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PURPOSE: The purpose of this study was to investigate acoustic spatiotemporal variability in children with childhood apraxia of speech (CAS) compared to children with typical development (TD). Increased spatiotemporal variability in children with CAS relative to nonapraxic peers has been documented in multiple kinematic studies. To date, few studies have investigated spatiotemporal variability in CAS using an acoustic signal. METHOD: Data were drawn from 10 children with CAS and 10 children with TD, ranging in age from 5;0 to 11;2 (years;months), who participated in an online study. All children with CAS had a verified diagnosis and showed at least five CAS features across at least two tasks, independently confirmed by two speech-language pathologists with expertise in CAS. Children repeated the sentences "Buy Bobby a puppy" (BBAP) and "Mom pets the puppy" (MPP) 10 times each. The acoustic spatiotemporal index (STI), calculated from the amplitude envelope, was used to investigate acoustic spatiotemporal variability. Using a regression analysis, we analyzed group differences in STI values while controlling for age and gender. RESULTS: Children with CAS did not produce enough usable tokens of BBAP to analyze. MPP revealed significantly higher acoustic STI values in children with CAS compared to children with TD. No significant effects were observed for age or gender. Acoustic data collected online without the use of specialized equipment yielded high-quality audio data from which amplitude envelope tracing could be reliably accomplished. CONCLUSIONS: This study adds to a growing body of empirical data indicating that children with CAS produce speech with more variable spatiotemporal control compared to children with TD. In addition to producing more variable speech, children with CAS were less consistently able to form productions free of phonetic errors. These findings also demonstrate the feasibility of using remote data collection to investigate acoustic spatiotemporal variability, which allows for the recruitment of larger samples of low-incidence populations. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.26814883.
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Apraxias , Acústica del Lenguaje , Humanos , Masculino , Femenino , Apraxias/fisiopatología , Preescolar , Niño , Medición de la Producción del Habla/métodos , Trastornos del Habla/fisiopatologíaRESUMEN
BACKGROUND: Biallelic SUFU variants have originally been linked to Joubert syndrome, comprising cerebellar abnormalities, dysmorphism, and polydactyly. In contrast, heterozygous truncating variants have recently been associated with developmental delay and ocular motor apraxia, but only a limited number of patients have been reported. Here, we aim to delineate further the mild end of the phenotypic spectrum related to SUFU haploinsufficiency. METHODS: Nine individuals (from three unrelated families) harboring truncating SUFU variants were investigated, including two previously reported individuals (from one family). We provide results from a comprehensive assessment comprising neuroimaging, neuropsychology, video-oculography, and genetic testing. RESULTS: We identified three inherited or de novo truncating variants in SUFU (NM_016169.4): c.895C>T p.(Arg299∗), c.71dup p.(Ala25Glyfs∗23), and c.71del p.(Pro24Argfs∗72). The phenotypic expression showed high variability both between and within families. Clinical features include motor developmental delay (seven of nine), axial hypotonia (five of nine), ocular motor apraxia (three of nine), and cerebellar signs (three of nine). Four of the six reported children had macrocephaly. Neuropsychological and developmental assessments revealed mildly delayed language development in the youngest children, whereas general cognition was normal in all variant carriers. Subtle but characteristic SUFU-related neuroimaging abnormalities (including superior cerebellar dysplasia, abnormalities of the superior cerebellar peduncles, rostrally displaced fastigium, and vermis hypoplasia) were observed in seven of nine individuals. CONCLUSIONS: Our data shed further light on the mild but recognizable features of SUFU haploinsufficiency and underline its marked phenotypic variability, even within families. Notably, neurodevelopmental and behavioral abnormalities are mild compared with Joubert syndrome and seem to be well compensated over time.
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Discapacidades del Desarrollo , Haploinsuficiencia , Fenotipo , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Anomalías Múltiples/genética , Anomalías Múltiples/diagnóstico por imagen , Anomalías Múltiples/fisiopatología , Apraxias/diagnóstico por imagen , Apraxias/genética , Apraxias/fisiopatología , Apraxias/congénito , Cerebelo/diagnóstico por imagen , Cerebelo/anomalías , Síndrome de Cogan , Discapacidades del Desarrollo/diagnóstico por imagen , Discapacidades del Desarrollo/genética , Discapacidades del Desarrollo/etiología , Discapacidades del Desarrollo/fisiopatología , Anomalías del Ojo/genética , Anomalías del Ojo/diagnóstico por imagen , Enfermedades Renales Quísticas/genética , Enfermedades Renales Quísticas/diagnóstico por imagen , Neuroimagen , Retina/diagnóstico por imagen , Retina/anomalías , Proteínas Represoras/genética , Proteínas Represoras/metabolismoRESUMEN
We identified a syndrome characterized by a relatively isolated progressive impairment of reading words that the patient was able to understand and repeat but without other components of speech apraxia. This cluster of symptoms fits a new syndrome designated Progressive Verbal Apraxia of Reading. A right-handed man (AB) came with a 2.5-year history of increasing difficulties in reading aloud. He was evaluated twice, 2 years apart, using multimodal neuroimaging techniques and quantitative neurolinguistic assessment. In the laboratory, reading difficulties arose in the context of intact visual and auditory word recognition as well as intact ability to understand and repeat words he was unable to read aloud. The unique feature was the absence of dysarthria or speech apraxia in tasks other than reading. Initial imaging did not reveal statistically significant atrophy. Structural magnetic resonance and FDG-PET imaging at the second assessment revealed atrophy and hypometabolism in the right posterior cerebellum, in areas shown to be part of his language network by task-based functional neuroimaging at initial assessment. This syndromic cluster can be designated Progressive Verbal Apraxia of Reading, an entity that has not been reported previously to the best of our knowledge. We hypothesize a selective disconnection of the visual word recognition system from the otherwise intact articulatory apparatus, a disconnection that appears to reflect the disruption of multisynaptic cerebello-cortical circuits.
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Apraxias , Imagen por Resonancia Magnética , Tomografía de Emisión de Positrones , Lectura , Humanos , Masculino , Apraxias/fisiopatología , Apraxias/diagnóstico por imagen , Tomografía de Emisión de Positrones/métodos , Persona de Mediana Edad , Atrofia/patología , Pruebas Neuropsicológicas , Encéfalo/diagnóstico por imagen , Encéfalo/fisiopatología , Encéfalo/patologíaRESUMEN
Patients who have a yes-no reversal respond "yes" when they mean no and vice versa. The unintentional response can be made both verbally and with gestures (e.g., head shake or nod, thumbs up or down). Preliminary reports associate this phenomenon with 4-repeat tauopathies including primary progressive apraxia of speech (PPAOS), nonfluent/agrammatic primary progressive aphasia, and corticobasal syndrome; however, the significance and timing of this symptom relative to others are not well understood. Whereas some accounts associate yes-no reversals with other binary reversals (e.g., up/down, hot/cold) and attribute the reversals to disturbances of selection within the language system, others implicate more general inhibitory control processes. Here, we compared clinical and neuroimaging findings across 30 patients with PPAOS (apraxia of speech in the absence of aphasia), 15 of whom had a yes-no reversal complaint and 15 who did not. The two groups did not differ on any of the language or motor speech measures; however, patients who had the yes-no reversal received lower scores on the Frontal Assessment Battery and motor assessments. They also had greater hypometabolism in the left supplementary motor area and bilateral caudate nuclei on [18F]-fluorodeoxyglucose PET, but only the right caudate nucleus cluster survived correction for multiple comparisons. We interpret these results to suggest that the yes-no reversal phenomenon is associated with cognitive abilities that are supported by the frontostriatal network; more specifically, impaired response inhibition.
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Apraxias , Humanos , Masculino , Femenino , Anciano , Persona de Mediana Edad , Apraxias/fisiopatología , Habla/fisiología , Tomografía de Emisión de Positrones , Pruebas Neuropsicológicas , Afasia Progresiva Primaria/fisiopatología , Afasia Progresiva Primaria/diagnóstico por imagen , Imagen por Resonancia Magnética , Encéfalo/fisiopatología , Encéfalo/diagnóstico por imagenAsunto(s)
ADN Helicasas , Enzimas Multifuncionales , ARN Helicasas , Humanos , Enzimas Multifuncionales/genética , ARN Helicasas/genética , Masculino , ADN Helicasas/genética , Femenino , Apraxias/genética , Apraxias/fisiopatología , Apraxias/congénito , Síndrome de Cogan/genética , Adulto , Linaje , Ataxias Espinocerebelosas/genética , Ataxias Espinocerebelosas/congénito , Ataxias Espinocerebelosas/patología , MutaciónRESUMEN
PURPOSE: This study examines whether there are differences in the speech of speakers with dysarthria, speakers with apraxia and healthy speakers in spectral acoustic measures during production of the central-peninsular Spanish alveolar sibilant fricative /s/. METHOD: To this end, production of the sibilant was analyzed in 20 subjects with dysarthria, 8 with apraxia of speech and 28 healthy speakers. Participants produced 12 sV(C) words. The variables compared across groups were the fricative's spectral amplitude difference (AmpD) and spectral moments in the temporal midpoint of fricative execution. RESULTS: The results indicate that individuals with dysarthria can be distinguished from healthy speakers in terms of the spectral characteristics AmpD, standard deviation (SD), center of gravity (CoG) and skewness, the last two in context with unrounded vowel, while no differences in kurtosis were detected. Participants with AoS group differ significantly from healthy speaker group in AmpD, SD and CoG and Kurtosis, the first one followed unrounded vowel and the latter two followed by rounded vowels. In addition, speakers with apraxia of speech group returned significant differences with respect to speakers with dysarthria group in AmpD, CoG and skewness. CONCLUSIONS: The differences found between the groups in the measures studied as a function of the type of vowel context could provide insights into the distinctive manifestations of motor speech disorders, contributing to the differential diagnosis between apraxia and dysarthria in motor control processes.
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Apraxias , Disartria , Acústica del Lenguaje , Humanos , Disartria/fisiopatología , Disartria/etiología , Apraxias/fisiopatología , Masculino , Femenino , Persona de Mediana Edad , Adulto , Anciano , Fonética , Medición de la Producción del HablaRESUMEN
Limb apraxia is a motor disorder frequently observed following a stroke. Apraxic deficits are classically assessed with four tasks: tool use, pantomime of tool use, imitation, and gesture understanding. These tasks are supported by several cognitive processes represented in a left-lateralized brain network including inferior frontal gyrus, inferior parietal lobe (IPL), and lateral occipito-temporal cortex (LOTC). For the past twenty years, voxel-wise lesion symptom mapping (VLSM) studies have been used to unravel the neural correlates associated with apraxia, but none of them has proposed a comprehensive view of the topic. In the present work, we proposed to fill this gap by performing a systematic Anatomic Likelihood Estimation meta-analysis of VLSM studies which included tasks traditionally used to assess apraxia. We found that the IPL was crucial for all the tasks. Moreover, lesions within the LOTC were more associated with imitation deficits than tool use or pantomime, confirming its important role in higher visual processing. Our results questioned traditional neurocognitive models on apraxia and may have important clinical implications.
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Apraxias , Humanos , Apraxias/fisiopatología , Apraxias/diagnóstico por imagen , Apraxias/etiología , Apraxias/patología , Mapeo Encefálico , Encéfalo/fisiopatología , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Funciones de Verosimilitud , Lesiones Encefálicas/fisiopatología , Lesiones Encefálicas/patología , Lesiones Encefálicas/diagnóstico por imagen , Accidente Cerebrovascular/fisiopatología , Accidente Cerebrovascular/diagnóstico por imagen , Accidente Cerebrovascular/patología , Accidente Cerebrovascular/complicacionesRESUMEN
BACKGROUND AND OBJECTIVES: Nonfluent variant primary progressive aphasia (nfvPPA) and primary progressive apraxia of speech (PPAOS) can be precursors to corticobasal syndrome (CBS). Details on their progression remain unclear. We aimed to examine the clinical and neuroimaging evolution of nfvPPA and PPAOS into CBS. METHODS: We conducted a retrospective longitudinal study in 140 nfvPPA or PPAOS patients and applied the consensus criteria for possible and probable CBS for every visit, evaluating limb rigidity, akinesia, limb dystonia, myoclonus, ideomotor apraxia, alien limb phenomenon, and nonverbal oral apraxia (NVOA). Given the association of NVOA with AOS, we also modified the CBS criteria by excluding NVOA and assigned every patient to either a progressors or non-progressors group. We evaluated the frequency of every CBS feature by year from disease onset, and assessed gray and white matter volume loss using SPM12. RESULTS: Asymmetric akinesia, NVOA, and limb apraxia were the most common CBS features that developed; while limb dystonia, myoclonus, and alien limb were rare. Eighty-two patients progressed to possible CBS; only four to probable CBS. nfvPPA and PPAOS had a similar proportion of progressors, although nfvPPA progressed to CBS earlier (p-value = 0.046), driven by an early appearance of limb apraxia (p-value = 0.0041). The non-progressors and progressors both showed premotor/motor cortex involvement at baseline, with spread into prefrontal cortex over time. DISCUSSION: An important proportion of patients with nfvPPA and PPAOS progress to possible CBS, while they rarely develop features of probable CBS even after long follow-up.
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Apraxias , Progresión de la Enfermedad , Afasia Progresiva Primaria no Fluente , Humanos , Masculino , Femenino , Estudios Longitudinales , Anciano , Persona de Mediana Edad , Apraxias/etiología , Apraxias/fisiopatología , Apraxias/diagnóstico por imagen , Estudios Retrospectivos , Afasia Progresiva Primaria no Fluente/fisiopatología , Afasia Progresiva Primaria no Fluente/diagnóstico por imagen , Imagen por Resonancia MagnéticaRESUMEN
PURPOSE: Neurogenic speech and language disorders-such as acquired apraxia of speech (AOS) and aphasia with phonemic paraphasia (APP)-are often misdiagnosed due to similarities in clinical presentation. Word syllable duration (WSD)-a measure of average syllable length in multisyllabic words-serves as a proxy for speech rate, which is an important and arguably more objective clinical characteristic of AOS and APP. This study reports stability of WSD over time for speakers with AOS (and aphasia). METHOD: Twenty-nine participants with AOS and aphasia (11 women and 18 men, Mage = 53.5 years, SD = 13.3) repeated 30 multisyllabic words (of three-, four-, and five-syllable lengths) on three occasions across 4 weeks. WSDs were calculated for each word and then averaged across each list (i.e., word length), as well as across combined lists (i.e., all 30 words) to yield four WSDs for each participant at each time point. Stability over time was calculated using Friedman's test for the group and using Spearman's rho for the individual level. Effects of time and word length were examined using robust mixed-effects linear regression. RESULTS: Friedman's tests and correlations indicated no significant difference in WSDs across sampling occasions for each word length separately or combined. WSD correlated positively with AOS severity and negatively with intelligibility but was not correlated with aphasia severity. Regression analyses confirmed WSD to be stable over time, while WSD calculated from only five tokens (i.e., WSD-5) was less stable over time. CONCLUSIONS: Results indicate that WSD can be a stable measure over time, at the individual and group level, providing support for its use in diagnosis and/or as an outcome measure, both clinically and for research. In general, WSD outperformed WSD-5, suggesting that it may be better to calculate WSD from more than five tokens. Stability of WSD in other populations and suitability for differential diagnosis need to be determined. Currently, differentiating disorders by speaking rate, alone, is not recommended. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.25438735.
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Afasia , Apraxias , Humanos , Masculino , Femenino , Persona de Mediana Edad , Apraxias/diagnóstico , Apraxias/fisiopatología , Afasia/diagnóstico , Afasia/etiología , Anciano , Adulto , Medición de la Producción del Habla/métodos , Habla/fisiología , Factores de Tiempo , FonéticaRESUMEN
OBJECTIVE: Resection of glioma in the nondominant hemisphere involving the motor areas and pathways requires the use of brain-mapping techniques to spare essential sites subserving motor control. No clear indications are available for performing motor mapping under either awake or asleep conditions or for the best mapping paradigm (e.g., resting or active, high-frequency [HF] or low-frequency [LF] stimulation) that provides the best oncological and functional outcomes when tailored to the clinical context. This work aimed to identify clinical and imaging factors that influence surgical strategy (asleep motor mapping vs awake motor mapping) and that are associated with the best functional and oncological outcomes and to design a "motor mapping score" for guiding tumor resection in this area. METHODS: The authors evaluated a retrospective series of patients with nondominant-hemisphere glioma-located or infiltrating within 2 cm anteriorly or posteriorly to the central sulcus and affecting the primary motor cortex, its fibers, and/or the praxis network-who underwent operations with asleep (HF monopolar probe) or awake (LF and HF probes) motor mapping. Clinical and imaging variables were used to design a motor mapping score. A prospective series of patients was used to validate this motor mapping score. RESULTS: One hundred thirty-five patients were retrospectively analyzed: 69 underwent operations with asleep (HF stimulation) motor mapping, and 66 underwent awake (LF and HF stimulation and praxis task evaluation) motor mapping. Previous motor (strength) deficit, previous treatment (surgery/radiotherapy), tumor volume > 30 cm3, and tumor involvement of the praxis network (on MRI) were identified and used to design the mapping score. Motor deficit, previous treatment, and location within or close to the central sulcus favor use of asleep motor mapping; large tumor volume and involvement of the praxis network favor use of awake motor mapping. The motor mapping score was validated in a prospective series of 52 patients-35 underwent operations with awake motor mapping and 17 with asleep motor mapping on the basis of the score indications-who had a low rate of postoperative motor-praxis deficit (3%) and a high extent of resection (median 97%; complete resection in > 70% of patients). CONCLUSIONS: Extensive resection of tumor involving the eloquent areas for motor control is feasible, and when an appropriate mapping strategy is applied, the incidence of postoperative motor-praxis deficit is low. Asleep (HF stimulation) motor mapping is preferable for lesions close to or involving the central sulcus and/or in patients with preoperative strength deficit and/or history of previous treatment. When a patient has no motor deficit or previous treatment and has a lesion (> 30 cm3) involving the praxis network, awake mapping is preferable.
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Mapeo Encefálico/métodos , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/cirugía , Glioma/diagnóstico por imagen , Glioma/cirugía , Procedimientos Neuroquirúrgicos/métodos , Sueño , Vigilia , Adolescente , Adulto , Anciano , Apraxias/etiología , Apraxias/fisiopatología , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Corteza Motora/diagnóstico por imagen , Corteza Motora/cirugía , Planificación de Atención al Paciente , Complicaciones Posoperatorias/fisiopatología , Reproducibilidad de los Resultados , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Alzheimer's disease (AD) affects several cognitive functions and causes altered motor function. Fine motor deficits during object manipulation are evident in other neurological conditions, but have not been assessed in dementia patients yet. OBJECTIVE: Investigate reactive and anticipatory grip force control in response to unexpected and expected load force perturbation in AD. METHODS: Reactive and anticipatory grip force was investigated using a grip-device with force sensors. In this pilot study, fifteen AD patients and fourteen healthy controls performed a catching task. They held the device with one hand while a sandbag was dropped into an attached receptacle either by the experimenter or by the participant. RESULTS: In contrast to studies of other neurological conditions, the majority of AD patients exerted lower static grip force levels than controls. Interestingly, patients who were slow in the Luria's three-step test produced normal grip forces. The timing and magnitude of reactive grip force control were largely preserved in patients. In contrast, timing and extent of anticipatory grip forces were impaired in patients, although anticipatory control was generally preserved. These deficits were correlated with decreasing Mini-Mental State Examination scores. Apraxia scores, assessed by pantomime of tool-use, did not correlate with performance in the catching task. CONCLUSION: We interpreted the decreased grip force in AD in the context of loss of strength and lethargy, typical for patients with AD. The lower static grip force during object manipulation may emerge as a potential biomarker for early stages of AD, but more studies with larger sample sizes are necessary.
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Enfermedad de Alzheimer/fisiopatología , Fuerza de la Mano/fisiología , Desempeño Psicomotor/fisiología , Anciano , Anciano de 80 o más Años , Apraxias/fisiopatología , Femenino , Humanos , Masculino , Pruebas de Estado Mental y Demencia/estadística & datos numéricos , Persona de Mediana Edad , Proyectos PilotoRESUMEN
We report a case series of children with childhood apraxia of speech, by describing behavioral and white matter microstructural changes following 2 different treatment approaches.Five children with childhood apraxia of speech were assigned to a motor speech treatment (PROMPT) and 5 to a language, nonspeech oral motor treatment. Speech assessment and brain MRI were performed pre- and post-treatment. The ventral (tongue/larynx) and dorsal (lips) corticobulbar tracts were reconstructed in each subject. Mean fractional anisotropy and mean diffusivity were extracted. The hand corticospinal tract was assessed as a control pathway. In both groups speech improvements paralleled changes in the left ventral corticobulbar tract fractional anisotropy. The PROMPT treated group also showed fractional anisotropy increase and mean diffusivity decrease in the left dorsal corticobulbar tract. No changes were detected in the hand tract. Our results may provide preliminary support to the possible neurobiologic effect of a multimodal speech motor treatment in childhood apraxia of speech.
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Apraxias/fisiopatología , Apraxias/terapia , Mapeo Encefálico/métodos , Imagen por Resonancia Magnética/métodos , Logopedia/métodos , Niño , Preescolar , Femenino , Humanos , Masculino , Resultado del TratamientoRESUMEN
OBJECTIVE: To better characterize children with glioblastoma, assess outcomes, and identify prognostic factors associated with overall survival and progression-free survival in a relatively large cohort from a single institution. METHODS: For this retrospective review, 38 pediatric patients with a diagnosis of glioblastoma who were treated at The First Affiliated Hospital of Zhengzhou University between January 2015 and January 2020 were selected. Clinical and pathological characteristics, imaging, treatment, and survival variables were compared. RESULTS: There were 24 boys and 14 girls with a median age of 11.5 years (range, 3-18 years). All patients underwent surgery, with gross total resection in 16 and subtotal resection in 22. Of patients, 18 received radiation combined with chemotherapy, 6 received radiation or chemotherapy alone, and 14 did not receive any adjuvant therapy. Contrast-enhanced magnetic resonance imaging of 21 patients showed rim enhancement, while heterogeneous enhancement was shown on imaging of the other 17 patients. Tumors were observed in hemispheric locations in 19 cases and in central locations in the others. Median overall survival was 10.5 months with a median progression-free survival of 6 months. Extent of resection, adjuvant therapy, and original site of tumor were identified as independent predictors for progression-free survival and overall survival on multivariate analysis. There were significant differences in prognosis among different enhancement characteristics; patients with rim-enhancing tumors had a better prognosis. CONCLUSIONS: Pediatric glioblastoma carries a dismal prognosis. Maximum safe resection followed by adjuvant radiation with chemotherapy is considered standard treatment. Better outcomes are associated with hemispheric tumor locations and rim enhancement on magnetic resonance imaging.
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Neoplasias Encefálicas/terapia , Quimioradioterapia Adyuvante , Glioblastoma/terapia , Procedimientos Neuroquirúrgicos , Adolescente , Antineoplásicos Alquilantes/uso terapéutico , Apraxias/fisiopatología , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/fisiopatología , Quimioterapia Adyuvante , Niño , Preescolar , Femenino , Glioblastoma/diagnóstico por imagen , Glioblastoma/fisiopatología , Humanos , Hipertensión Intracraneal/fisiopatología , Isocitrato Deshidrogenasa/genética , Imagen por Resonancia Magnética , Masculino , Pronóstico , Supervivencia sin Progresión , Radioterapia Adyuvante , Estudios Retrospectivos , Convulsiones/fisiopatología , Tasa de Supervivencia , Temozolomida/uso terapéutico , Resultado del TratamientoRESUMEN
INTRODUCTION: Apraxia is a core clinical feature of corticobasal syndrome (CBS). Among the subtypes of apraxia, ideomotor and imitation apraxia are frequently found in CBS. However, little is known about the brain networks that are characteristic of each apraxia subtype or their clinical implication. In this study, we used 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) to explore the specific patterns of glucose hypometabolism that are characteristic of apraxia subtypes by focusing on ideomotor and imitation apraxia. METHODS: We compared the areas of glucose hypometabolism in the brains of 52 patients with CBS and 13 healthy controls, both as a whole and according to apraxia subtypes. In addition, we investigated the relationship between the apraxia subtypes and the clinical phenotype of CBS. RESULTS: In patients with CBS, common hypometabolism was observed in the frontal gyrus, precentral gyrus and caudate regardless of apraxia subtypes. In particular, ideomotor apraxia was associated with hypometabolism in the angular gyrus, while imitation apraxia was associated with hypometabolism in the posterior part including the postcentral gyrus, precuneus, and posterior cingulate gyrus. Patients who showed both ideomotor and imitation apraxia were more likely to show the typical features of CBS and progressive supranuclear palsy compared with patients showing only one type of apraxia. CONCLUSION: Group comparison analysis using FDG-PET revealed distinct pathways of ideomotor and imitation apraxia in CBS. These findings add to our understanding of the brain networks underlying apraxia in association with the clinical features of CBS.
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Apraxias/fisiopatología , Núcleo Caudado/fisiopatología , Corteza Cerebral/fisiopatología , Degeneración Corticobasal/fisiopatología , Conducta Imitativa , Red Nerviosa/fisiopatología , Anciano , Apraxia Ideomotora/diagnóstico por imagen , Apraxia Ideomotora/etiología , Apraxia Ideomotora/metabolismo , Apraxia Ideomotora/fisiopatología , Apraxias/diagnóstico por imagen , Apraxias/etiología , Apraxias/metabolismo , Núcleo Caudado/diagnóstico por imagen , Núcleo Caudado/metabolismo , Corteza Cerebral/diagnóstico por imagen , Corteza Cerebral/metabolismo , Degeneración Corticobasal/complicaciones , Degeneración Corticobasal/diagnóstico por imagen , Degeneración Corticobasal/metabolismo , Femenino , Fluorodesoxiglucosa F18 , Humanos , Masculino , Persona de Mediana Edad , Red Nerviosa/diagnóstico por imagen , Red Nerviosa/metabolismo , Tomografía de Emisión de PositronesRESUMEN
The aim of this paper is to develop further the idea that symptoms that emerge in speech and language processing following brain damage can make a contribution to discussions of the early evolution of language. These diverse impairments are called aphasia, and this paper proposes that the recovery of a non-fluent aphasia syndrome following stroke could provide insights into the course of the pre-history of human language evolution. The observable symptoms emerge during recovery, crucially enabled by (dis)inhibition in parallel with a range of impairments in action processing (apraxias), including apraxia of speech. They are underpinned by changes in cortical and subcortical status following brain damage. It is proposed that the observed recovery mimics ontogenic and phylogenic processes in human speech and language. The arguments put forward provide insights tending to support the motor-gestural model of speech and language evolution. This article is part of the theme issue 'Reconstructing prehistoric languages'.
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Afasia/fisiopatología , Apraxias/fisiopatología , Encefalopatías/fisiopatología , Evolución Cultural , Lenguaje , Habla , Lesiones Encefálicas/fisiopatología , HumanosRESUMEN
OBJECTIVE: To analyze the effectiveness of a home-based restorative and compensatory upper limb apraxia (ULA) rehabilitation program. DESIGN: Randomized controlled trial. SETTING: Neurology Unit of San Cecilio Hospital and 2 private and specialized health care centers. PARTICIPANTS: Community dwelling participants (N=38) between the ages of 25 and 95 years old (sex ratio, 1:1) with unilateral mild-to-moderate poststroke lesions (time of evolution since stroke, 12.03±8.98mo) and secondary ULA. INTERVENTIONS: Participants were randomly assigned to an 8-week combined ULA functional rehabilitation group (n=19) 3 days per week for 30 minutes or to a traditional health care education protocol group (n=19) once a month for 8 weeks. Both interventions were conducted at home. MAIN OUTCOME MEASURES: Sociodemographic and clinical data, Barthel Index (primary outcome), Lawton and Brody Scale, observation and scoring activities of daily living, the De Renzi tests for ideational and ideomotor apraxia and imitating gestures test, recognition of gestures, test for upper limb apraxia , and stroke-specific quality of life scale were assessed at 3 time points: baseline, posttreatment (8wk), and follow-up (8wk). RESULTS: There were statistically significant differences among the groups regarding ideomotor apraxia, imitating gestures, global recognition of gestures, intransitive gestures, and comprehension of gesture production (P<.05) in favor of the experimental group. However, no statistically significant differences were found between the groups regarding functionality or quality of life (P>.05). Regarding the within-group effect, statistically significant differences were found in all neuropsychological outcomes at posttreatment and follow-up (P<.05). CONCLUSION: A functional rehabilitation program was found to be superior to a traditional health care education program and resulted in improvements in neuropsychological functioning in ULA poststroke. Conventional education showed an insufficient effect on apraxia recovery. Further studies with larger sample sizes are needed to determine the effect of rehabilitation strategies on functionality and quality of life of poststroke ULA patients.
Asunto(s)
Apraxias/fisiopatología , Apraxias/rehabilitación , Rehabilitación de Accidente Cerebrovascular/métodos , Extremidad Superior/fisiopatología , Actividades Cotidianas , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Calidad de VidaRESUMEN
Children with autism spectrum disorder (ASD) have difficulties perceiving and producing skilled gestures, or praxis. The inferior parietal lobule (IPL) is crucial to praxis acquisition and expression, yet how IPL connectivity contributes to autism-associated impairments in praxis as well as social-communicative skill remains unclear. Using resting-state functional magnetic resonance imaging, we applied independent component analysis to test how IPL connectivity relates to praxis and social-communicative skills in children with and without ASD. Across all children (with/without ASD), praxis positively correlated with connectivity of left posterior-IPL with the left dorsal premotor cortex and with the bilateral posterior/medial parietal cortex. Praxis also correlated with connectivity of right central-IPL connectivity with the left intraparietal sulcus and medial parietal lobe. Further, in children with ASD, poorer praxis and social-communicative skills both correlated with weaker right central-IPL connectivity with the left cerebellum, posterior cingulate, and right dorsal premotor cortex. Our findings suggest that IPL connectivity is linked to praxis development, that contributions arise bilaterally, and that right IPL connectivity is associated with impaired praxis and social-communicative skills in autism. The findings underscore the potential impact of IPL connectivity and impaired skill acquisition on the development of a range of social-communicative and motor functions during childhood, including autism-associated impairments.
Asunto(s)
Apraxias/diagnóstico por imagen , Trastorno del Espectro Autista/diagnóstico por imagen , Cerebelo/diagnóstico por imagen , Giro del Cíngulo/diagnóstico por imagen , Corteza Motora/diagnóstico por imagen , Lóbulo Parietal/diagnóstico por imagen , Habilidades Sociales , Apraxias/fisiopatología , Trastorno del Espectro Autista/fisiopatología , Encéfalo/diagnóstico por imagen , Encéfalo/fisiopatología , Estudios de Casos y Controles , Cerebelo/fisiopatología , Niño , Femenino , Neuroimagen Funcional , Gestos , Giro del Cíngulo/fisiopatología , Humanos , Imagen por Resonancia Magnética , Masculino , Corteza Motora/fisiopatología , Vías Nerviosas/diagnóstico por imagen , Vías Nerviosas/fisiopatología , Lóbulo Parietal/fisiopatologíaRESUMEN
Hand function and apraxia are equally relevant to neurosurgeons: as a symptom, as well as through the functional anatomy of "praxis" which underlies the dexterity needed for neurosurgical practice. The supplementary motor area is crucial for its understanding. Historically, Hugo Liepmann dominated the apraxia debate at the beginning of the twentieth century, a debate that has remained influential until today. Kurt Goldstein, a contemporary of Liepmann, is regularly mentioned as the first to have described the alien hand syndrome in 1909. Wilder Penfield was a key figure in exploring the role of the fronto-mesial cortex in human motor control and coined the term "supplementary motor area". It was Goldstein who not only contributed substantially to the apraxia debate more than 100 years ago; he also established the link between the dysfunction of the fronto-mesial cortex and abnormal higher motor control in humans.
Asunto(s)
Apraxias/patología , Corteza Motora/patología , Apraxias/fisiopatología , Cuerpo Calloso/patología , Cuerpo Calloso/fisiopatología , Femenino , Historia del Siglo XX , Humanos , Masculino , Corteza Motora/fisiopatologíaRESUMEN
This study investigated the underlying nature of apraxia of speech (AOS) by testing two competing hypotheses. The Reduced Buffer Capacity Hypothesis argues that people with AOS can plan speech only one syllable at a time Rogers and Storkel [1999. Planning speech one syllable at a time: The reduced buffer capacity hypothesis in apraxia of speech. Aphasiology, 13(9-11), 793-805. https://doi.org/10.1080/026870399401885]. The Program Retrieval Deficit Hypothesis states that selecting a motor programme is difficult in face of competition from other simultaneously activated programmes Mailend and Maas [2013. Speech motor programming in apraxia of speech: Evidence from a delayed picture-word interference task. American Journal of Speech-Language Pathology, 22(2), S380-S396. https://doi.org/10.1044/1058-0360(2013/12-0101)]. Speakers with AOS and aphasia, aphasia without AOS, and unimpaired controls were asked to prepare and hold a two-word utterance until a go-signal prompted a spoken response. Phonetic similarity between target words was manipulated. Speakers with AOS had longer reaction times in conditions with two similar words compared to two identical words. The Control and the Aphasia group did not show this effect. These results suggest that speakers with AOS need additional processing time to retrieve target words when multiple motor programmes are simultaneously activated.