Buccofacial apraxia in primary progressive aphasia.

Buccofacial apraxia (BFA) is associated with nonfluent/agrammatic variant primary progressive aphasia (nfvPPA) as well as with the severity of apraxia of speech (AOS), a core symptom of nfvPPA. However, an association with agrammatism has not been established. The aim of this study was to examine the association between BFA and agrammatism in nfvPPA and to determine differences in atrophic regions in primary progressive aphasia (PPA) with and without BFA. Seventy-four patients with PPA were recruited, including 34, 15, 10, and 15 patients with nfvPPA, semantic variant PPA, logopenic variant PPA, and unclassified PPA, respectively. All patients underwent language examination and BFA evaluations. Voxel-based morphometry (VBM) was performed to determine whether atrophy of a specific lesion correlated with the presence of BFA. BFA was observed in 20 and 3 patients with nfvPPA and unclassified PPA, respectively. In a comparison of patients with nfvPPA with and without BFA, the BFA group showed significantly worse spontaneous speech and writing in the Western Aphasia Battery. The agrammatism ratio or the ratio of agrammatic errors to the total number of particles was higher in the BFA group; however, the severity of prosodic and phonetic components of AOS did not differ between the two groups. VBM showed that the severity of BFA correlated with atrophy of the opercular and triangular areas of the inferior frontal gyrus to a part of the left middle frontal gyrus. BFA has a different anatomical basis from AOS in patients with nfvPPA and that BFA is characterized by more anterior degeneration compared to that of AOS.

Virtual Reality Augmented Feedback Rehabilitation Associated to Action Observation Therapy in Buccofacial Apraxia: Case Report.

BACKGROUND: Buccofacial Apraxia is defined as the inability to perform voluntary movements of the larynx, pharynx, mandible, tongue, lips and cheeks, while automatic or reflexive control of these structures is preserved. Buccofacial Apraxia frequently co-occurs with aphasia and apraxia of speech and it has been reported as almost exclusively resulting from a lesion of the left hemisphere. Recent studies have demonstrated the benefit of treating apraxia using motor training principles such as Augmented Feedback or Action Observation Therapy. In light of this, the study describes the treatment based on immersive Action Observation Therapy and Virtual Reality Augmented Feedback in a case of Buccofacial Apraxia. PARTICIPANT AND METHODS: The participant is a right-handed 58-years-old male. He underwent a neurosurgery intervention of craniotomy and exeresis of infra axial expansive lesion in the frontoparietal convexity compatible with an atypical meningioma. Buccofacial Apraxia was diagnosed by a neurologist and evaluated by the Upper and Lower Face Apraxia Test. Buccofacial Apraxia was quantified also by a specific camera, with an appropriately developed software, able to detect the range of motion of automatic face movements and the range of the same movements on voluntary requests. In order to improve voluntary movements, the participant completed fifteen 1-hour rehabilitation sessions, composed of a 20-minutes immersive Action Observation Therapy followed by a 40-minutes Virtual Reality Augmented Feedback sessions, 5 days a week, for 3 consecutive weeks. RESULTS: After treatment, participant achieved great improvements in quality and range of facial movements, performing most of the facial expressions (eg, kiss, smile, lateral angle of mouth displacement) without unsolicited movement. Furthermore, the Upper and Lower Face Apraxia Test showed an improvement of 118% for the Upper Face movements and of 200% for the Lower Face movements. CONCLUSION: Performing voluntary movement in a Virtual Reality environment with Augmented Feedbacks, in addition to Action Observation Therapy, improved performances of facial gestures and consolidate the activations by the central nervous system based on principles of experience-dependent neural plasticity.

Buccofacial Apraxia, Oropharyngeal Dysphagia, and Dementia Severity in Community-Dwelling Elderly Patients.

Apraxia occurs frequently in patients with dementia. Buccofacial apraxia (BFA) characteristics have been less investigated than limb or speech apraxia. An association between BFA and oropharyngeal dysphagia (OD) in old patients with dementia has not yet been explored. We aimed to assess the prevalence of BFA in patients with dementia and evaluate the relationship between BFA, OD, and dementia. We have prospectively included 117 outpatients with dementia referred to a geriatric consultation. Oropharyngeal dysphagia was diagnosed using the volume viscosity swallowing test (V-VST). Buccofacial apraxia was evaluated by miming 7 meaningless gestures. A complementary geriatric assessment of 6-domains completed the evaluation. Buccofacial apraxia was present in 54 (48.6%) patients. Proxies reported OD more frequently in the group of patients with BFA compared to the group without (P = .04). Prevalence of OD assessed with the V-VST was similar between patients with and without apraxia (P = .9). Patients with BFA had a significant lower Mini-Mental State Examination suggesting a more severe cognitive decline (18.1 ± 4.5 vs 15.8 ± 5, P = .01), a lower activities of daily living relative to disabilities (5 ± 0.8 vs 4.3 ± 1.3, P = .001), and had a lower gait speed that indicated frailty (P = .03).In conclusion, our results indicate a relationship between BFA and severity of dementia, disability, and frailty with no significant association between BFA and OD.

A Case of Frontotemporal Dementia Presenting with Apraxia of Speech

Apraxia of speech (AOS) is a speech disorder due to an impaired ability to program the positioning of the speech musculature and the sequencing of muscle movements. It differs from dysarthria because speech errors are inconsistent depending on the locus of the sound. We report a 66-year-old man who presented with AOS which evolved into frontotemporal dementia 5 years later. Initial neuropsychological test showed mainly buccofacial apraxia and frontal lobe dysfunction. Brain FDG-PET disclosed hypometabolism in frontotemporal region.