Afasia primaria progresiva variante no fluente/agramática / Non-fluent/agrammatic variant of primary progressive aphasia

Introducción: Entre las enfermedades neurodegenerativas se encuentra un grupo de patologías que se caracterizan por un compromiso prominente del lenguaje, denominadas usualmente afasias primarias progresivas, las cuales se subdividen en 3 tipos: variante logopénica, variante semántica y variante no fluente o agramática. Presentación del caso: Paciente con cuadro clínico que inicia a los 65 años, con disminución en la interacción social. Un par de meses después, la esposa nota que el lenguaje del paciente se torna poco fluido, habla con palabras o frases cortas, no logra decir oraciones completas, además de presentar cambios en la entonación de las palabras y alteraciones del lenguaje escrito. El paciente manifiesta que su principal limitación en el momento es el no poder expresar lo que quiere decir, y por este motivo consulta. Discusión: En el caso de este paciente, se describe inicialmente un cambio en su personalidad que no compromete su funcionalidad, sin embargo, al poco tiempo se presenta compromiso del lenguaje como síntoma prominente y que genera mayor compromiso en su calidad de vida, con pruebas neuropsicológicas y hallazgos de neuroimagen que apoyan el diagnóstico de afasia primaria progresiva (APP) variante no fluente o agramatical, con síntomas comportamentales y motores asociados. Conclusión: Las APP son un grupo de trastornos neurocognitivos cuya característica primordial es el compromiso en el lenguaje, cada variante de APP tiene unas características clínicas y criterios diagnósticos específicos que se deben conocer para lograr sospechar el diagnóstico y hacer un abordaje apropiado en el paciente.

Introduction: In the group of neurodegenerative diseases, there is a group of pathologies that are characterized by a prominent compromise of language, normally called primary progressive aphasias, these are subdivided into 3 types: logopenic variant, semantic variant and non-fluent or agrammatic variant. Case presentation: Patient with a clinical picture that begins at age 65, with decreased social interaction, a couple of months later his wife notices that his language becomes not fluent, speaks in short words or phrases, cannot say complete sentences, in addition to changes in the intonation of words and alterations in written language, the patient states that his main limitation at the moment is not being able to express what he wants to say and for this reason they consult. Discussion: In the case of this patient, a change in his personality is initially described that does not compromise his functionality, however soon after a language involvement is presented as the main symptom and the one that generates a compromise in his quality of life, with neuropsychological tests and findings on neuroimaging that supports the diagnosis of primary progressive aphasia (PPA) non-fluent or agrammatical variant, with associated behavioral and motor symptoms. Conclusion: APPs are a group of neurocognitive disorders whose primary characteristic is language impairment. Each APP variant has specific clinical characteristics and diagnostic criteria that must be known in order to suspect the diagnosis and make an appropriate approach to the patient.

Effect of intermittent theta burst transcranial magnetic stimulation on non-fluent aphasia after stroke / 中国康复理论与实践

ObjectiveTo explore the therapeutic effect and mechanism of intermittent theta burst transcranial magnetic stimulation (iTBS) on non-fluent aphasia after stroke. MethodsFrom August, 2021 to August, 2022, 50 patients with non-fluent aphasia after stroke in the First People's Hospital of Lianyungang were randomly divided into sham stimulation group (n = 25) and iTBS group (n = 25). Both groups accepted speach training. iTBS group accepted iTBS, and the sham stimulation group received sham iTBS, for four weeks. The serum brain-derived neurotrophic factor (BDNF) was measured, and they were assessed by China Rehabilitation Research Center Standard Aphasia Examination (CRRCAE) and Boston Diagnostic Aphasia Examination before and after treatment. ResultsTwo cases in the sham stimulation group and three cases in iTBS group dropped down. The BDNF level, and listen and understand, repeat, name, read aloud, and total score of CRRCAE improved in two groups after treatment (|t| > 5.012, P < 0.001); and they were better in iTBS group than in the sham stimulation group (|t| > 3.968, P < 0.001). The total effective rate was more in iTBS group than in the sham stimulation group (χ2 = 8.835, P < 0.05). ConclusioniTBS can improve speech function in patients with non-fluent aphasia after stroke, which may associate with the promotion of BDNF.

Effect of Dynamic or Static Hand Action Observation on Chronic Non-fluent Aphasia after Stroke / 中国康复理论与实践

Objective:To compare the effects of observation to non-goal-oriented hand actions with video or pictures on speech recovery in stroke patients with chronic non-fluent aphasia. Methods:From January, 2016 to December, 2018, 30 patients with non-fluent aphasia after stroke were randomly divided into two groups. The control group (n = 15) repeated the verbs after watching picture, and the experimental group (n = 15) repeated the verbs after watching videos, for four weeks. They were assessed with Western Aphasia Battery (WAB) before treatment and every week after treatment. Results:Aphasia quotient (AQ) increased with time (F > 32.922, P < 0.001), but no significant difference was found between two groups at any time points (t < 0.699, P > 0.05). The interactive effect between time and group was significant (F = 6.398, P < 0.001), which suggested that AQ increased more in the experimental group than in the control group. Conclusion:For non-goal-oriented hand action observation, video observation maybe promote speech recovery in stroke patients with chronic non-fluent aphasia.

The effects of low-frequency transcranial magnetic stimulation combined with hyperbaric oxygen in the treat-ment of non-fluent aphasia / 中华物理医学与康复杂志

Objective To investigate the effect of repeated application of low-frequency transcranial magne-tic stimulation ( rTMS) when combined with hyperbaric oxygen ( HBO) in the treatment non-fluent aphasia after a stroke. Methods Forty-eight stroke survivors with non-fluent aphasia were randomly assigned to a control group, an HBO group or an observation group, each of 16. All received conventional rehabilitation therapy consisting of drug therapy, speech training and conventional physical exercises, while the HBO and observation groups were additionally given HBO and rTMS combined with HBO respectively. The 1 Hz rTMS was applied over the Broca's homologues of the unaffected hemisphere. Before and after 4 weeks of treatment, the Western Aphasia Battery ( WAB) was used to evaluate the subjects' language function and the Modified Barthel Index ( MBI) was used to assess their ability in the activities of daily living. Results There was no significant difference in the average WAB scores among the three groups before the treatment. After 4 weeks of treatment the average WAB and MBI scores of the HBO group and the observation group had improved significantly, and there was then a significant difference among the three groups. The observation group was performing significantly better than the HBO group and the control group in spontaneous speech, auditory comprehension, repetition, naming, AQ score and MBI score. Pairwise comparisons showed that the observation group's average WAB score, spontaneous speech, auditory comprehension, repetition, naming, AQ score and MBI score were the best, followed by those of the HBO group and then those of the control group. All of the differences were statistically significant. Conclusions Low-frequency rTMS combined with HBO can significantly improve the language function and the quality of life of patients with non-fluent aphasia. Such combined therapy is worthy of clinical promotion and application.

Low frequency, repetitive transcranial magnetic stimulation can alleviate non-fluent aphasia after stroke / 中华物理医学与康复杂志

Objective To investigate the effects of low frequency repetitive transcranial magnetic stimulation (rTMS) on non-fluent aphasia in patients after stroke.Methods Forty stroke patients displaying non-fluent aphasia were randomly assigned to an rTMS group and a control group using a random number table.Both groups were treated with conventional language therapy,while the rTMS group was additionally given 0.5 Hz rTMS over the Broca's homologues of the unaffected hemisphere 5 days a week for 3 weeks.The patients were stimulated at 90％ of the motor threshold (MT),with 16 second trains and intervals of 3 seconds 48 times (384 pulses) in a session.Before and after the 3 weeks of treatment,the Western Aphasia Battery (WAB) and the Communicative Abilities in Daily Living (CADL) test were conducted in both groups to evaluate their language function and communication ability.Results Only spontaneous speech improved significantly (P＜0.05) in the control group after 3 weeks of treatment.In the rTMS group,the spontaneous speech,auditory comprehension,repetition,naming and aphasia quotient (AQ) had all improved significantly (P＜0.05).Moreover,after the treatment,the average score of the auditory comprehension (153.90± 31.79),repetition (82.65± 15.14),naming(81.28±22.12) and AQ (63.66± 13.64) of the rTMS group were significantly higher than those of the control group (P＜0.05).Conclusion rTMS applied to the Broca's homologues of the unaffected hemisphere can significantly improve language function in those exhibiting non-fluent aphasia after stroke.

Positive impact of speech therapy in progressive non-fluent aphasia / Impacto positivo de la terapia del lenguaje en afasia progresiva no fluente / Impacto positivo da terapia da linguagem em afasia progressiva não fluente

The aim of this paper is to analyze the effects of intensive speech therapy intervention in a case of progressive non-fluent aphasia (PNFA). This is a dementia syndrome characterized by a progressive deficit in expressive language fluency and syntactic analysis, and by agrammatism and phonemic paraphasias. Although in the early stages there are no alterations in memory, comprehension, or visual processing, personality changes can slightly occur. To analyze the effects of speech therapy in this syndrome, a single case design with pre- and post-test was used. The participant was a male patient of 84 years with PNFA, who for twelve months received weekly speech therapy to stimulate the phonological, lexical and syntactic processing. He underwent neuropsychological assessment in three stages: six months before the onset of therapy, six months after therapy started and after completing 12 months of intervention. Assessment involved linguistic processing, general cognition, neuropsychiatric symptoms, quality of life (QOL) and activities of daily living (ADL). As a result of therapy, the patient showed a slight improvement in language prosody, fluency, and content of spontaneous speech, and a significant improvement in repetition, reading aloud, and oral-phonatory praxis. Other aspects of cognitive functioning (orientation, verbal naming, praxis, and memory) remained stable; ADLs and QOL improved. It is concluded that prolonged speech therapy can improve language processing and have a positive impact on other cognitive and socio-emotional processes in PNFA. This 12-month therapeutic stimulation not only slowed cognitive decline, but allowed to see maintenance of achievements and improvement of symptoms, which can be regarded as a success in PNFA treatment, considering the rapid progression of the disease.

El objetivo de este artículo es analizar los efectos de una intervención intensiva de terapia del lenguaje en un caso de afasia progresiva no fluente (APNF). Este es un síndrome demencial caracterizado por un déficit progresivo en la fluidez del lenguaje expresivo y el análisis sintáctico, y por agramatismo y parafasias fonémicas. Aunque en las primeras etapas no presenta alteraciones en la memoria, la comprensión o el procesamiento visual, sí pueden presentarse ligeros cambios en la personalidad. Para analizar los efectos de la terapia del lenguaje en este síndrome, se utilizó un diseño de caso único con pre y post prueba. El participante fue un paciente masculino de 84 años con APNF, quien durante doce meses recibió una terapia de lenguaje semanal para estimular el procesamiento fonológico, léxico y sintáctico. Se le realizó una evaluación neuropsicológica en tres etapas: seis meses antes del inicio de la terapia, después de seis meses de intervención, y al completar 12 meses de esta. Específicamente se evaluó el procesamiento lingüístico, la cognición general, los síntomas neuropsiquiátricos, la calidad de vida (CdV) y las actividades de la vida diaria (AVD). Como resultado de la terapia, el paciente mostró ligeras mejorías en la prosodia, la fluidez y el contenido del lenguaje espontáneo, y una mejoría significativa en la repetición, la lectura en voz alta y las praxias orofonatorias. Otros aspectos cognitivos (orientación, denominación verbal, praxias y memoria) se mantuvieron estables; las AVD y la CDV mejoraron. Se concluye que la terapia del lenguaje prolongada puede mejorar el procesamiento lingüístico y también tener un impacto positivo en otros procesos cognitivos y socio-emocionales en la APNF. La intervención no solo disminuyó la velocidad del deterioro cognitivo, sino que permitió ver el mantenimiento de los logros y la mejoría de los síntomas, lo cual es un éxito en el tratamiento de la APNF, debido a su rápida progresión.

O objetivo deste artigo é analisar os efeitos de uma intervenção intensiva de terapia da linguagem em um caso de afasia progressiva não fluente (APNF). Esta é uma síndrome demencial caracterizada por um déficit progressivo na fluência da linguagem expressiva e da análise sintática, e por agramatismo e parafasias fonêmicas. Ainda que nas primeiras etapas não presenta alterações na memória, na compreensão ou no processamento visual, podem aparecer pequenas mudanças na personalidade. Para analisar os efeitos da terapia da linguagem nesta síndrome, utilizou-se um desenho de caso único com testes antes e depois. O participante foi um paciente masculino de 84 anos com APNF, que durante doze meses recebeu uma terapia de linguagem semanal para estimular o processamento fonológico, léxico e sintático. Realizou-se uma avaliação neuropsicológica em três etapas: seis meses antes do início da terapia, depois de seis meses de intervenção, e ao completar 12 meses desta. Avaliou-se especificamente o processamento linguístico, a cognição geral, os sintomas neuropsiquiátricos, a qualidade de vida (QdV) e as atividades da vida diária (AVD). Como resultado da terapia, o paciente mostrou pequenas melhorias na prosódia, na fluência e no conteúdo da linguagem espontânea, e uma melhoria significativa na repetição, na leitura em voz alta e nas praxias orofonatórias. Outros aspectos cognitivos (orientação, denominação verbal, praxias e memória) mantiveram-se estáveis; as AVD e a QdV melhoraram. Conclui-se que a terapia da linguagem prolongada pode melhorar o processamento linguístico e também ter um impacto positivo em outros processos cognitivos e sócio emocionais na APNF. A intervenção diminuiu não somente a velocidade da deterioração cognitiva, senão que permitiu ver a manutenção dos êxitos e a melhoria dos sintomas, o que representa um sucesso no tratamento da APNF, devido a sua rápida progressão.

Resting-state fMRI fALFF analysis in patients with non-fluent aphasia after ischemic stroke / 实用医学杂志

Objective To understand the impairment and compensation mechanism of brain function in pa-tients with non-fluent aphasia after ischemic stroke. The fractional amplitude of low frequency fluctuation (fALFF) method was used to analyze the functional magnetic resonance imaging (fMRI) data in the resting state between the aphasia patients and the normal controls. Methods The scans of the resting state of fMRI were performed in 17 aphasia patients and 19 age-, education-, and sex-matched healthy control subjects. The scan sequence was single-shot echo planar image,DPARSF software was used to analyze fALFF data of the aphasia patients and the healthy controls. Results Compared to the control group, the value in right superior temporal gurus, inferior parietal lob-ule, frontal lobe cortex, and postcentral gurus were significantly increased in the aphasia group. The fALFF in bilat-eral cerebellum and right thalamus were also decreased in the aphasia group. Conclusions The fALFF values in some brain region in the aphasia group were abnormal in the resting state , indicating a few pathological change of brain function in patients with non-fluent aphasia after ischemic stroke.

The effects of transcranial magnetic stimulations on non-fluent aphasia following stroke / 中华物理医学与康复杂志

Objective To explore the effects and mechanism of repetitive transcranial magnetic stimulation (rTMS) applied to the right Broca's homologue of stroke patients with non-fluent aphasia.Methods One stroke patient with non-fluent aphasia received rTMS at 1 Hz and another received the same treatment at 10 Hz.The western aphasia battery (WAB) and functional magnetic resonance imaging (fMRI) were used to evaluate their language function before and after the intervention.Results After treatment,language function in both patients had improved significantly.The aphasia quotient (AQ) score of patient 1 had improved from 37.2 to 66.6,and the AQ score of patient 2 had improved from 36.2 to 60.8.Before treatment,patient 1's activated brain areas during a vocabulary reading task were the left anterior central gyrus and the left gyrus frontalis medius.After the 1 Hz rTMS treatment the activated brain areas were the left medial surface of the lobus frontalis,the left gyrus frontalis inferior,the left prefrontal area,the left preinsula,the left lobulus parietalis inferior,and the left middle/inferior temporal gyrus.Before the 10 Hz rTMS treatment,patient 2's activated brain areas with the same vocabulary reading task were the bilateral medial surface of the temporal lobe,and the bilateral anterior central gyrus.After treatment the bilateral medial surface gyrus,the frontalis medius and lobus frontalis,the right gyrus frontalis inferior,the left prefrontal area,the bilateral lobulus parietalis superior,and the right superior/middle temporal gyrus were activated.Conclusion rTMS can significantly improve language function in stroke patients with non-fluent aphasia.Patients with smaller lesions in the left hemisphere language area can achieve hemisphere function restructuring.Larger lesions in the left hemisphere language area will probably yield bilateral restructuring in both hemispheres.

Afasia logopénica: presentación de un caso y revisión de la literatura / Logopenic Aphasia: a case report an review of the literature

With the progressive aging of the Chilean population the diagnosis of neurodegenerative disorders is increasingly common, and among them is Primary Progressive Aphasia (PPA), with specific symptoms but late consultation. PPA is a clinical syndrome characterized by the degeneration of language regions in the dominant hemisphere that determines an insidious and progressive loss of language. Two types of PPA were recognized: Progressive non-fluent Aphasia (APnF) and Progressive Semantic Aphasia (DS), and was recently identified as a new type, Logopénica Progressive Aphasia (APL). We describe a case evaluated at the University of Chile Clinical Hospital of a woman of 54 years who have a history of 2-3 years of fluent speech with reduced speed due to the difficulty in finding words, had shortcomings in repetition of complex words, phrases and sentences, presence of phonemic paraphasias and impaired episodic memory. What in the phonological assessment supports a diagnosis of APL. Despite the above, the neurological examination was normal. The APL has been associated with Alzheimer's disease because it presents impaired episodic memory and the neuropathological changes most frequently encountered are amyloid plaques and neurofibrillary tangles. Through this article you may learn more about this disease and who to go if you or some colleague have symptoms to receive some guidance.

Con el progresivo envejecimiento de la población Chilena el diagnóstico de enfermedades neurodegenerativas es cada vez más com ún, y entre ellas está la afasia progresiva primaria (PPA), de síntomas específicos pero consulta tardía. PPA es un síndrome clínico caracterizado por la degeneración de las regiones del lenguaje en el hemisferio dominante que determina una pérdida insidiosa y progresiva del lenguaje. Fueron reconocidos dos tipos de PPA: Afasia Progresiva no fluente (APnF) y Afasia Progresiva Semántica (DS), y recientemente fue identificado un nuevo tipo como, Afasia Progresiva Logopénica (APL). Se describe un caso evaluado en el Hospital Clínico Universidad de Chile de una mujer de 54 años que presenta una historia de 2-3 años de habla fluida, con disminución de la velocidad debido a la dificultad para encontrar palabras, presentaba fallas en repetición de palabras complejas, frases y oraciones, presencia de parafasias fonémicas y problemas de memoria episódica. Lo que en la evaluación fonológica es compatible con un diagnóstico de APL. A pesar de todo lo anterior el examen neurológico fue normal. La APL se ha asociado con la enfermedad de Alzheimer, ya que presenta deterioro de la memoria episódica y los cambios neuropatológicos más frecuentes son las placas amiloides y los ovillos neurofibrilares. A través de este artículo podrán conocer más acerca de esta enfermedad y a quien acudir en caso de presentar síntomas usted o algún conocido para recibir alguna orientación.

Adaptação do instrumento de avaliação neuropsicológica breve Neupsilin para avaliar pacientes com Afasia Expressiva: Neupsilin-Af / Adaptation of the Brazilian Brief Neuropsychologica l Assessment Battery NEUPSILIN to evaluate patients with expressive aphasia: NEUPSILIN-Af

Este trabalho apresenta o processo de adaptação do Instrumento de Avaliação Neuropsicológica Breve NEUPSILIN para afásicos expressivos (NEUPSILIN-Af). Esta bateria abrange a avaliação breve de componentes das funções Orientação Têmporo-Espacial, Atenção, Percepção, Memória (de Trabalho, Episódico-Semântica, Semântica de Longo Prazo, Visual e Prospectiva), Habilidades Aritméticas, Linguagem (Oral e Escrita), Praxias (Ideomotora, Construtiva e Reflexiva) e Funções Executivas (Resolução de Problemas Simples e Fluência Verbal Fonêmica-Ortográfica). Devido aos déficits primariamente linguísticos de pacientes afásicos expressivos, que podem influenciar na verbalização das respostas, a adaptação foi necessária. O processo envolveu seis etapas: 1) revisão da literatura, 2) análise do instrumento original e construção da versão preliminar adaptada do NEUPSILIN–Af, 3) análise de juízes especialistas na área da saúde, 4) análise de juízes especialistas em neuropsicologia, 5) estudo piloto, e 6) versão final do instrumento. Esses procedimentos permitiram testar a validade de conteúdo do NEUPSILIN-Af. Estudos futuros serão direcionados para testar a sensibilidade dessa bateria para detectar déficits cognitivos em pacientes com afasia de expressão.

Clinicopathologic analysis of progressive non-fluent aphasia and corticobasal degeneration:case report and review / Estudo clínicopatologico de afasia progressiva não-fluente e degeneração córtico-basal: relato de caso e revisão

Objective: To investigate progressive non-fluent aphasia and histopathologically-proven corticobasal degeneration. Methods: We evaluated symptoms, signs, neuropsychological deficits, and radiology data longitudinally, in a patient with autopsy-proven corticobasal degeneration and correlated these observations directly to the neuroanatomic distribution of the disease. Results: At presentation, a specific pattern of cognitive impairment was evident with an extreme extrapyramidal motor abnormality. Follow-up examination revealed persistent impairment of praxis and executive functioning, progressive worsening of language performance, and moderately preserved memory. The motor disorder manifested and worsened as the condition progressed. Many of the residual nerve cells were ballooned and achromatic with eccentric nuclei. Tau-immunoreactive pathology was significantly more prominent in neurons in the frontal and parietal cortices and dentate nuclei than in temporal neocortex, hippocampi and brainstem. Conclusion: The clinical diagnosis of progressive non-fluent aphasia secondary to corticobasal degeneration hinged on a specific pattern of impaired cognition as well as an extrapyramidal motor disorder, reflecting the neuroanatomic distribution of the disease in frontal and anterior temporal cortices and the dentate nuclei.

Objetivo: Investigar o quadro clínico de afasia progressiva não-fluente e confirmação histopatológica de degeneração córtico-basal. Métodos: Foram avaliadas as alterações clínicas, neuropsicológicas e de neuroimagem, durante todo o curso clínico da doença. O diagnóstico de degeneração córtico-basal foi confirmado por estudo histopatológico. Essas observações foram diretamente relacionadas com a distribuição anatômica da doença. Resultados: Foi observada uma forma específica de prejuízo cognitivo associada com importante alteração extrapiramidal. Durante o curso clínico, surgiram apraxia e disfunção executiva, piora progressiva da linguagem e memória moderadamente preservada. As alterações extrapiramidais pioraram progressivamente à rigidez universal e postura distônica. As reações de imunohistoquímica para a proteína tau foram significativamente mais proeminentes nos neurônios residuais de aspecto baloniformes e acromáticos do córtex frontal e parietal e núcleo denteado do cerebelo do que nos do neocórtex temporal, hipocampo e tronco cerebral. Conclusão: O diagnóstico clínico de afasia progressiva não-fluente quando evolui para degeneração córtico-basal deve apresentar uma forma específica de prejuízo cognitivo, incluindo as alterações motoras, refletindo uma distribuição neuroanatômica da doença no córtex frontal e temporal anterior e no núcleo denteado do cerebelo.