Functional ataxia in a specialized ataxia center.

BACKGROUND: Functional gait is a disorder of ambulation and balance internally inconsistent and incongruent with the phenotypic spectrum of neurological gait disorders. OBJECTIVES: This paper aims to clinically characterize patients with functional ataxia. METHODS: Patients with functional ataxia were analyzed out of 1350 patients in Ataxia Unit of the Federal University of São Paulo circa 2008 to 2022. RESULTS: Thirteen patients (1 %) presented with functional ataxia; all female, with a median age of 34.8 years. Six (46.2 %) had psychiatric comorbidities and 7 (53.8 %) endorsed a trigger. Diagnostic features included variable base and stride (100 %), "huffing and puffing" (30.7 %), knee-buckling (30.7 %), uneconomic posturing (38.5 %), tightrope walking (23 %), and trembling gait (15.4 %). Remarkably, no falls were reported in any case. 53.8 % recovered fully or partially, despite no treatment. CONCLUSIONS: Variability of base and stride are universal features of functional ataxia, yet falls are inconspicuous. Functional Ataxia is rare even in a specialized ataxia center.

Effects of COVID-19 on auditory and vestibular function in an adult patient: a longitudinal case study / Efeitos da COVID-19 na função auditiva e vestibular em paciente adulta: um estudo de caso longitudinal

ABSTRACT COVID-19 is a viral infection that mainly affects the lower respiratory tract and with the affinity of the Spike protein to the Angiotensin 2 converter enzyme, inducing a prothrombotic state which may lead to compromising of the cardiovascular, central nervous, auditory, and vestibular systems. Viral infections knowingly affect vestibular and auditory systems. This study aimed to present the auditory and vestibular findings of a woman without otoneurological complaints, before the infection with SARS-CoV-2. This is a longitudinal case report, presenting the evolution of the otoneurological clinical picture accompanied by the results of a battery of tests. The results evidenced a unilateral, permanent, cochlear sensorineural hearing loss through altered otoacoustic emissions albeit with normal tonal thresholds, accompanied by peripheral vestibular hypofunction which worsened with time, without any other factor that could justify these findings. This study shows the importance of the complete protocol of tests performed over time for a better understanding of long-time implications of the SARS-CoV-2 infection.

RESUMO A COVID-19 é uma infecção viral que afeta, principalmente, a via respiratória inferior e, com a afinidade da proteína Spike com a Enzima Conversora de Angiotensina 2, gerando estado protrombótico, pode haver comprometimento dos sistemas cardiovasculares, nervoso central e renal. As infecções virais são capazes, também, de causar alterações nos sistemas auditivo e vestibular. O presente estudo teve por finalidade apresentar os achados auditivos e vestibulares de uma mulher sem queixas otoneurológicas, previamente à infecção pela SARS-CoV-2. Trata-se de um relato de caso longitudinal, com apresentação da evolução do quadro clínico otoneurológico acompanhado de uma bateria de exames. Os resultados evidenciaram o aparecimento de uma perda auditiva neurossensorial unilateral prevista por emissões otoacústicas alteradas na presença limiares tonais normais. Com a sequência de exames, observou-se piora dos limiares auditivos e alteração da função vestibular, de maneira permanente, sem qualquer outro histórico que pudesse justificar a instalação do quadro. Em quadros infecciosos faz-se importante a longitudinalidade de avaliação com uso de protocolo completo de exames para melhor compreensão dos efeitos tardios.

Electroencephalographic findings among inpatients with COVID-19 in a tertiary hospital from a middle-income country.

BACKGROUND: In 2019, the world witnessed the emergence of a new type of coronavirus - the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The spectrum of coronavirus disease 2019 (COVID-19) is variable, and amongst its manifestations are neurological implications. OBJECTIVE: This report aimed to describe electroencephalographic findings in COVID-19 patients from a general tertiary hospital in São Paulo, Brazil. METHODS: It was a retrospective, observational, and non-interventional study. Data were collected anonymously, comprising inpatients from Mar 1 to Jun 30, 2020, either confirmed (positive RT-PCR) or probable cases (CO-RADS 4/5) who had performed EEG during hospitalization. RESULTS: Twenty-eight patients were enrolled, 17 (60.7%) women and 11 men, with a median age of 58 (minimum and maximum: 18-86; IQR 23.5). COVID-19 diagnosis was confirmed in 22 (78.5%). Twenty-one patients (75%) had severe disease, requiring mechanical ventilation due to acute respiratory distress syndrome (ARDS); 16 (57.1%) patients developed adjunct sepsis throughout hospitalization. There was no specific pattern found for COVID-19 in EEG. No patients presented with status epilepticus or electrographic events; most patients developed an encephalopathic pattern, as seen in most studies, with a high prevalence of altered mental status as an indication for EEG. Adjunct sepsis was associated with higher mortality. CONCLUSIONS: EEG presents as a useful tool in the context of COVID-19, as in other conditions, to differentiate nonconvulsive status epilepticus (NCSE) from encephalopathy and other causes of mental status alterations. Further studies are required to analyze whether there might be a specific EEG pattern to the disease.

Electroencephalographic findings among inpatients with COVID-19 in a tertiary hospital from a middle-income country / Achados eletroencefalográficos entre pacientes internados com COVID-19 em um hospital terciário de um país em desenvolvimento

ABSTRACT Background: In 2019, the world witnessed the emergence of a new type of coronavirus - the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The spectrum of coronavirus disease 2019 (COVID-19) is variable, and amongst its manifestations are neurological implications. Objective: This report aimed to describe electroencephalographic findings in COVID-19 patients from a general tertiary hospital in São Paulo, Brazil. Methods: It was a retrospective, observational, and non-interventional study. Data were collected anonymously, comprising inpatients from Mar 1 to Jun 30, 2020, either confirmed (positive RT-PCR) or probable cases (CO-RADS 4/5) who had performed EEG during hospitalization. Results: Twenty-eight patients were enrolled, 17 (60.7%) women and 11 men, with a median age of 58 (minimum and maximum: 18-86; IQR 23.5). COVID-19 diagnosis was confirmed in 22 (78.5%). Twenty-one patients (75%) had severe disease, requiring mechanical ventilation due to acute respiratory distress syndrome (ARDS); 16 (57.1%) patients developed adjunct sepsis throughout hospitalization. There was no specific pattern found for COVID-19 in EEG. No patients presented with status epilepticus or electrographic events; most patients developed an encephalopathic pattern, as seen in most studies, with a high prevalence of altered mental status as an indication for EEG. Adjunct sepsis was associated with higher mortality. Conclusions: EEG presents as a useful tool in the context of COVID-19, as in other conditions, to differentiate nonconvulsive status epilepticus (NCSE) from encephalopathy and other causes of mental status alterations. Further studies are required to analyze whether there might be a specific EEG pattern to the disease.

RESUMO Antecedentes: Em 2019, testemunhou-se o surgimento de um novo tipo de coronavírus - o coronavírus associado à síndrome da angústia respiratória tipo 2 (SARS-CoV-2). O espectro da doença associada ao novo coronavírus, a COVID-19, é variável e, dentre suas manifestações, há implicações neurológicas. Objetivos: O presente estudo objetivou descrever achados eletroencefalográficos em pacientes com COVID-19 internados em um hospital terciário em São Paulo. Métodos: Tratou-se de estudo observacional, retrospectivo e não-intervencionista, realizado por meio de coleta anônima e retrospectiva de dados de prontuário médico de pacientes com diagnóstico confirmado (RT-PCR positivo) ou provável (CO-RADS 4 ou 5), que realizaram eletroencefalograma durante internação hospitalar. Resultados: Vinte e oito pacientes foram elencados, 17 (60,7%) mulheres e 11 homens. O diagnóstico de COVID-19 foi confirmado em 22 (78,5%) dos casos. Dos pacientes, 21 (75%) apresentaram a doença, requerendo suporte ventilatório, e 16 (57,1%) desenvolveram sepse sobreposta. Não houve padrão específico de EEG para COVID-19, e nenhum paciente apresentou estado de mal epiléptico ou crise eletrográfica; a maioria desenvolveu padrão de encefalopatia, com alentecimento da atividade cerebral, sendo a alta prevalência de alteração de estado mental a indicação para o exame. A sepse sobreposta foi associada a um pior desfecho, com maior mortalidade. Conclusão: No contexto da COVID-19, o EEG figura como ferramenta importante, auxiliando, como em outras condições, na diferenciação entre estado de mal epiléptico, encefalopatia e outras causas de alteração do estado mental. Estudos adicionais são necessários para avaliar a existência de padrão específico de alteração eletroencefalográfica na COVID-19.

Acute Disseminated Encephalomyelitis in COVID-19: presentation of two cases and review of the literature.

BACKGROUND: Neurological manifestations of COVID-19 are still incompletely understood. Neurological manifestations may be due to direct viral effect on neurons and glial cells, to an immune-mediated response to the virus, or to a hypercoagulable state and associated endothelial damage, as well as to severe systemic disease with prolonged intensive care unit stay. OBJECTIVE: To describe two patients with severe SARS-CoV-2 infection and delayed recovery of consciousness after sedation withdrawal, in whom MRI disclosed multifocal white matter brain lesions, compatible with the diagnosis of acute disseminated encephalomyelitis. METHODS: Observational report of two cases of severe COVID-19 infection in patients from two tertiary hospitals in São Paulo, Brazil. RESULTS: These patients underwent neurologic and systemic evaluation for delayed awakening after sedation withdrawal. MRI displayed multifocal centrum semiovale lesions, suggestive of demyelinating inflammation. Cerebrospinal fluid (CSF) polymerase chain reaction (PCR) for SARS-CoV-2 was negative in both cases. CONCLUSION: A recurrent pattern of multifocal white matter lesions can occur in COVID-19 patients, possibly associated with delayed awakening. Additional studies are necessary to elucidate the role of the viral infection and of inflammatory and immune-mediated associated changes in neurological manifestations of COVID-19.

Acute Disseminated Encephalomyelitis in COVID-19: presentation of two cases and review of the literature / Encefalomielite disseminada aguda associada a COVID-19: relato de dois casos e revisão de literatura

ABSTRACT Background: Neurological manifestations of COVID-19 are still incompletely understood. Neurological manifestations may be due to direct viral effect on neurons and glial cells, to an immune-mediated response to the virus, or to a hypercoagulable state and associated endothelial damage, as well as to severe systemic disease with prolonged intensive care unit stay. Objective: To describe two patients with severe SARS-CoV-2 infection and delayed recovery of consciousness after sedation withdrawal, in whom MRI disclosed multifocal white matter brain lesions, compatible with the diagnosis of acute disseminated encephalomyelitis. Methods: Observational report of two cases of severe COVID-19 infection in patients from two tertiary hospitals in São Paulo, Brazil. Results: These patients underwent neurologic and systemic evaluation for delayed awakening after sedation withdrawal. MRI displayed multifocal centrum semiovale lesions, suggestive of demyelinating inflammation. Cerebrospinal fluid (CSF) polymerase chain reaction (PCR) for SARS-CoV-2 was negative in both cases. Conclusion: A recurrent pattern of multifocal white matter lesions can occur in COVID-19 patients, possibly associated with delayed awakening. Additional studies are necessary to elucidate the role of the viral infection and of inflammatory and immune-mediated associated changes in neurological manifestations of COVID-19.

RESUMO Introdução: As manifestações neurológicas causadas pela COVID-19 ainda não estão completamente elucidadas. O comprometimento neurológico pode decorrer de um efeito viral direto em neurônios ou em células gliais, a efeito imunomediado em resposta à infecção viral, ou de um efeito secundário a estados de hipercoagulabilidade e danos endoteliais, assim como decorrente de complicações sistêmicas graves relacionadas a cuidados intensivos prolongados na unidade de terapia intensiva. Objetivo: Descrever dois pacientes com recuperação tardia do nível de consciência após a retirada da sedação associados à infecção grave pelo SARS-CoV-2, que apresentaram lesões multifocais de substância branca, compatíveis com o diagnóstico de encefalomielite disseminada aguda. Métodos: Estudo observacional, com relato de dois casos de infecção grave pela COVID-19, em dois hospitais terciários na cidade de São Paulo, Brasil. Resultados: Os pacientes foram submetidos à investigação sistêmica e neurológica para avaliação de estado alterado de consciência após retirada de sedação. A ressonância magnética de crânio evidenciou lesões multifocais no centro semioval, sugestivos de processo inflamatório desmielinizante. Análise liquórica evidenciou PCR negativo para SARS-CoV-2 em ambos os casos. Conclusão: Lesões multifocais de substância branca podem ocorrer em pacientes com COVID-19, possivelmente associadas a estados alterados de consciência. Estudos adicionais são necessários para determinar o processo fisiopatológico da infecção viral e dos estados inflamatórios e imunomediados na gênese das manifestações neurológicas causadas pela COVID-19.

Audição em fumantes: uma revisão / The hearing of smokers: a review

RESUMO Dentre os fatores que influenciam o aparecimento de alterações auditivas pode-se citar o tabagismo, já que estudos revelam que o uso do tabaco pode causar um efeito ototóxico na audição. O objetivo do presente estudo foi analisar as produções científicas sobre o efeito do cigarro no sistema auditivo. A metodologia constou de levantamento dos periódicos indexados nas bases de dados, e foram incluídos na revisão de literatura os artigos científicos nacionais e internacionais, publicados em língua portuguesa ou inglesa, entre os anos de 2009 a 2016. Posteriormente a seleção e análise dos artigos na íntegra, foram classificados de acordo com a sua natureza, caracterizando-os em cinco categorias: base de dados, título do artigo científico, autor, ano e país da publicação. Em seguida, foram analisados os critérios utilizados pelos autores dos respectivos artigos, levando em consideração a amostra populacional, a faixa etária e o sexo. Por fim, foram feitas as análises dos exames auditivos utilizados em fumantes e os critérios de exclusão de cada estudo. Assim, por meio dos estudos publicados conclui-se que o uso do tabaco influencia no aparecimento de perda auditiva, prejudicando principalmente os limiares auditivos das altas frequências e inclusive a transmissão neural da informação auditiva.

ABSTRACT Amidst the factors that may cause hearing impairment, one can name smoking, since studies have shown that use of tobacco might have an ototoxic effect. The aim of this revision was to analyse scientific productions regarding smoking effects on the auditory system. Methodology was composed by database research, in which national and international scientific articles, in English or Portuguese, published from 2009 to 2016, were included. After being selected and examined, the articles were classified according to their type, and characterised into five categories: database, article title, author, year and country of publication. Afterwards, the criteria used by each author in their production were evaluated, considering population sample, age span and gender. Finally, analysis of auditory tests used in smokers and exclusion criteria of each study was performed. Thus, through the published studies, it was concluded that the use of tobacco influences the onset of hearing loss, damaging mostly high frequencies hearing thresholds and neural transmission of audio information.