Assuntos
Epilepsia Resistente a Medicamentos , Deficiência Intelectual , Malformações do Desenvolvimento Cortical , Polimicrogiria , Anormalidades Múltiplas , Córtex Cerebral , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia Resistente a Medicamentos/tratamento farmacológico , Epilepsia Resistente a Medicamentos/etiologia , Disartria/diagnóstico por imagem , Disartria/etiologia , Humanos , Imageamento por Ressonância Magnética , Malformações do Desenvolvimento Cortical/complicações , Malformações do Desenvolvimento Cortical/diagnóstico por imagem , Polimicrogiria/complicações , Polimicrogiria/diagnóstico por imagemRESUMO
RATIONALE: Anti-IgLON5 disease was first described as a progressive antibody-associated encephalopathy, with multiple non-specific clinical symptoms including sleep dysfunction, bulbar symptoms, progressive supranuclear palsy-like syndrome, cognitive impairment, and a variety of movement disorders. This newly discovered disease presents with unremarkable or unspecific brain magnetic resonance imagings (MRI), and have poor responsiveness to immunotherapy. PATIENT CONCERNS: In this case, a 37-year-old man presented with 4-day history of gait instability, dysarthria, and oculomotor abnormalities. The initial neurologic examination revealed mild unsteady gait, subtle dysarthria, and left abducent paralysis. DIAGNOSIS: The patient was diagnosed with anti-IgLON5 disease, based on clinical features and positive anti-IgLON5 antibodies in serum. INTERVENTIONS: Initially, the patient was treated with high dosages of methylprednisolone and immunoglobulins.Outcomes: The symptoms of patient rapidly improved after high-dose intravenous methylprednisolone and immunoglobulins. CONCLUSIONS: In this paper, we report a new case of anti-IgLON5 disease with major symptoms of gait instability, dysarthria, and oculomotor abnormalities, with distinctive brain MRI findings, and responsive to immunotherapy.
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Autoanticorpos/sangue , Doenças Autoimunes do Sistema Nervoso/diagnóstico por imagem , Moléculas de Adesão Celular Neuronais/imunologia , Encefalite/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Adulto , Autoanticorpos/imunologia , Doenças Autoimunes do Sistema Nervoso/tratamento farmacológico , Doenças Autoimunes do Sistema Nervoso/imunologia , Encéfalo/diagnóstico por imagem , Disartria/diagnóstico por imagem , Disartria/tratamento farmacológico , Disartria/imunologia , Encefalite/tratamento farmacológico , Encefalite/imunologia , Transtornos Neurológicos da Marcha/diagnóstico por imagem , Transtornos Neurológicos da Marcha/tratamento farmacológico , Transtornos Neurológicos da Marcha/imunologia , Humanos , Masculino , Neuroimagem/métodos , Doenças do Nervo Oculomotor/diagnóstico por imagem , Doenças do Nervo Oculomotor/tratamento farmacológico , Doenças do Nervo Oculomotor/imunologiaAssuntos
Transtornos de Deglutição/diagnóstico por imagem , Transtornos de Deglutição/etiologia , Disartria/diagnóstico por imagem , Disartria/etiologia , Paralisia Facial/diagnóstico por imagem , Paralisia Facial/etiologia , AVC Isquêmico/diagnóstico por imagem , AVC Isquêmico/etiologia , Transtornos Puerperais/diagnóstico por imagem , Adulto , Feminino , Humanos , Imageamento por Ressonância MagnéticaAssuntos
Transtornos de Deglutição/diagnóstico por imagem , Disartria/diagnóstico por imagem , Miastenia Gravis/diagnóstico por imagem , Idoso , Transtornos de Deglutição/sangue , Transtornos de Deglutição/etiologia , Diagnóstico Diferencial , Disartria/sangue , Disartria/etiologia , Humanos , Masculino , Miastenia Gravis/sangue , Miastenia Gravis/complicaçõesRESUMO
OBJECTIVE: This study was undertaken to gain insights into structural networks associated with stimulation-induced dysarthria (SID) and to predict stimulation-induced worsening of intelligibility in essential tremor patients with bilateral thalamic deep brain stimulation (DBS). METHODS: Monopolar reviews were conducted in 14 essential tremor patients. Testing included determination of SID thresholds, intelligibility ratings, and a fast syllable repetition task. Volumes of tissue activated (VTAs) were calculated to identify discriminative fibers for stimulation-induced worsening of intelligibility in a structural connectome. The resulting fiber-based atlas structure was then validated in a leave-one-out design. RESULTS: Fibers determined as discriminative for stimulation-induced worsening of intelligibility were mainly connected to the ipsilateral precentral gyrus as well as to both cerebellar hemispheres and the ipsilateral brain stem. In the thalamic area, they ran laterally to the thalamus and posteromedially to the subthalamic nucleus, in close proximity, mainly anterolaterally, to fibers beneficial for tremor control as published by Al-Fatly et al in 2019. The overlap of the respective clinical stimulation setting's VTAs with these fibers explained 62.4% (p < 0.001) of the variance of stimulation-induced change in intelligibility in a leave-one-out analysis. INTERPRETATION: This study demonstrates that SID in essential tremor patients is associated with both motor cortex and cerebellar connectivity. Furthermore, the identified fiber-based atlas structure might contribute to future postoperative programming strategies to achieve optimal tremor control without speech impairment in essential tremor patients with thalamic DBS. ANN NEUROL 2021;89:315-326.
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Cerebelo/fisiopatologia , Estimulação Encefálica Profunda/efeitos adversos , Disartria/etiologia , Tremor Essencial/terapia , Córtex Motor/fisiopatologia , Inteligibilidade da Fala , Idoso , Ataxia/fisiopatologia , Conectoma , Disartria/diagnóstico por imagem , Disartria/fisiopatologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Contração Muscular/fisiologia , Vias Neurais/fisiopatologia , Núcleos Ventrais do TálamoAssuntos
Disartria/diagnóstico por imagem , Paresia/diagnóstico por imagem , Acidente Vascular Cerebral/diagnóstico por imagem , Adulto , Disartria/etiologia , Fibrinolíticos/uso terapêutico , Humanos , Imageamento por Ressonância Magnética , Masculino , Paresia/etiologia , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/tratamento farmacológico , Ativador de Plasminogênio Tecidual/uso terapêuticoRESUMO
RATIONALE: Basilar artery (BA) fenestration is a congenital anomaly with duplicated BA, which can cause ischemic stroke. However, the stroke mechanism is not clearly verified in patients with BA fenestration. PATIENT CONCERNS: Here, we report a case of 64-year-old man with well-controlled hypertension admitted with dysarthria, only. DIAGNOSES: Diffusion weighted image showed a bilateral symmetric pontine infarction sparing the midline. BA fenestration was observed from magnetic resonance angiography. INTERVENTION: High-resolution magnetic resonance image (MRI) and 4D flow MRI was performed to verify the mechanism of stroke associated with BA fenestration. OUTCOMES: No plaque was observed at the area of BA fenestration from high-resolution MRI. 4D flow MRI showed bifurcated flow with high flow velocity and low shear stress at the area of BA fenestration. LESSONS: A turbulent flow with high flow velocity and low shear stress at the BA fenestration area may have influenced the flow through the bilateral perforating arteries resulting in a bilateral symmetric pontine infarction with sparing the midline where the septa of BA is located. 4D flow dynamic studies may be beneficial for verifying the mechanism of stroke.
Assuntos
Artéria Basilar/anormalidades , Infartos do Tronco Encefálico/congênito , Disartria/diagnóstico por imagem , Acidente Vascular Cerebral/diagnóstico por imagem , Artéria Basilar/diagnóstico por imagem , Infartos do Tronco Encefálico/diagnóstico por imagem , Disartria/etiologia , Humanos , Angiografia por Ressonância Magnética , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Ponte , Acidente Vascular Cerebral/congênitoRESUMO
Paroxysmal dysarthria and ataxia (PDA) syndrome constitutes a rare neurological disorder, and is generally reported in cases of multiple sclerosis (MS) involving the midbrain. We present an illustrative case of 32-year-old female who developed clinically isolated syndrome manifested paroxysmal dysarthria, ataxia, ptosis and diplopia, coexisting with anti-N-methyl-d-aspartate receptor antibodies. We review the literature and identify 23 other cases with brain MRI examinations to summarize the lesion locations and clinical characteristics of PDA syndrome, and ultimately provide a new framework for understanding this rare condition. The current case expands the spectrum of symptoms in PDA syndrome, which was including but not limited to dysarthria and ataxia. Caudal paramedian midbrain lesions involving decussation of the superior cerebellar peduncles appear to be critical for PDA syndrome.
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Ataxia/diagnóstico por imagem , Autoanticorpos , Doenças Desmielinizantes/diagnóstico por imagem , Disartria/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Receptores de N-Metil-D-Aspartato , Adulto , Ataxia/sangue , Ataxia/complicações , Autoanticorpos/sangue , Doenças Desmielinizantes/sangue , Doenças Desmielinizantes/complicações , Disartria/sangue , Disartria/complicações , Feminino , Humanos , Receptores de N-Metil-D-Aspartato/sangue , SíndromeRESUMO
BACKGROUND: Up to 90% of patients with Parkinson's disease (PD) eventually develop the speech and voice disorder referred to as hypokinetic dysarthria (HD). However, the brain morphological changes associated with HD have not been investigated. Moreover, no reliable model for predicting the severity of HD based on neuroimaging has yet been developed. METHODS: A total of 134 PD patients were included in this study and divided into a training set and a test set. All participants underwent a structural magnetic resonance imaging (MRI) scan and neuropsychological evaluation. Individual cortical thickness, subcortical structure, and white matter volume were extracted, and their association with HD severity was analyzed. After feature selection, a machine-learning model was established using a support vector machine in the training set. The severity of HD was then predicted in the test set. RESULTS: Atrophy of the right precentral cortex and the right fusiform gyrus was significantly associated with HD. No association was found between HD and volume of white matter or subcortical structures. Favorable and optimal performance of machine learning on HD severity prediction was achieved using feature selection, giving a correlation coefficient (r) of .7516 and a coefficient of determination (R2 ) of .5649 (P < .001). CONCLUSION: The brain morphological changes were associated with HD. Excellent prediction of the severity of HD was achieved using machine learning based on neuroimaging.
Assuntos
Encéfalo/diagnóstico por imagem , Disartria/diagnóstico por imagem , Hipocinesia/diagnóstico por imagem , Aprendizado de Máquina , Doença de Parkinson/diagnóstico por imagem , Índice de Gravidade de Doença , Idoso , Disartria/epidemiologia , Feminino , Humanos , Hipocinesia/epidemiologia , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/epidemiologia , Valor Preditivo dos Testes , Estudos RetrospectivosRESUMO
INTRODUCTION: Non-linguistic properties of speech are widely heterogeneous and require complex neurological integration. The association between white matter integrity and the severity of dysarthria was investigated in a group of patients diagnosed with amyotrophic lateral sclerosis (ALS). METHODS: Thirty-six patients diagnosed with amyotrophic lateral sclerosis completed a magnetic resonance imaging protocol inclusive of diffusion-weighted images. A clinical assessment of pneumo-phono-articulatory abilities was conducted for each patient, and a composite score of residual speech capacity was calculated. Tract-Based Spatial Statistics was carried out to model the potential association between residual speech capacity and microstructural properties of white matter (fractional anisotropy, mean and radial diffusivity). RESULTS: A significant negative association was found between residual speech capacity and mean diffusivity in a large white matter cluster located in frontal, parietal and right temporal regions. These subcortical areas were characterised by pathological microstructural disruption, as revealed by post hoc analyses. CONCLUSIONS: Non-linguistic aspects of speech are associated with microstructural integrity of frontal, parietal and right temporal white matter in amyotrophic lateral sclerosis. Such mapping is consistent with the centres responsible of volitional control of speech and sensory feedback during non-linguistic speech production.
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Esclerose Lateral Amiotrófica/diagnóstico por imagem , Disartria/diagnóstico por imagem , Lobo Frontal/diagnóstico por imagem , Lobo Parietal/diagnóstico por imagem , Substância Branca/diagnóstico por imagem , Adulto , Idoso , Esclerose Lateral Amiotrófica/complicações , Esclerose Lateral Amiotrófica/fisiopatologia , Disartria/etiologia , Disartria/fisiopatologia , Feminino , Seguimentos , Lobo Frontal/fisiopatologia , Humanos , Masculino , Pessoa de Meia-Idade , Lobo Parietal/fisiopatologia , Substância Branca/fisiopatologiaAssuntos
Calcinose/genética , Glicosídeo Hidrolases/genética , Transtornos Heredodegenerativos do Sistema Nervoso/genética , Irmãos , Idoso , Doenças dos Gânglios da Base/diagnóstico por imagem , Doenças dos Gânglios da Base/genética , Doenças dos Gânglios da Base/fisiopatologia , Tronco Encefálico/diagnóstico por imagem , Tronco Encefálico/fisiopatologia , Calcinose/diagnóstico por imagem , Calcinose/fisiopatologia , Doenças Cerebelares/diagnóstico por imagem , Doenças Cerebelares/genética , Doenças Cerebelares/fisiopatologia , Disartria/diagnóstico por imagem , Disartria/genética , Disartria/fisiopatologia , Feminino , Transtornos Neurológicos da Marcha/diagnóstico por imagem , Transtornos Neurológicos da Marcha/genética , Transtornos Neurológicos da Marcha/fisiopatologia , Transtornos Heredodegenerativos do Sistema Nervoso/diagnóstico por imagem , Transtornos Heredodegenerativos do Sistema Nervoso/fisiopatologia , Humanos , Itália , Masculino , Mutação , Transtornos Parkinsonianos/diagnóstico por imagem , Transtornos Parkinsonianos/genética , Transtornos Parkinsonianos/fisiopatologia , LinhagemAssuntos
Anticorpos/efeitos adversos , Disartria/imunologia , Encefalite/imunologia , Glicoproteína Mielina-Oligodendrócito/imunologia , Anticorpos/sangue , Disartria/complicações , Disartria/diagnóstico por imagem , Disartria/patologia , Encefalite/complicações , Encefalite/diagnóstico por imagem , Encefalite/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Neuroimagem , Tegmento Mesencefálico/patologiaRESUMO
Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by JC virus reactivation. Its occurrence is very rare after solid organ transplantation, especially liver transplantation. We report a patient who received liver transplantation due to liver failure resulting from autoimmune hepatitis and advanced PML presenting with aphasia. A 41-year-old female with a history of liver transplantation who received a usual immunosuppression regimen was admitted with a stroke attack resulting in right hemiplegia 2 months after liver transplantation. Surprisingly, she gradually developed dysarthria and left central facial paresis. A brain MRI showed an abnormal multifocal area with a high T2/flair signal in the deep subcortical white matter of the left hemisphere as well as the splenium of the corpus callosum. PCR evaluation of CSF for JCV was positive while other PCR results were negative. A liver transplant recipient receiving immunosuppressive treatment for a long time could develop PML due to JCV reactivation. Only eight cases of JCV infection were reported after liver transplantation by the time of reporting this case. Unfortunately, there is no definite treatment for PML.
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Hepatite Autoimune/imunologia , Vírus JC/genética , Leucoencefalopatia Multifocal Progressiva/imunologia , Transplante de Fígado , Adulto , Afasia/diagnóstico por imagem , Afasia/fisiopatologia , Afasia/virologia , Córtex Cerebral/diagnóstico por imagem , Córtex Cerebral/efeitos dos fármacos , Córtex Cerebral/patologia , Córtex Cerebral/virologia , Corpo Caloso/diagnóstico por imagem , Corpo Caloso/efeitos dos fármacos , Corpo Caloso/patologia , Corpo Caloso/virologia , Disartria/diagnóstico por imagem , Disartria/fisiopatologia , Disartria/virologia , Feminino , Hemiplegia/diagnóstico por imagem , Hemiplegia/fisiopatologia , Hemiplegia/virologia , Hepatite Autoimune/patologia , Hepatite Autoimune/cirurgia , Hepatite Autoimune/virologia , Humanos , Imunossupressores/administração & dosagem , Vírus JC/imunologia , Vírus JC/isolamento & purificação , Leucoencefalopatia Multifocal Progressiva/diagnóstico por imagem , Leucoencefalopatia Multifocal Progressiva/patologia , Leucoencefalopatia Multifocal Progressiva/cirurgia , Fígado/efeitos dos fármacos , Fígado/imunologia , Fígado/patologia , Fígado/cirurgia , Imageamento por Ressonância Magnética , Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/fisiopatologia , Acidente Vascular Cerebral/virologia , Ativação Viral/imunologiaRESUMO
Although dysarthria is a common pattern in multiple sclerosis (MS), the contribution of specific brain areas to key factors of dysarthria remains unknown. Speech data were acquired from 123 MS patients with Expanded Disability Status Scale (EDSS) ranging from 1 to 6.5 and 60 matched healthy controls. Results of computerized acoustic analyses of subtests on spastic and ataxic aspects of dysarthria were correlated with MRI-based brain volume measurements. Slow articulation rate during reading was associated with bilateral white and grey matter loss whereas reduced maximum speed during oral diadochokinesis was related to greater cerebellar involvement. Articulation rate showed similar correlation to whole brain atrophy (râ¯=â¯0.46, pâ¯<â¯0.001) as the standard clinical scales such as EDSS (râ¯=â¯-0.45, pâ¯<â¯0.001). Our results support the critical role of the pyramidal tract and cerebellum in the modification of motor speech timing in MS.
Assuntos
Encéfalo/diagnóstico por imagem , Disartria/diagnóstico por imagem , Esclerose Múltipla/diagnóstico por imagem , Adulto , Atrofia/diagnóstico por imagem , Encéfalo/patologia , Disartria/etiologia , Feminino , Substância Cinzenta/diagnóstico por imagem , Substância Cinzenta/patologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/complicações , LeituraAssuntos
Estimulação Encefálica Profunda/métodos , Disartria/diagnóstico por imagem , Disartria/terapia , Globo Pálido/diagnóstico por imagem , Neuroacantocitose/diagnóstico por imagem , Neuroacantocitose/terapia , Adulto , Disartria/complicações , Feminino , Humanos , Neuroacantocitose/complicações , Resultado do TratamentoRESUMO
INTRODUCTION: Hypokinetic dysarthria (HD) is a common symptom of Parkinson's disease (PD) which does not respond well to PD treatments. We investigated acute effects of repetitive transcranial magnetic stimulation (rTMS) of the motor and auditory feedback area on HD in PD using acoustic analysis of speech. METHODS: We used 10â¯Hz and 1â¯Hz stimulation protocols and applied rTMS over the left orofacial primary motor area, the right superior temporal gyrus (STG), and over the vertex (a control stimulation site) in 16 PD patients with HD. A cross-over design was used. Stimulation sites and protocols were randomised across subjects and sessions. Acoustic analysis of a sentence reading task performed inside the MR scanner was used to evaluate rTMS-induced effects on motor speech. Acute fMRI changes due to rTMS were also analysed. RESULTS: The 1â¯Hz STG stimulation produced significant increases of the relative standard deviation of the 2nd formant (pâ¯=â¯0.019), i.e. an acoustic parameter describing the tongue and jaw movements. The effects were superior to the control site stimulation and were accompanied by increased resting state functional connectivity between the stimulated region and the right parahippocampal gyrus. The rTMS-induced acoustic changes were correlated with the reading task-related BOLD signal increases of the stimulated area (Râ¯=â¯0.654, pâ¯=â¯0.029). CONCLUSION: Our results demonstrate for the first time that low-frequency stimulation of the temporal auditory feedback area may improve articulation in PD and enhance functional connectivity between the STG and the cortical region involved in an overt speech control.
Assuntos
Conectoma , Disartria/fisiopatologia , Retroalimentação Sensorial/fisiologia , Córtex Motor/fisiopatologia , Rede Nervosa/fisiopatologia , Giro Para-Hipocampal/fisiopatologia , Doença de Parkinson/fisiopatologia , Lobo Temporal/fisiopatologia , Estimulação Magnética Transcraniana , Idoso , Disartria/diagnóstico por imagem , Disartria/etiologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Córtex Motor/diagnóstico por imagem , Rede Nervosa/diagnóstico por imagem , Giro Para-Hipocampal/diagnóstico por imagem , Doença de Parkinson/complicações , Doença de Parkinson/diagnóstico por imagem , Acústica da Fala , Lobo Temporal/diagnóstico por imagemRESUMO
We describe a gentleman diagnosed with a posterior fossa medulloblastoma in 1998, successfully treated with craniospinal radiation therapy (RT) and posterior fossa RT boost, followed by 12 months of adjuvant chemotherapy. Nineteen years later, at the age of 28, the patient presented with multiple cranial neuropathies and was found to have disseminated high-grade glioma with leptomeningeal dissemination. In addition to the salient features of this case, we provide a brief review of RT-induced malignancies and the need for further research regarding surveillance and prevention strategies.