The Mini Mental State Examination does not accurately screen for objective cognitive impairment in Fabry Disease
Abstract Fabry disease (FD) patients may suffer from objective cognitive impairment (OCI). This study assessed the accuracy of the Mini Mental State Examination (MMSE) to screen for OCI in FD patients. Presence or absence of OCI was established using a neuropsychological test battery. For different...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2019-07-01
|
| Series: | JIMD Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/jmd2.12036 |
| _version_ | 1811196107995217920 |
|---|---|
| author | Simon Körver Sara A. J. van deSchraaf Gert J. Geurtsen Carla E. M. Hollak Ivo N. vanSchaik Mirjam Langeveld |
| author_facet | Simon Körver Sara A. J. van deSchraaf Gert J. Geurtsen Carla E. M. Hollak Ivo N. vanSchaik Mirjam Langeveld |
| author_sort | Simon Körver |
| collection | DOAJ |
| description | Abstract Fabry disease (FD) patients may suffer from objective cognitive impairment (OCI). This study assessed the accuracy of the Mini Mental State Examination (MMSE) to screen for OCI in FD patients. Presence or absence of OCI was established using a neuropsychological test battery. For different MMSE cutoffs sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and clinical utility index (CUI) to identify OCI were calculated. Eighty‐one patients were included (mean age 44.5 ± 14.3, 35% men, 74% classical phenotype) of which 13 patients (16%) had OCI. The median MMSE score was 29 (range: 25‐30). MMSE cutoffs ≤28 and ≤29 had the highest sensitivity and specificity, with higher specificity reached at cutoff ≤28 (sensitivity: .46, specificity: .73) and higher sensitivity at cutoff ≤29 (sensitivity: .92, specificity: .40). PPV was low for both cutoffs (PPV ≤28: .25, PPV ≤29: .23) resulting in a low positive CUI (case finding ability). The results of our study indicate that the MMSE does not accurately screen for OCI in FD, with poor sensitivity‐specificity trade‐off at all cutoffs. The low PPV shows that the majority of FD patients that score below the cutoffs do not suffer from OCI. Administering the MMSE as a screening test will lead to unnecessary referrals for neuropsychological testing, which is time consuming and burdensome. Screening tools designed to accurately detect mild (executive) impairment might prove more appropriate to screen for OCI in FD. |
| first_indexed | 2024-04-12T00:53:13Z |
| format | Article |
| id | doaj.art-13a4de560d144299b36a3734fc782735 |
| institution | Directory Open Access Journal |
| issn | 2192-8312 |
| language | English |
| last_indexed | 2024-04-12T00:53:13Z |
| publishDate | 2019-07-01 |
| publisher | Wiley |
| record_format | Article |
| series | JIMD Reports |
| spelling | doaj.art-13a4de560d144299b36a3734fc7827352022-12-22T03:54:40ZengWileyJIMD Reports2192-83122019-07-01481535910.1002/jmd2.12036The Mini Mental State Examination does not accurately screen for objective cognitive impairment in Fabry DiseaseSimon Körver0Sara A. J. van deSchraaf1Gert J. Geurtsen2Carla E. M. Hollak3Ivo N. vanSchaik4Mirjam Langeveld5Department of Endocrinology and Metabolism Amsterdam UMC, Location AMC, University of Amsterdam Amsterdam The NetherlandsDepartment of Medical Psychology Amsterdam UMC, Location AMC, University of Amsterdam Amsterdam The NetherlandsDepartment of Medical Psychology Amsterdam UMC, Location AMC, University of Amsterdam Amsterdam The NetherlandsDepartment of Endocrinology and Metabolism Amsterdam UMC, Location AMC, University of Amsterdam Amsterdam The NetherlandsDepartment of Neurology Amsterdam UMC, Location AMC, University of Amsterdam Amsterdam The NetherlandsDepartment of Endocrinology and Metabolism Amsterdam UMC, Location AMC, University of Amsterdam Amsterdam The NetherlandsAbstract Fabry disease (FD) patients may suffer from objective cognitive impairment (OCI). This study assessed the accuracy of the Mini Mental State Examination (MMSE) to screen for OCI in FD patients. Presence or absence of OCI was established using a neuropsychological test battery. For different MMSE cutoffs sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and clinical utility index (CUI) to identify OCI were calculated. Eighty‐one patients were included (mean age 44.5 ± 14.3, 35% men, 74% classical phenotype) of which 13 patients (16%) had OCI. The median MMSE score was 29 (range: 25‐30). MMSE cutoffs ≤28 and ≤29 had the highest sensitivity and specificity, with higher specificity reached at cutoff ≤28 (sensitivity: .46, specificity: .73) and higher sensitivity at cutoff ≤29 (sensitivity: .92, specificity: .40). PPV was low for both cutoffs (PPV ≤28: .25, PPV ≤29: .23) resulting in a low positive CUI (case finding ability). The results of our study indicate that the MMSE does not accurately screen for OCI in FD, with poor sensitivity‐specificity trade‐off at all cutoffs. The low PPV shows that the majority of FD patients that score below the cutoffs do not suffer from OCI. Administering the MMSE as a screening test will lead to unnecessary referrals for neuropsychological testing, which is time consuming and burdensome. Screening tools designed to accurately detect mild (executive) impairment might prove more appropriate to screen for OCI in FD.https://doi.org/10.1002/jmd2.12036Fabry diseaselysosomal storage diseasesmini mental state examinationMMSEneurocognitive tests |
| spellingShingle | Simon Körver Sara A. J. van deSchraaf Gert J. Geurtsen Carla E. M. Hollak Ivo N. vanSchaik Mirjam Langeveld The Mini Mental State Examination does not accurately screen for objective cognitive impairment in Fabry Disease JIMD Reports Fabry disease lysosomal storage diseases mini mental state examination MMSE neurocognitive tests |
| title | The Mini Mental State Examination does not accurately screen for objective cognitive impairment in Fabry Disease |
| title_full | The Mini Mental State Examination does not accurately screen for objective cognitive impairment in Fabry Disease |
| title_fullStr | The Mini Mental State Examination does not accurately screen for objective cognitive impairment in Fabry Disease |
| title_full_unstemmed | The Mini Mental State Examination does not accurately screen for objective cognitive impairment in Fabry Disease |
| title_short | The Mini Mental State Examination does not accurately screen for objective cognitive impairment in Fabry Disease |
| title_sort | mini mental state examination does not accurately screen for objective cognitive impairment in fabry disease |
| topic | Fabry disease lysosomal storage diseases mini mental state examination MMSE neurocognitive tests |
| url | https://doi.org/10.1002/jmd2.12036 |
| work_keys_str_mv | AT simonkorver theminimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease AT saraajvandeschraaf theminimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease AT gertjgeurtsen theminimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease AT carlaemhollak theminimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease AT ivonvanschaik theminimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease AT mirjamlangeveld theminimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease AT simonkorver minimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease AT saraajvandeschraaf minimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease AT gertjgeurtsen minimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease AT carlaemhollak minimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease AT ivonvanschaik minimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease AT mirjamlangeveld minimentalstateexaminationdoesnotaccuratelyscreenforobjectivecognitiveimpairmentinfabrydisease |