Analysis of events from sudden isolated dysarthria to diagnosis of myasthenic crisis: myasthenia gravis mimicking acute lacunar stroke-a case report.

BACKGROUND: Myasthenic crisis (MC) is a life-threatening complication of myasthenia gravis (MG), necessitating ventilation. Achieving a safe and timely diagnosis of myasthenic crisis with atypical, isolated presentation is a considerable challenge particularly in elderly patients, where myasthenia gravis can present with isolated dysarthria in rare instances, giving a clinical impression of lacunar stroke. CASE PRESENTATION: We present a compelling case of a 73-year-old Caucasian female presenting with abrupt onset of isolated dysarthria. Despite initial treatment for a presumed lacunar stroke, subsequent evaluations led to her diagnosis of a myasthenic crisis. Within 72 h of admission, the patient developed dysphagia and shortness of breath, requiring supplemental oxygen. The case highlights the sequential progression of events from the atypical presentation of isolated dysarthria and its course to the management of a myasthenic crisis. CONCLUSION: Our reported case focuses on the discussion of myasthenia that mimicked a lacunar stroke and was finally diagnosed at a critical time of medical crisis. This case highlights the imperative notion that isolated dysarthria in elderly individuals warrants vigilant monitoring for possible myasthenia gravis, given the low incidence of lacunar stroke presenting with only dysarthria.

Autoimmune nodopathy with anti-contactin 1 antibody characterized by cerebellar dysarthria: a case report and literature review.

Background: Autoimmune nodopathy (AN) has emerged as a novel diagnostic category that is pathologically different from classic chronic inflammatory demyelinating polyneuropathy. Clinical manifestations of AN include sensory or motor neuropathies, sensory ataxia, tremor, and cranial nerve involvement. AN with a serum-positive contactin-1 (CNTN1) antibody usually results in peripheral nerve demyelination. In this study, we reported a rare case of AN with CNTN1 antibodies characterized by the presence of CNTN1 antibodies in both serum and cerebrospinal fluid, which is associated with cerebellar dysarthria. Methods: A 25-year-old man was admitted to our hospital due to progressive dysarthria with limb tremors. The patient was initially diagnosed with peripheral neuropathy at a local hospital. Three years after onset, he was admitted to our hospital due to dysarthria, apparent limb tremor, and limb weakness. At that time, he was diagnosed with spinocerebellar ataxia. Eight years post-onset, during his second admission, his condition had notably deteriorated. His dysarthria had evolved to typical distinctive cerebellar characteristics, such as tremor, loud voice, stress, and interrupted articulation. Additionally, he experienced further progression in limb weakness and developed muscle atrophy in the distal limbs. Magnetic resonance imaging (MRI), nerve conduction studies (NCS), and autoimmune antibody tests were performed. Results: The results of the NCS suggested severe demyelination and even axonal damage to the peripheral nerves. MRI scans revealed diffuse thickening of bilateral cervical nerve roots, lumbosacral nerve roots, cauda equina nerve, and multiple intercostal nerve root sheath cysts. Furthermore, anti-CNTN1 antibody titers were 1:10 in the cerebrospinal fluid (CSF) and 1:100 in the serum. After one round of rituximab treatment, the patient showed significant improvement in limb weakness and dysarthria, and the CSF antibodies turned negative. Conclusion: Apart from peripheral neuropathies, cerebellar dysarthria (central nervous system involvement) should not be ignored in AN patients with CNTN1 antibodies.

Topographic Consideration on the Occurrence of Ipsilesional Facial Paresis in Lateral Medullary Infarction.

INTRODUCTION: The purpose of this study was to identify course of the corticobulbar tract and factors associated with the occurrence of facial paresis (FP) in lateral medullary infarction (LMI). METHODS: Patients diagnosed with LMI who were admitted to tertiary hospital were retrospectively investigated and divided into two groups based on the presence of FP. FP was defined as grade 2 or more by the House-Brackmann scale. Differences between the two groups were analyzed with respect to anatomical location of the lesions, demographic data (age, sex), risk factors (diabetes, hypertension, smoking, prior stroke, atrial fibrillation, and other cardiac risk factors for stroke), large vessel involvement on magnetic resonance angiography, other symptoms and signs (sensory symptoms, gait ataxia, limb ataxia, dizziness, Horner syndrome, hoarseness, dysphagia, dysarthria, nystagmus, nausea/vomiting, headache, neck pain, diplopia, and hiccup). RESULTS: Among 44 LMI patients, 15 patients (34%) had FP, and all of them had ipsilesional central-type FP. The FP group tended to involve upper (p < 0.0001) and relative ventral (p = 0.019) part of the lateral medulla. Horizontally large lesion was also related to the presence of FP (p = 0.044). Dysphagia (p = 0.001), dysarthria (p = 0.003), and hiccups (p = 0.034) were more likely to be accompanied by FP. Otherwise, there were no significant differences. CONCLUSION: The results of present study indicate that the corticobulbar fibers innervating the lower face decussate at the upper level of the medulla and ascend through the dorsolateral medulla, where the concentration of the fibers is densest near the nucleus ambiguus.

[Developmental cerebral thrombosed venous anomaly: findings in magnetic resonance imaging]. / Anomalía venosa del desarrollo cerebral trombosada: hallazgos en la resonancia magnética.

INTRODUCTION: Developmental venous anomalies are the most common cerebral vascular malformations. They are usually incidental and benign, although about 40% are associated with cavernous malformations, and so it is essential to look for other associated vascular or neurocutaneous anomalies. CASE REPORT: We report the case of a 34-year-old pregnant woman who presented with seizures and dysarthria, and was submitted to an urgent cranial MRI scan. She showed the classic 'Medusa head' sign and was diagnosed with developmental venous anomaly with partial peripheral thrombosis and slow proximal flow. CONCLUSIONS: Thrombosis rarely occurs as a complication of developmental venous anomalies and case reports in the literature suggest that they should be managed conservatively, leaving surgery for other associated complications. As radiologists, we must be aware of the main imaging features so as to be able to make an accurate diagnosis.

TITLE: Anomalía venosa del desarrollo cerebral trombosada: hallazgos en la resonancia magnética.Introducción. Las anomalías venosas del desarrollo son las malformaciones vasculares cerebrales más comunes, suelen ser incidentales y benignas, aunque alrededor del 40% se asocian a malformaciones cavernosas, por lo que es esencial buscar otras anomalías vasculares o neurocutáneas asociadas. Caso clínico. Se presenta una mujer de 34 años embarazada que acude por convulsiones y disartria, a la cual se le realiza una resonancia magnética de cráneo urgente. En ella se aprecia el clásico signo de la 'cabeza de Medusa', y se concluye como diagnóstico anomalía venosa del desarrollo con trombosis parcial periférica y flujo lento proximal. Conclusiones. La complicación con trombosis de las anomalías venosas del desarrollo es rara y los informes de casos de la bibliografía sugieren que deben manejarse de forma conservadora, como una trombosis del seno venoso, dejando la cirugía para otras complicaciones asociadas. Como radiólogos, debemos conocer las principales características por imagen para elaborar un diagnóstico certero.

Wilson Disease: A Case Report of Psychosis Preceding Parkinsonism.

BACKGROUND A first psychotic episode requires the exclusion of toxic-metabolic, inflammatory, infective, and neoplastic causes. Wilson disease is a rare, autosomal recessive disorder of copper metabolism and can present with neuropsychiatric symptoms secondary to copper accumulation in the brain. CASE REPORT We describe the case of a 48-year-old man with parkinsonism on a background of longstanding schizophrenia and psychotic depression in the setting of previously undiagnosed Wilson disease. The common history of neuropsychiatric disturbance and neuroleptic use complicated the assessment of parkinsonism. However, close attention to the temporal appearance of symptoms and signs differentiated his case from drug-induced parkinsonism, which commonly develops hours to weeks after commencement or uptitration of antipsychotic medication. The early features of sialorrhea and dysarthria were also atypical for idiopathic Parkinson disease. The diagnosis was confirmed by serum copper testing and supported by Kayser-Fleischer rings on bedside ophthalmological examination. Magnetic resonance imaging (MRI) of the brain demonstrated copper accumulation in the basal ganglia and pons, contributing to the characteristic neurological manifestations of an akinetic-rigid syndrome with dysarthria. CONCLUSIONS Serum copper testing is easily obtained and should be considered as part of the first-line investigations for new neuropsychiatric disturbances. Although rare, Wilson disease, if diagnosed early, is a potentially treatable and reversible cause of psychosis. With advanced disease, extrapyramidal findings on examination correlate with MRI brain changes, aiding the clinical assessment in differentiating the disease from drug-induced parkinsonism.

Pearls & Oy-sters: Cognitive and Affective Dysfunction Caused by a Small Cerebellar Hemangioblastoma.

The primary function of the cerebellum is the coordination and regulation of movement; therefore, cerebellar tumors usually present with ataxia, dysarthria, and vertigo. Large tumors also cause elevated intracranial pressure that may lead to a disturbance of consciousness. Furthermore, it has become increasingly evident that the cerebellum plays a substantial role in cognitive and affective processing. A 44-year-old female patient presented with a 1-month history of depression and flat affect. She had no cerebellar symptoms including no coordination dysfunction or dysarthria. Cognitive function tests revealed impairments in attention, execution, and processing speed. Hamilton Depression Scale and Hospital Anxiety Depression Scale indicated moderate-to-severe depression. Magnetic resonance (MR) imaging revealed a 7-mm enhancing lesion in the culmen of the cerebellar vermis with surrounding edema. Technetium-99m ethyl cysteinate dimer single-photon emission tomography (SPECT) showed hypoperfusion in the left frontal lobe. Although she was initially treated with corticosteroids for presumed sero-negative autoimmune encephalitis, her symptoms persisted. She then underwent cerebellar lesion resection. The histologic diagnosis was hemangioblastoma. The patient's symptoms dramatically improved within 1 week of resection, including improved batteries for cognitive function and depression. Complete regression of cerebellar edema and left frontal lobe hypoperfusion was observed on MR and SPECT images, respectively. This case reiterates the crucial influence of the cerebellum on cognitive and affective function. Moreover, cognitive dysfunction may be masked in cases with focal cerebellar symptoms or elevated intracranial pressure and, consequently, not adequately evaluated.

Increased Improvement in Speech-Language Skills After Transcranial Photobiomodulation Plus Speech-Language Therapy, Compared to Speech-Language Therapy Alone: Case Report with Aphasia.

Objective: This is a case report showing that transcranial photobiomodulation (tPBM) combined with traditional, speech-language therapy improved and accelerated the results from speech-language therapy, in a stroke person with aphasia (PWA). Background: tPBM is a safe, noninvasive technique using red and near-infrared light to improve the metabolism of cells. tPBM helps by promoting neuromodulation, while decreasing neuroinflammation and promoting vasodilation. Several studies have shown that tPBM can help individuals with stroke or traumatic brain injury achieve significant cognitive improvements. Methods: A 38-year-old female, who sustained an ischemic stroke on the left side of the brain, received two, 5-month series of treatments. The first series of treatments included traditional speech-language therapy, for the first 5 months poststroke. The second series of treatments included tPBM in combination with speech-language therapy, for the next 5 months. The tPBM treatments included application of red (630 and 660 nm) and near-infrared (850 nm) wavelengths of photons applied to left hemisphere scalp areas. The major cortical language areas were subjacent to the scalp placements along the line of the Sylvian fissure. At each session, first a light-emitting diode (LED) cluster head with red (630 and 660 nm) and near-infrared (850 nm) wavelengths, with an irradiance (power density) of 200 mW/cm2, a beam size of 4.9 cm2, and a fluence (energy density) of 12 J/cm2 per minute, was applied to the left side of the scalp/brain, along the Sylvian fissure for 60 sec at each at the following eight, language network target areas: frontal pole, prefrontal cortex, and inferior frontal gyrus (Broca's area); supramarginal gyrus and angular gyrus in the parietal lobe; inferior motor/sensory cortex (mouth area); and posterior superior temporal gyrus (Wernicke's area) and superior temporal sulcus in the temporal lobe, for a total of 8 min. Second, for the next 20 min (1200 sec), simultaneous with speech-language therapy, an LED PBM helmet was applied to the scalp/head. This helmet contained 256 separate LED lights, near-infrared (810 nm) wavelength, 60 mW power per LED light, total power, 15 W; energy, 72 Joules; fluence, 28.8 J/cm2; and irradiance, 24 mW/cm2. Results and conclusions: During the initial, 5-month treatment series with traditional speech-language therapy only, there was little to no improvement in dysarthria and expressive language. During the second, 5-month treatment series, however, with tPBM applied first, to the left hemisphere only, and second, to both hemispheres during each session plus simultaneous speech-language therapy, there was marked improvement in the dysarthria and expressive language. After the first 5-month series, this PWA had utilized a slow rate of speech with a production of ∼25 to 30 words-per-minute during conversations and spontaneous speech. Utterance length was only 4-6 words with simple, grammatical structure. After the second, 5-month series of treatment combining tPBM plus speech-language therapy, the rate of speech increased to 80+ words-per-minute and utterance length was increased to 9-10 words, with more complex grammatical structure.

Consequências da herpes tipo 1 no desenvolvimento neuropsicomotor / Consequences of herpes type 1 on neuropsymotor development / Consecuencias del herpes tipo 1 en el desarrollo neuropsicomotor

Objetivo: Descrever as consequências do herpes tipo 1 no desenvolvimento neuropsicomotor. Metodologia: Trata-se de uma revisão narrativa das consequências da Herpes do tipo 1 no Desenvolvimento Neuropsicomotor. Questão norteadora: "Quais os efeitos da infecção congênita da Herpes tipo 1 no Desenvolvimento Neuropsicomotor observadas entre os anos de 2010 a 2020?". O levantamento dos dados ocorreu: dezembro/2021 a janeiro/2022 nas bases de dados: Scientific Eletronic Library Online, Literatura Latino Americana e do Caribe em Ciências da Saúde e National Library of Medicine. Descritores: "Herpes"; "Desenvolvimento Neuropsicomotor"; "Herpes Congênita" e "Neuropsychomotor Development" com operadores booleanos AND e OR. Critérios de inclusão: artigos originais, texto completo. Exclusão: cartas ao editor, teses, dissertações e revisões de literatura. Resultados: Foram encontrados 21 artigos nas bases de dados pesquisadas, contudo, após a aplicação dos critérios de inclusão, 3 artigos foram considerados elegíveis. Após a análise, pode-se afirmar os impactos do Vírus Herpes pode gerar ao desenvolvimento neuropsicomotor, podendo ocasionar disfagia, disartria, alterações motoras, cognitivas e sociais, bem como outros sinais e sintomas que levarão ao atraso no desenvolvimento. Conclusão: O Herpes Vírus Simples pode ocasionar transtornos cerebrais em crianças, o que acarreta distúrbios para o amadurecimento normal, com consequente atrasos no desenvolvimento neuropsicomotor.

Objective: To describe the consequences of herpes type 1 on neuropsychomotor development. Methodology: This is a narrative review of the consequences of Herpes type 1 on Neuropsychomotor Development. Guiding question: "What are the effects of congenital Herpes type 1 infection on Neuropsychomotor Development observed between the years 2010 to 2020?". Data collection took place: December/2021 to January/2022 in the following databases: Scientific Electronic Library Online, Latin American and Caribbean Literature in Health Sciences and National Library of Medicine. Keywords: "Herpes"; "Neuropsychomotor Development"; "Herpes Congenita" and "Neuropsychomotor Development" with Boolean operators AND and OR. Inclusion criteria: original articles, full text. Exclusion: letters to the editor, theses, dissertations and literature reviews. Results: 21 articles were found in the searched databases, however, after applying the inclusion criteria, 3 articles were considered eligible. After the analysis, it can be said the impacts of the Herpes Virus can generate neuropsychomotor development, which can cause dysphagia, dysarthria, motor, cognitive and social changes, as well as other signs and symptoms that will lead to developmental delay. Conclusion: Herpes Simplex Virus can cause brain disorders in children, which causes disturbances to normal maturation, with consequent delays in neuropsychomotor development.

Objetivo: Describir las consecuencias del herpes tipo 1 en el desarrollo neuropsicomotor. Metodología: Se trata de una revisión narrativa de las consecuencias del Herpes Tipo 1 en el Desarrollo Neuropsicomotor. Pregunta norteadora: "¿Cuáles son los efectos de la infección congénita por Herpes tipo 1 en el desarrollo neuropsicomotor observados entre los años 2010 y 2020?". Los datos se recopilaron entre diciembre de 2021 y enero de 2022 en las siguientes bases de datos: Scientific Eletronic Library Online, Latin American and Caribbean Literature on Health Sciences y National Library of Medicine. Descriptores: "Herpes"; "Desarrollo neuropsicomotor"; "Herpes congénito" y "Desarrollo neuropsicomotor" con operadores booleanos AND y OR. Criterios de inclusión: artículos originales, texto completo. Criterios de exclusión: cartas al director, tesis, disertaciones y revisiones bibliográficas. Resultados: Se encontraron 21 artículos en las bases de datos consultadas, sin embargo, tras aplicar los criterios de inclusión, se consideraron elegibles 3 artículos. Después del análisis, se puede afirmar los impactos que el Herpes Virus puede tener en el desarrollo neuropsicomotor, que puede causar disfagia, disartria, alteraciones motoras, cognitivas y sociales, así como otros signos y síntomas que conducirán a un retraso en el desarrollo. Conclusión: El virus del herpes simple puede causar alteraciones cerebrales en los niños, lo que conduce a trastornos de la maduración normal, con los consiguientes retrasos en el desarrollo neuropsicomotor.

ATP1A3-related phenotypes in Chinese children: AHC, CAPOS, and RECA.

The aim of this research is to study the phenotype, genotype, treatment strategies, and short-term prognosis of Chinese children with ATP1A3 (Na+/K+-ATPase alpha 3 gene)-related disorders in Southwest China. Patients with pathogenic ATP1A3 variants identified using next-generation sequencing were registered at the Children's Hospital of Chongqing Medical University from December 2015 to May 2019. We followed them as a cohort and analyzed their clinical data. Eleven patients were identified with de novo pathogenic ATP1A3 heterozygous variants. One (c.2542 + 1G > T, splicing) has not been reported. Eight patients with alternating hemiplegia of childhood (AHC), one with cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss (CAPOS), and two with relapsing encephalopathy with cerebellar ataxia (RECA) were included. The initial manifestations of AHC included hemiplegia, oculomotor abnormalities, and seizures, and the most common trigger was an upper respiratory tract infection without fever. All patients had paroxysmal hemiplegic attacks during their disease course. The brain MRI showed no abnormalities. Six out of eight AHC cases reached a stable disease state after treatment. The initial symptom of the patient with CAPOS was ataxia followed by developmental regression, seizures, deafness, visual impairment, and dysarthria, and the brain MRI indicated mild cerebellar atrophy. No fluctuation was noted after using Acetazolamide. The initial manifestations of the two RECA cases were dystonia and encephalopathy, respectively. One manifested a rapid-onset course of dystonia triggered by a fever followed by dysarthria and action tremors, and independent walking was impossible. The brain MRI image was normal. The other one presented with disturbance of consciousness, seizures, sleep disturbance, tremor, and dyskinesias. The EEG revealed a slow background (δ activity), and the brain MRI result was normal. No response to Flunarizine was noted for them, and it took 61 and 60 months for them to reach a stable disease state, respectively. CONCLUSION: Pathogenic ATP1A3 variants play an essential role in the pathogenesis of Sodium-Potassium pump disorders, and AHC is the most common phenotype. The treatment strategies and prognosis depend on the phenotype categories caused by different variation sites and types. The correlation between the genotype and phenotype requires further exploration. WHAT IS KNOWN: • Pathogenic heterozygous ATP1A3 variants cause a spectrum of neurological phenotypes, and ATP1A3-disorders are viewed as a phenotypic continuum presenting with atypical and overlapping features. • The genotype-phenotype correlation of ATP1A3-disorders remains unclear. WHAT IS NEW: • In this study, the genotypes and phenotypes of ATP1A3-related disorders from Southwest of China were described. The splice-site variation c.2542+1G>T was detected for the first time in ATP1A3-related disorders. • The prognosis of twins with AHC p. Gly947Arg was more serious than AHC cases with other variants, which was inconsistent with previous reports. The phenomenon indicated the diversity of the correlation between the genotype and phenotype.

Capecitabine-related neurotoxicity presenting with agraphia.

INTRODUCTION: Capecitabine is a pre-metabolite of 5-fluorouracil and is used as a chemotherapeutic agent. Among the common side effects of capecitabine, there are gastrointestinal side effects including nausea, vomiting, and diarrhea, and dermatological side effects including hand-foot syndrome and skin pigmentation change. However, neurological side effects of capecitabine are very rare. We describe herein a patient who developed neurological side effects in the form of agraphia and dysarthria on the 7th day of capecitabine treatment. CASE REPORT: A 34-year-old male patient, who was being followed up with the diagnosis of colon cancer, presented with speech and writing disorder that developed while under capecitabine treatment. Dysarthria and agraphia were detected in his neurological examination. Diffusion-weighted magnetic resonance imaging (MRI) revealed acute diffusion restriction in the splenium of the corpus callosum and at the level of the bilateral centrum semiovale. Brain MRI revealed symmetrical T2-weighted fluid-attenuated inversion recovery (T2-FLAIR) signal increases at the right temporoparietal medial, corpus callosum level, and bilateral white matter level. MANAGEMENT & OUTCOME: The capecitabine treatment was terminated, and methylprednisolone treatment was administered and plasmapheresis procedure was carried out. Subsequently, significant improvement was observed in the clinical findings and neuroimaging. DISCUSSION: Capecitabine is used as an oral agent; thus, it provides ease of use. Neurological side effects associated with the use of capecitabine reportedly occur very rarely. The findings of this case demonstrated that leukoencephalopathy can be seen during the use of capecitabine, imaging results are very important in the diagnosis of leukoencephalopathy, and improvement can be achieved with the termination of the capecitabine treatment.

Alterações de linguagem em pacientes pós lesão encefálica adquirida na fase aguda / Language changes in patients after brain injury acquired in the acute phase

Caracterizar as alterações de linguagem encontradas em pacientes com lesão encefálica adquirida em fase aguda Métodos: Trata-se de uma pesquisa observacional, descritiva do tipo transversal. A avaliação foi realizada nas enfermarias de um hospital de urgências, com pacientes de idade superior a 18 anos, diagnóstico de lesão encefálica adquirida e tempo de internação de até 60 dias. O protocolo utilizado incluiu avaliação das praxias orais, expressão, compreensão da linguagem oral e leitura. Ao final, foi possível apresentar as hipóteses diagnósticas de afasias fluentes e não fluentes, disartria e sem alteração de linguagem. A análise dos dados foi realizada por meio de estatística descritiva através da distribuição de frequência absoluta e frequência relativa Resultados: A amostra foi composta por 24 pacientes sendo a maioria do gênero masculino, com média de idade de 51 anos. O diagnóstico de maior frequência foi Acidente Vascular Cerebral. Dos pacientes avaliados, 79% tiveram alterações de fala/linguagem. As hipóteses diagnósticas fonoaudiológicas encontradas foram: afasia global, afasia de broca, afasia transcortical mista, afasia de condução, afasia transcortical motora, afasia transcortical sensorial e disartria. Conclusão: A afasia global foi o transtorno de linguagem de maior ocorrência entre os indivíduos, bem como o gênero masculino e o acidente vascular cerebral. A avaliação da linguagem de pacientes com lesões encefálicas adquiridas na fase aguda é pertinente, pois promove o levantamento de alterações desde as perceptíveis até as mais discretas

To characterize the language disorders found in patients with brain injury acquired in the acute phase Methods: This is an observational, descriptive cross-sectional study. The evaluation was carried out in the wards of an emergency hospital, with patients aged over 18 years, diagnosed with acquired brain injury and hospitalization time of up to 60 days. The protocol used included assessment of oral praxis, expression, comprehension of oral language and reading. In the end, it was possible to present the diagnostic hypotheses of fluent and non-fluent aphasias, dysarthria and without language alteration. Data analysis was performed using descriptive statistics through the distribution of absolute frequency and relative frequency. Results: The sample consisted of 24 patients, most of whom were male, with a mean age of 51 years. The most frequent diagnosis was Cerebral Vascular Accident. Of the evaluated patients, 79% had speech/language disorders. The speech-language diagnostic hypotheses found were: global aphasia, drill aphasia, mixed transcortical aphasia, conduction aphasia, motor transcortical aphasia, sensory transcortical aphasia and dysarthria. Conclusion: Global aphasia was the most frequent language disorder among individuals, as well as males and stroke. The evaluation of the language of patients with brain injuries acquired in the acute phase is relevant, as it promotes the survey of changes from the perceptible to the most discreet

Efeitos da estimulação transcraniana por corrente contínua (ETCC) na voz e fala na doença de Parkinson: relato de caso / Effects of transcranial direct current stimulation (tDCS) on voice and speech in Parkinson's disease: a case report

RESUMO Este estudo descreve os resultados de duas intervenções diferentes para a disartria na doença de Parkinson (DP). Trata-se de dois pacientes do sexo masculino com DP e disartria hipocinética que foram submetidos à avaliação fonoaudiológica. O primeiro (S1) recebeu dez sessões de 20 minutos de estimulação transcraniana por corrente contínua (ETCC), enquanto o segundo (S2) recebeu dez sessões de 20 minutos de ETCC e terapia fonoaudiológica. Os pacientes foram reavaliados imediatamente após a intervenção e novamente após 30 dias. A melhora no tempo de fonação, movimento velar e outras medidas de disartria foram mais significativas no S1. De acordo com os parâmetros da análise acústica de fonte glótica, a melhora nas medidas de frequência e Jitter, Shimmer e ruído foi maior no S2 tanto na avaliação pós-intervenção imediata quanto na avaliação 30 dias pós-intervenção, seguida por S1 na avaliação pós-intervenção imediata. S1 apresentou melhores resultados na avaliação perceptivo-auditiva de fala e voz, enquanto S2 obteve melhores escores na análise acústica. Esses achados sugerem que a terapia fonoaudiológica convencional associada à ETCC tem um impacto mais significativo na fala e voz do que a ETCC isolada, demonstrando o potencial da ETCC como tratamento complementar para pacientes com DP.

ABSTRACT This study describes the results of two different interventions for dysarthria in Parkinson's disease (PD). It concerns two male patients with PD and hypokinetic dysarthria who underwent speech and voice assessment. The first (S1) received ten 20-minute sessions of transcranial direct current stimulation (tDCS), while the second (S2) received ten 20-minute sessions of tDCS as well as speech therapy. The patients were re-evaluated immediately after the intervention and again after 30 days. Improvements in phonation time, velar movement, and other measures of dysarthria were more significant in S1. According to the acoustic analysis source parameters, improvements in the frequency measurements and Jitter, Shimmer, and noise measurements were greater in S2 in both the immediate post-intervention and 30-day postintervention assessments, followed by S1 in the immediate post-intervention assessment. S1 showed better results in the auditory-perceptual evaluation of speech and voice, whereas S2 obtained better scores in acoustic analysis. These findings suggest that conventional speech therapy combined with tDCS has a more significant impact on speech and voice than tDCS alone, demonstrating the potential of tDCS as an adjuvant treatment for patients with PD.

Parâmetros e tipos de avaliação da disartria na esclerose lateral amiotrófica / Parameters and types of dysarthria assessment in amyotrophic lateral sclerosis

RESUMO Objetivo identificar estudos a respeito dos parâmetros e dos tipos de avaliação utilizados para avaliar a disartria na esclerose lateral amiotrófica (ELA). Estratégia de pesquisa estudo de revisão integrativa da literatura realizada nas bases de dados LILACS, SciELO, PubMed, Web of Science, CINAHL, Scopus e Cochrane, por meios dos descritores, em português e em inglês, "Avaliação AND Disartria AND Esclerose Lateral Amiotrófica". Critérios de seleção os critérios de inclusão foram: artigos que abordavam estudos sobre avaliação da disartria na ELA, nas línguas inglesa, espanhola e portuguesa, disponíveis na íntegra, no período de 2015 a 2022. Resultados do total de 38 estudos, apenas 3 usaram um único tipo de avaliação da disartria. A maior parte dos estudos utilizou mais de um tipo de avaliação variando de 2 a 4. Foram 3 os tipos de avaliação mais utilizados, com o intuito de avaliar o grau de inteligibilidade de fala: avaliação perceptivo-auditiva (31 estudos), avaliação acústica (18 estudos) e avaliação do movimento (27 estudos). Conclusão a avaliação da disartria na ELA é realizada por diferentes procedimentos e com vários parâmetros de análise, em especial pela avaliação perceptivo-auditiva e do movimento.

ABSTRACT Purpose to identify studies regarding the parameters and types of assessment used to evaluate dysarthria in amyotrophic lateral sclerosis (ALS). Research strategy an integrative literature review study was conducted on the LILACS, SciELO, PubMed, Web of Science, CINAHL, Scopus, and Cochrane databases using the descriptors "Assessment AND Dysarthria AND Amyotrophic Lateral Sclerosis" in both Portuguese and English. Selection criteria the inclusion criteria consisted of articles that addressed studies on dysarthria assessment in ALS, written in English, Spanish, and Portuguese, which should be available in full, and published from 2015 to 2022. Results: out of the total of 38 studies, only 3 used a single type of dysarthria assessment. Most studies employed more than one type of assessment, ranging from 2 to 4 types. Three assessment types were predominantly used to assess the degree of speech intelligibility: auditoryperceptual assessment (31 studies), acoustic assessment (18 studies), and movement assessment (27 studies). Conclusion dysarthria assessment in ALS is conducted through various procedures and with multiple analysis parameters, notably through auditory-perceptual and movement assessments.

Correlation of magnetic resonance images with neuropathology of irreversible metronidazole-induced encephalopathy: an autopsy case report.

BACKGROUND: Neurological symptoms and radiographic abnormalities may remain in a small proportion of patients with metronidazole-induced encephalopathy (MIE). Although experimental animal models of MIE have suggested a Wernicke's encephalopathy-like pathology, little is known about the histopathological features of MIE. Here we report the first autopsy case of irreversible MIE. CASE PRESENTATION: A 72-year-old Japanese woman with pancreatic neuroendocrine tumour and metastatic tumours in the liver developed intraabdominal bleeding from a hepatic abscess. She was administered metronidazole for 79 days (1.5 g/day), which caused dysarthria followed by hand tremor and altered mental status. Brain magnetic resonance imaging at the time of onset revealed hyperintensities in the deep white matter of the bilateral parietal lobes and splenium of the corpus callosum on diffusion-weighted imaging (DWI) with reduced apparent diffusion coefficient (ADC) values. Despite the improvement of dysarthria and hand tremor, her cognition remained affected even after the withdrawal of metronidazole. She died of pancreatic neuroendocrine tumour at the age of 74 years. Histopathological examinations of the brain confirmed a combination of severe demyelination and moderate axonal degeneration, which corresponded to the regions showing abnormal signal intensities on DWI with reduced ADC values. There were no pathological findings suggestive of Wernicke's encephalopathy in the brain. CONCLUSION: We have demonstrated the clinical, radiographic and histopathological aspects of irreversible MIE. Hyperintensities on DWI with reduced ADC values in affected regions may indicate a poor clinical prognosis due to irreversible pathological damage.

Correlates of vowel clarity in the spectrotemporal modulation domain: Application to speech impairment evaluation.

This article reports on vowel clarity metrics based on spectrotemporal modulations of speech signals. Motivated by previous findings on the relevance of modulation-based metrics for speech intelligibility assessment and pathology classification, the current study used factor analysis to identify regions within a bi-dimensional modulation space, the magnitude power spectrum, as in Elliott and Theunissen [(2009). PLoS Comput. Biol. 5(3), e1000302] by relating them to a set of conventional acoustic metrics of vowel space area and vowel distinctiveness. Two indices based on the energy ratio between high and low modulation rates across temporal and spectral dimensions of the modulation space emerged from the analyses. These indices served as input for measurements of central tendency and classification analyses that aimed to identify vowel-related speech impairments in French native speakers with head and neck cancer (HNC) and Parkinson dysarthria (PD). Following the analysis, vowel-related speech impairment was identified in HNC speakers, but not in PD. These results were consistent with findings based on subjective evaluations of speech intelligibility. The findings reported are consistent with previous studies indicating that impaired speech is associated with attenuation in energy in higher spectrotemporal modulation bands.

Relato da experiência da atuação fonoaudiológica em telessaúde em pacientes neurológicos com disfagia e disartria durante a pandemia de Covid-19 / Experience report of speech therapy in telehealth with neurological patients with dysphagia and dysarthria during the Covid-19 pandemic / Reporte de experiencia de desempeño logopédico en telesalud en pacientes neurológicos con disfagia y disartria durante la pandemia de Covid-19

Introdução: Devido à COVID-19, os pacientes com doenças neurológicas deixaram de frequentar presencialmente as consultas fonoaudiológicas em ambulatórios. Objetivo: Descrever o relato da experiência fonoaudiológica em pacientes com doença neurológica com disartria e/ou disfagia durante a pandemia da COVID-19 através da telessaúde. Método: Trata-se de um relato de experiência. Foram incluídos pacientes do ambulatório de fonoaudiologia de um hospital universitário, que ficaram privados do acompanhamento fonoaudiológico em período pandêmico e que tinham diagnóstico de disfagia e/ou disartria (prévios à pandemia). No total, 43 pacientes foram convidados a participar do estudo. Os indivíduos foram separados de acordo com seu diagnóstico fonoaudiológico: disfagia, disartria e disfagia/disartria. No início, todos foram reavaliados em videochamadas: disfagia (Northwestern dysphagia patient check sheet, Escala Funcional de Ingestão Via Oral e Instrumento de Autoavaliação da Alimentação); disartria (coleta de fala e questionário de autopercepção Radbould Oral Inventory Motor for Parkinson's disease). Após, os pacientes foram alocados aleatoriamente: teleatendimento fonoaudiológico por quatro semanas consecutivas, sendo o outro grupo controle, sem intervenções e/ou orientações. Todos foram reavaliados para a comparação pré e pós-acompanhamento fonoaudiológico. Resultados: Nove participantes concluíram todas as etapas do estudo, sendo 6 (66,66%) homens. A média de idade foi de 60,44 anos (±16,13). Os participantes possuíam diagnóstico médico de doença neurológica, sendo 2 neurogenética (22,22%), 5 neurodegenerativa (55,5%) e 2 neurológicas (22,22%). Não foram observadas diferenças descritivas entre os grupos nas avaliações pré e pós-intervenção. A perda na amostra aconteceu devido à falta de dispositivos tecnológicos e à sobrecarga dos cuidadores. Conclusões: A experiência em tele fonoaudiologia, apesar de ter sido positiva, revelou a dificuldade da sua implementação em pacientes neurológicos de baixa condições sócio financeiras e educacional.

Introduction: Due to COVID-19, patients with neurological disease no longer attend face-to-face speech therapy consultations in outpatient clinics. Objective: To describe the report of the speech therapy experience patients with neurological disease with dysarthria and/or dysphagia during the COVID-19 pandemic through telehealth. Method: This is an experience report. Patients from the speech therapy outpatient clinic of a university hospital who were deprived of speech therapy during a pandemic period and had a diagnosis of dysphagia and/or dysarthria (prior to the pandemic) were included. In total, 43 patients were invited to participate in the study. Individuals were separated according to their speech-language diagnosis: dysphagia, dysarthria, and dysphagia/dysarthria. In the beginning, all were reassessed in video calls: dysphagia (Northwestern dysphagia patient check sheet, Functional Oral Intake Scale, and Food Self-Assessment Instrument); dysarthria (speech collection and self-perception questionnaire Radbould Oral Motor Inventory for Parkinson's disease). Afterward, the patients were randomly allocated: speech therapy telecare for four consecutive weeks, with the other being a control group, without interventions and/or guidance. All were reassessed for comparison before and after speech therapy follow-up. Results:Nine participants completed all stages of the study, 6 (66.66%) men. The mean age was 60.44 years (±16.13). Participants had a medical diagnosis of neurological disease, 2 of which were neurogenetic (22.22%), five neurodegenerative (55.5%), and two neurologic (22.22%). No descriptive differences were observed between groups in pre- and post-intervention assessments. The loss in the sample happened due to the lack of technological devices and the overload of caregivers. Conclusions: The experience in telehealth was positive, revealing the difficulty of its implementation in neurological patients with low socio-financial and educational conditions.

Introducción: Debido al COVID-19, los pacientes con enfermedades neurologicas ya no asisten a consultas de logopedia presenciales en consultas externas. Objetivo: Describir el relato de la experiencia fonoaudiológica en pacientes con enfermedades neurologicas con disartria y/o disfagia durante la pandemia de COVID-19 a través de telesalud. Método: Este es un relato de experiencia. Se incluyeron pacientes de la consulta externa de logopedia de un hospital universitario, que fueron privados de logopedia durante un período de pandemia y que tenían diagnóstico de disfagia y/o disartria (previo a la pandemia). En total, 43 pacientes fueron invitados a participar en el estudio. Los individuos se separaron según su diagnóstico del habla y el lenguaje: disfagia, disartria y disfagia/disartria. Al principio, todos fueron reevaluados en videollamadas: disfagia (Northwestern dysphagia patient check sheet), Escala de ingesta oral funcional e Instrumento de autoevaluación de alimentos); disartria (cuestionario de recogida de voz y autopercepción Radbould Oral Motor Inventory for Parkinson's disease). Posteriormente, los pacientes fueron asignados aleatoriamente: teleasistencia logopédica durante cuatro semanas consecutivas, siendo el otro grupo control, sin intervenciones y/u orientaciones. Todos fueron reevaluados para compararlos antes y después del seguimiento con logopedia. Resultados: Nueve participantes completaron todas las etapas del estudio, 6 (66,66%) hombres. La edad media fue de 60,44 años (±16,13). Los participantes tenían diagnóstico médico de enfermedad neurológica, 2 de ellas neurogenéticas (22,22%), 5 neurodegenerativas (55,5%) y 2 neurológica (22,22%). No se observaron diferencias descriptivas entre los grupos en las evaluaciones previas y posteriores a la intervención. La pérdida en la muestra ocurrió por la falta de dispositivos tecnológicos y la sobrecarga de cuidadores. Conclusiones: La experiencia en telefonoaudiología, a pesar de ser positiva, reveló la dificultad de su implementación en pacientes neurológicos de baja condición socioeconómica y educativa.

Severe Hyperkalemia Masquerading as Acute Ischemic Stroke: A Case Report.

Severe hyperkalemia usually presents as cardiac or neurologic manifestations. We report a case of a 63-year-old Caucasian woman, who was admitted to Namazi Hospital, affiliated with Shiraz University of Medical Sciences (Shiraz, Iran) in August 2019. The patient suffered from left-sided weakness and slurred speech for one hour prior to admission. Initially, the patient was treated for acute ischemic stroke, and an intravenous recombinant tissue plasminogen activator (IV-rTPA) was prescribed. However, further investigations showed severe hyperkalemia. Hemiparesis and slurred speech improved significantly with appropriate management of hyperkalemia. To the best of our knowledge, this is the first case of hyperkalemia masquerading as acute ischemic stroke without evidence of concomitant central nervous system malignancies, large vessel atherosclerosis, or recreational drug abuse. Stroke mimics due to hyperkalemia should be considered in any patient with simultaneous sudden onset of focal neurologic deficits and tall peaked T waves, particularly in the context of renal failure and a history of potassium-sparing drug use.