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Neuropsychological Functioning in PLS: A Comparison with ALS

  • Gloria M. Grace (a1), J. B. Orange (a2), Ann Rowe (a3), Karen Findlater (a3), Morris Freedman (a4) and Michael J. Strong (a3)...

Abstract:

Objective:

In order to characterize the nature and extent of neuropsychological dysfunction in primary lateral sclerosis (PLS), we studied prospectively cognitive, emotional, and behavioral functioning in PLS, and compared performances to functioning in amyotrophic lateral sclerosis (ALS).

Methods:

Eighteen patients with PLS and 13 patients with ALS completed a neuropsychological test battery assessing both cognitive skills and emotional/behavioral functioning.

Results:

Both PLS and ALS groups scored broadly within normal limits (mean T-scores greater than 40) on all cognitive measures and no significant between-group differences were found with the exception of one variable. However, when examined on a case by case basis, the data revealed considerable heterogeneity amongst patients in both groups. Overall, 39% of PLS patients and 31% of ALS patients were considered cognitively impaired. A higher than expected frequency of abnormal scores was noted for several tests of executive function in both groups, and a majority of PLS patients also exhibited abnormal behavioural symptoms. There was no relationship in PLS or ALS groups between cognitive functioning and disease duration, current site of disease, site of onset, functional status, and respiratory variables. Comparison between the PLS and ALS groups indicated virtually no differences in cognitive test scores and overall emotional/behavioural symptoms.

Conclusions:

We observed deficits in cognition and behaviour in a significant proportion of PLS patients which were comparable to those observed in ALS cases. Although deficits were not in the range of frontotemporal dementia, both ALS and PLS cases demonstrated deficits most prominently on tests of executive functioning.

Résumé: Objectif:

Nous avons étudié prospectivement le fonctionnement cognitif, émotionnel et comportemental dans la sclérose latérale primaire (SLP) afin de caractériser la nature et l'étendue de la dysfonction neuropsychologique et nous l'avons comparé au fonctionnement dans la sclérose latérale amyotrophique (SLA).

Méthodes:

Dix-huit patients atteints de SLP et 13 patients atteints de SLA ont complété une batterie de tests neuropsychologiques évaluant les habiletés cognitives et le fonctionnement émotionnel/comportemental.

Résultats:

Les scores dans les deux groupes étaient dans les limites normales (scores T moyen > 40) pour toutes les mesures cognitives et nous n'avons pas observé de différences significatives entre les groupes à l'exception d'une variable. Cependant, à l'examen cas par cas, nous avons constaté une hétérogénéité considérable des données chez les patients des deux groupes. Globalement, 39% des patients atteints de SLP et 31 % des patients atteints de SLA avaient un déficit cognitif. Nous avons noté une fréquence plus élevée qu'attendue de scores anormaux pour plusieurs tests de fonction exécutive dans les deux groupes et une majorité de patients atteints de SLP avaient également des symptômes comportementaux anormaux. Il n'y avait pas de relation dans le groupe SLP ou dans le groupe SLA entre le fonctionnement cognitif et la durée de la maladie, la localisation actuelle de la maladie, la localisation de début de la maladie, l'état fonctionnel et les variables respiratoires. La comparaison des deux groupes n'a pas révélé de différences dans les scores aux teste cognitifs et dans les symptômes émotionnels/comportementaux globaux.

Conclusions:

Nous avons observé des déficits cognitifs et comportementaux chez une proportion significative de patients atteints de SLP qui étaient comparables à ceux observés chez les patients atteints de SLA. Bien que les déficits n'étaient pas dans l'écart de la démence frontotemporale, tant les patients atteints de SLA que les patients atteints de SLP présentaient des déficits plus évidents aux tests de fonctionnement exécutif.

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Corresponding author

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Neuropsychological Functioning in PLS: A Comparison with ALS

  • Gloria M. Grace (a1), J. B. Orange (a2), Ann Rowe (a3), Karen Findlater (a3), Morris Freedman (a4) and Michael J. Strong (a3)...

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