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L’apport critique de l’évaluation de la communication dans le diagnostic précoce de l’apraxie primaire progressive de la parole

  • Liziane Bouvier (a1), Laura Monetta (a1) (a2), Robert Laforce (a3), Louis Verret (a3) and Vincent Martel-Sauvageau (a1) (a4)...

Abstract

Primary progressive apraxia of speech (PPAoS) is a neurodegenerative syndrome characterized by speech apraxia at its onset; as it progresses, it often evolves into total mutism. Even though this syndrome is increasingly recognized, its early differential diagnostic is still complex. The objective of this study was to illustrate why a fine evaluation of speech and language is essential for the differential diagnosis of PPAoS. This longitudinal case study presents the progression of a PPAoS patient over a period of 5 years. Periodic neurological and speech-language assessments were carried out to follow the progression of neurological, memory, language and speech symptoms. The different diagnostic labels established over time were also reported. The evolution of the patient’s communication profile was characterized by a preservation of language components and episodic memory, in parallel with a progressive deterioration of speech which gradually reduced intelligibility, and was associated with signs of spasticity, resulting in a complete anarthria. This case study sheds light upon the evolution of a patient with PPAoS. A better understanding of the clinical profile and progression of PPAoS is necessary in order to improve early diagnosis and adequate care for these patients.

L’apraxie primaire progressive de la parole (APPP) est un syndrome neurodégénératif dans lequel l’apraxie de la parole est le symptôme initial, évoluant souvent jusqu’à un mutisme total. Malgré une reconnaissance grandissante de ce syndrome, son diagnostic différentiel précoce demeure complexe. L’objectif de cette étude est d’illustrer l’importance de l’évaluation fine de la parole et du langage dans l’établissement du diagnostic différentiel de l’APPP. Cette étude de cas unique présente de façon longitudinale le parcours évolutif d’un cas d’APPP pendant 5 ans. Des évaluations périodiques en neurologie et en orthophonie permettent de suivre l’évolution des aspects neurologiques, de la mémoire, du langage et de la parole. Les différentes étiquettes diagnostiques posées à travers le temps sont également rapportées. L’évolution du profil communicationnel de la patiente est caractérisée par une préservation des composantes langagières ainsi que de la mémoire épisodique, parallèlement à l’atteinte progressive de la parole, se traduisant par une réduction grandissante de l’intelligibilité et par des indices de spasticité, évoluant jusqu’à une anarthrie complète. Cette étude de cas met en lumière un exemple d’évolution d’APPP. Une meilleure connaissance du profil clinique et évolutif de l’APPP facilitera le diagnostic précoce et la prise en charge adéquate de ces patients.

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Corresponding author

La correspondance et les demandes de tirés à part doivent être adressées à : / Correspondence and requests for offprints should be sent to: Vincent Martel Sauvageau, Ph.D. Programme de maîtrise en orthophonie Département de réadaptation Université Laval, 1050 avenue de la médecine bureau VND-4477 Québec, QC G1V 0A6 (Vincent.Martel-Sauvageau@fmed.ulaval.ca)

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Les auteurs tiennent à remercier GS pour sa collaboration indéfectible aux évaluations, de même que les équipes de la CEO et de la Clinique Interdisciplinaire de Mémoire du CHU de Québec. Cette recherche n’a pas reçu de financement spécifique de la part d’agences publiques, commerciales ou caritatives. Les auteurs n’ont pas de conflit d’intérêt à déclarer.

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References

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