• IMA sites
  • IMAJ services
  • IMA journals
  • Follow us
  • Alternate Text Alternate Text
עמוד בית
Sun, 14.07.24

Original Articles

IMAJ | volume 14

Journal 10, October 2012
pages: 624-628

Cognitive Testing in Fabry Disease: Pilot Using a Brief Computerized Assessment Tool


    Background: Recently, cognitive assessments of patients with Fabry disease highlighted neurocognitive impairment using test batteries that are time and labor intensive.

    Objectives: To introduce a user-friendly self-administered tool for cognitive testing in patients with Fabry disease.

    Methods: We used a computerized system requiring about 1 hour for patient follow-up. All patients with enzymatic and/or molecular diagnosis of Fabry disease seen in our clinic underwent assessment with the Fabry-specific Mainz Severity Score Index (MSSI) with subscores (neurological, renal, cardiac, and general) and a Mindstreams neurocognitive battery for mild impairment, evaluating memory, executive function, attention, information processing, visual spatial processing, verbal function, and motor skills. A Global Cognitive Score (GCS) was also computed.

    Results: Ten patients (3 males, 7 females) were tested (mean age 41.5, range 25–56 years). Males were younger, had moderate nephropathy and no cerebrovascular accident (CVA); their Mindstreams GCS was 85.6–107 points. Three females had mild-moderate (8,10,15 points) neurological MSSI subscores (two CVA); all females had Mindstreams GCS of 59–107.7 points. Below-average performance was prevalent, particularly in information processing and motor skills consistent with mild impairment. Average GCS in females (90.3 points) was lower than in males (98.2 points). For individual patients, performance was poorest in information processing (n=4), attention (n=2), motor skills (n=2), verbal function (n=1), and visual spatial processing (n=1).

    Conclusions: MindStreams may simplify cognitive assessment monitoring in Fabry disease.


    Legal Disclaimer: The information contained in this website is provided for informational purposes only, and should not be construed as legal or medical advice on any matter.
    The IMA is not responsible for and expressly disclaims liability for damages of any kind arising from the use of or reliance on information contained within the site.
    © All rights to information on this site are reserved and are the property of the Israeli Medical Association. Privacy policy

    2 Twin Towers, 35 Jabotinsky, POB 4292, Ramat Gan 5251108 Israel