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Cognitive testing in Fabry disease: pilot using a brief computerized assessment tool.

Deborah Elstein¹, Glen M Doniger, Gheona Altarescu

¹Gaucher Clinic, Shaare Zedek Medical Center, P.O. Box 3235, Jerusalem, 91031, Israel.

The Israel Medical Association Journal : IMAJ

|December 1, 2012

Summary

This summary is machine-generated.

A new tool, MindStreams, simplifies cognitive testing for Fabry disease patients, revealing prevalent mild impairments, especially in information processing and motor skills. This user-friendly assessment aids in monitoring neurocognitive function in Fabry disease.

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Area of Science:

Neurology
Genetics
Medical Diagnostics

Background:

Fabry disease patients often experience neurocognitive impairment.
Current cognitive assessment methods are time-consuming and labor-intensive.

Purpose of the Study:

To introduce a user-friendly, self-administered tool for cognitive testing in Fabry disease.
To assess the utility of the MindStreams neurocognitive battery for mild impairment in this population.

Main Methods:

A computerized system, MindStreams, was used for cognitive assessment.
Patients also underwent the Fabry-specific Mainz Severity Score Index (MSSI).
The MindStreams battery evaluated memory, executive function, attention, processing speed, and motor skills.

Main Results:

Ten patients (3 males, 7 females) were evaluated.
Below-average performance was common, particularly in information processing and motor skills.
Females showed lower average Global Cognitive Scores (GCS) than males.

Conclusions:

MindStreams offers a simplified approach to monitoring cognitive function in Fabry disease.
The tool effectively identifies mild neurocognitive impairments in patients.