, Fabry females performed at a similar level to controls. Correlational analyses indicated a link between cognitive and clinical measures of disease severity.

UR - http://www.scopus.com/inward/record.url?scp=84911378849&partnerID=8YFLogxK

U2 - 10.1093/arclin/acu047

DO - 10.1093/arclin/acu047

M3 - Article

VL - 29

SP - 642

EP - 650

JO - Archives of Clinical Neuropsychology

T2 - Archives of Clinical Neuropsychology

JF - Archives of Clinical Neuropsychology

SN - 0887-6177

IS - 7

ER -

Sigmundsdottir L, Tchan MC, Knopman AA, Menzies GC, Batchelor J, Sillence DO. Cognitive and psychological functioning in Fabry disease. Archives of Clinical Neuropsychology. 2014 Nov 1;29(7):642-650. https://doi.org/10.1093/arclin/acu047