Assuntos
Córtex Cerebral/patologia , Transtornos de Deglutição/etiologia , Disartria/etiologia , Paralisia Facial/etiologia , Infarto da Artéria Cerebral Média/complicações , Neoplasias Supratentoriais/complicações , Idoso , Encefalopatias/diagnóstico , Transtornos de Deglutição/diagnóstico por imagem , Diagnóstico Diferencial , Dominância Cerebral , Disartria/diagnóstico por imagem , Paralisia Facial/diagnóstico por imagem , Evolução Fatal , Humanos , Infarto da Artéria Cerebral Média/diagnóstico por imagem , Masculino , Paralisia Pseudobulbar/diagnóstico , Neoplasias Supratentoriais/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Recusa do Paciente ao TratamentoRESUMO
We retrospectively analyzed the clinical features of two cases of neurodegenerative disease, whose initial symptoms were motor speech disorder and dementia, brought to autopsy. We compared the distributions of pathological findings with the clinical features. The main symptom of speech disorder was dysarthria, involving low pitch, slow rate, hypernasality and hoarseness. Other than these findings, effortful speech, sound prolongation and initial difficulty were observed. Moreover, repetition of multisyllables was severely impaired compared to monosyllables. Repetition and comprehension of words and sentences were not impaired. Neither atrophy nor fasciculation of the tongue was observed. Both cases showed rapid progression to mutism within a few years. Neuropathologically, frontal lobe degeneration including the precentral gyrus was observed. The bilateral pyramidal tracts also showed severe degeneration. However, the nucleus of the hypoglossal nerve showed only mild degeneration. These findings suggest upper motor neuron dominant motor neuron disease with dementia. We believe the results indicate a subgroup of motor neuron disease with dementia whose initial symptoms involve pseudobulbar palsy and dementia, and which shows rapid progression to mutism.
Assuntos
Encéfalo/patologia , Demência/complicações , Doença dos Neurônios Motores/complicações , Paralisia Pseudobulbar/etiologia , Idoso , Autopsia , Encéfalo/diagnóstico por imagem , Encéfalo/metabolismo , Proteínas de Ligação a DNA/metabolismo , Demência/diagnóstico , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Doença dos Neurônios Motores/diagnóstico , Paralisia Pseudobulbar/diagnóstico , Proteína FUS de Ligação a RNA/metabolismo , Estudos Retrospectivos , Coloração e Rotulagem , Tomografia Computadorizada de Emissão de Fóton ÚnicoRESUMO
Symptomatic treatment of amyotrophic lateral sclerosis (ALS) is relevant in preventing complications and improving quality of life as long as curative therapies are still out of sight. About one third of ALS patients show disabling problems associated with dysarthria, dysphagia, sialorrhea, and a pseudobulbar affective disorder already in the early stages of ALS. A multidisciplinary approach is the cornerstone of symptomatic treatment of bulbar and pseudobulbar ALS features. Except for riluzole randomized controlled trials are lacking. Here, we review the current views with regard to epidemiology, pathophysiology, diagnosis, and practical aspects of treating bulbar and pseudobulbar symptoms.
Assuntos
Esclerose Lateral Amiotrófica/terapia , Transtornos de Deglutição/terapia , Disartria/terapia , Cuidados Paliativos/métodos , Equipe de Assistência ao Paciente , Paralisia Pseudobulbar/terapia , Sialorreia/terapia , Sintomas Afetivos/diagnóstico , Sintomas Afetivos/fisiopatologia , Sintomas Afetivos/terapia , Esclerose Lateral Amiotrófica/diagnóstico , Esclerose Lateral Amiotrófica/fisiopatologia , Esclerose Lateral Amiotrófica/psicologia , Antidepressivos/uso terapêutico , Terapia Combinada , Auxiliares de Comunicação para Pessoas com Deficiência , Transtornos de Deglutição/diagnóstico , Transtornos de Deglutição/fisiopatologia , Disartria/diagnóstico , Disartria/fisiopatologia , Antagonistas de Aminoácidos Excitatórios/uso terapêutico , Humanos , Paralisia Pseudobulbar/diagnóstico , Paralisia Pseudobulbar/fisiopatologia , Qualidade de Vida/psicologia , Ensaios Clínicos Controlados Aleatórios como Assunto , Riluzol/uso terapêutico , Sialorreia/diagnóstico , Sialorreia/fisiopatologiaRESUMO
We report the case of a 14-year-old boy with bilateral congenital perisylvian syndrome as evidenced by polymicrogyria in the left and right frontotemporal regions, pseudobulbar paresis, seizures, and spastic diplegia. This is the first description of comprehensive neuropsychological assessment in a child with this syndrome. The results reflect significant impairments in auditory processing and independent learning and retention, but relatively preserved cognitive performance on interactive tasks involving frequent feedback and redirection. The findings are interpreted in the context of the neuropathology of this syndrome, with an emphasis on pragmatic implications for rehabilitation.
Assuntos
Anormalidades Múltiplas/diagnóstico , Córtex Cerebral/patologia , Cognição , Malformações do Desenvolvimento Cortical/patologia , Malformações do Sistema Nervoso/diagnóstico , Anormalidades Múltiplas/patologia , Anormalidades Múltiplas/fisiopatologia , Adolescente , Córtex Cerebral/anormalidades , Córtex Cerebral/fisiopatologia , Lateralidade Funcional , Humanos , Imageamento por Ressonância Magnética , Masculino , Malformações do Desenvolvimento Cortical/diagnóstico , Malformações do Sistema Nervoso/patologia , Malformações do Sistema Nervoso/fisiopatologia , Testes Neuropsicológicos , Paralisia Pseudobulbar/congênito , Paralisia Pseudobulbar/diagnóstico , SíndromeRESUMO
This monograph summarizes the proceedings of a roundtable meeting convened to discuss pseudobulbar affect (PBA). Two didactic lectures were presented followed by a moderated discussion among 11 participants. Post-meeting manuscript development synthesized didactic- and discussion-based content ad incorporated additional material from the neuroscience literature. A conceptual framework with which to distinguish between disorders of mood and affect is presented first, and disorders of affect regulation are then reviewed briefly. A detailed description of the most common of these disorders, PBA, is the focus of the remainder of the monograph. The prevalence, putative neuranatomic and neurochemical bases of PBA are reviewed, and current and emerging methods of evaluation and treatment of persons with PBA are discussed. The material presented in this monograph will help clinicians better recognize, diagnose, and treat PBA, and will form a foundation for understanding and interpreting future studies of this condition.
Assuntos
Transtornos do Humor/diagnóstico , Paralisia Pseudobulbar/diagnóstico , Diagnóstico Diferencial , Humanos , Programas de Rastreamento , Transtornos do Humor/epidemiologia , Paralisia Pseudobulbar/epidemiologia , Paralisia Pseudobulbar/psicologiaRESUMO
A case of left atrial (LA) myxoma presenting as pseudobulbar palsy, due to multiple cerebral infarcts, without any cardiac manifestations, is presented. LA myxoma is rare cause of embolization to CNS causing ischemic infarcts. Due to multiple CNS infarcts patient can present with varied clinical picture and pseudobulbar palsy is not a very common presentation. It was a real diagnostic dilemma before LA myxoma was diagnosed on echocardiography.