Effects of onabotulinum toxin type A injections in patients with Meige's syndrome.

BACKGROUND: Meige's syndrome is a type of facial dystonia characterized by the simultaneous occurrence of blepharospasm and oromandibular dystonia. Although botulinum toxin type A (OBTA) injections are the standard treatment, evidence of their effectiveness and safety in this scenario is still lacking. OBJECTIVE: Our research aimed to evaluate the improvement and occurrence of side effects following injections of onabotulinum toxin type A (OBTA) in patients with Meige's syndrome. METHODS: Patients with Meige's syndrome undergoing botulinum toxin injections were enrolled in this study. We assessed dystonia intensity before and 14 days after OBTA injection using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) to measure the response of symptoms in the eyes (blepharospasm) and mouth (oromandibular dystonia). Other variables, such as dosage, side effects, and demographic data, were also recorded. RESULTS: The study included 41 participants, with a mean age of 67.7 years and a female-to-male ratio of 3.5:1. The mean BFMDRS score before the injections was 8.89, and after 14 days, it was 2.88. The most reported side effect was ptosis, with a 7.3% incidence. OBTA significantly reduced dystonia severity (p < 0.0001). The clinical response for the blepharospasm component was superior to the oromandibular dystonia component. CONCLUSION: Our results support that OBTA seems to be an effective and safe therapeutic option for treating Meige's syndrome. The effect of OBTA was more pronounced in the treatment of blepharospasm than in oromandibular dystonia.

ANTECEDENTES: A síndrome de Meige (SM) é caracterizada pela ocorrência concomitante de blefarospasmo e distonia oromandibular. Embora a toxina onabotulínica do tipo A (TBA) seja o tratamento de escolha, há uma falta de evidências sobre sua eficácia e segurança nesse cenário. OBJETIVO: O objetivo do nosso estudo foi avaliar os efeitos obtidos com a aplicação de TBA em pacientes com SM. MéTODOS: Pacientes com SM que realizam aplicação de TBA foram convidados a participar desse estudo. Os participantes foram questionados sobre a intensidade da distonia antes e 14 dias após a injeção de TBA, utilizando a Escala de Distonia de Burke-Fahn-Marsden (EDBFM) para mensurar a resposta obtida em cada segmento. Outras variáveis, como dose, ocorrência de efeitos colaterais e dados demográficos, também foram registradas. RESULTADOS: O estudo contou com 41 participantes (idade média de 67,7; razão de 3,5 pacientes do sexo feminino para cada participante do sexo masculino). O escore médio na EDBFM antes das aplicações de TBA era 8,89, e, após 14 dias, 2,88. O efeito colateral mais reportado foi ptose (7.3%). A TBA foi capaz de reduzir a severidade da distonia (p < 0.0001), principalmente do blefarospasmo. CONCLUSãO: Nossos resultados corroboram que a TBA é uma terapêutica eficaz e segura no tratamento da SM. O efeito da TBA é superior no manejo do blefarospasmo em relação à distonia oromandibular.

"I'm gonna lose my strength, I'm gonna seize and die, And all that Jazz"! Neurological diseases in jazz legends / "I'm gonna lose my strength, I'm gonna seize and die, And all that Jazz"! Doenças neurológicas em lendas do jazz

ABSTRACT Even though jazz is a musical style that excels in improvisation and virtuosity, it is not without its share of anecdotes, drama, and downright tragedy, and the biographies of jazz musicians and their demise are fraught with ominous and dire straits. Unsurprisingly, some would develop chronic and fatal diseases. The neurological diseases that afflicted the following six composers and musicians, all of whom are considered jazz legends, are briefly discussed: Charles Mingus, diagnosed with amyotrophic lateral sclerosis; Lester Young and Charlie Parker, both diagnosed with neurosyphilis; Thelonius Monk, who had possible frontotemporal dementia; George Gershwin, who died as a result of brain glioma; and Cole Porter, who developed phantom limb pain following an amputation. The association of lifestyles, with drug abuse, particularly alcohol and heroin, in addition to great sexual promiscuity factors contributed to the development of a series of diseases such as syphilis. In addition, we also described some fatalities such as neurodegenerative diseases and cerebral glioma.

RESUMO Embora o jazz seja um estilo musical que prima pela improvisação e pelo virtuosismo, não é isento de drama e tragédia, e as biografias dos músicos de jazz e a sua morte estão repletas de dificuldades sinistras e terríveis. Alguns desenvolveriam doenças crônicas e fatais. São brevemente discutidas as doenças neurológicas que afligiram os seguintes seis compositores e músicos, todos eles considerados lendas do jazz: Charles Mingus, diagnosticado com esclerose lateral amiotrófica; Lester Young e Charlie Parker, ambos com neurosífilis; Thelonius Monk, que teve uma possível demência frontotemporal; George Gershwin, que morreu em decorrência de glioma cerebral; e Cole Porter, que desenvolveu dor de membro fantasma após uma amputação. A associação do estilo de vida com o abuso de drogas, particularmente álcool e heroína, além de promiscuidade sexual, contribuiu para o desenvolvimento de uma série de doenças, por exemplo, a sífilis. Também descrevemos algumas fatalidades, como doenças neurodegenerativas e glioma cerebral.

"I'm gonna lose my strength, I'm gonna seize and die, And all that Jazz"! Neurological diseases in jazz legends.

Even though jazz is a musical style that excels in improvisation and virtuosity, it is not without its share of anecdotes, drama, and downright tragedy, and the biographies of jazz musicians and their demise are fraught with ominous and dire straits. Unsurprisingly, some would develop chronic and fatal diseases. The neurological diseases that afflicted the following six composers and musicians, all of whom are considered jazz legends, are briefly discussed: Charles Mingus, diagnosed with amyotrophic lateral sclerosis; Lester Young and Charlie Parker, both diagnosed with neurosyphilis; Thelonius Monk, who had possible frontotemporal dementia; George Gershwin, who died as a result of brain glioma; and Cole Porter, who developed phantom limb pain following an amputation. The association of lifestyles, with drug abuse, particularly alcohol and heroin, in addition to great sexual promiscuity factors contributed to the development of a series of diseases such as syphilis. In addition, we also described some fatalities such as neurodegenerative diseases and cerebral glioma.

Beyond 'A Clinical Lesson at La Salpêtrière': a brief assessment of André Brouillet's other paintings on medical subjects, life, and times / Além de 'Une Leçon Clinique à La Salpêtrière': uma breve apreciação das outras pinturas sobre temas médicos, vida e época de André Brouillet

ABSTRACT André Brouillet's (1857-1914) famous group tableau 'A Clinical Lesson at La Salpêtrière' (French: Une leçon clinique à la Salpêtrière) is possibly the most celebrated painting in the history of neurology. His depiction of one of Jean-Martin Charcot's legendary "Tuesday Lessons" includes portraits of not only one of the master's most famous patients, but also of his pupils, the heirs to the founder of modern neurology. However, the painter himself has long been neglected, and even his other paintings on medical subjects are little acknowledged. The authors aim to bring attention to Brouillet's life and times, as well as the remainder of his notable works; and in giving a proper context to the famous painting, neurologists today may be able to appreciate better the early history of our field and its cultural impact.

RESUMO A famosa pintura 'Une leçon clinique à la Salpêtrière', de André Brouillet's (1857-1914), é possivelmente a representação mais célebre da história da Neurologia. Seu retrato de uma das lendárias "lições de terça-feira" dirigidas por Jean-Martin Charcot inclui ainda não apenas uma das pacientes mais famosas do mestre como também seus pupilos, os fundadores da Neurologia moderna. Entretanto, o pintor propriamente dito é ainda negligenciado, e mesmo outras pinturas suas sobre temas médicos são pouco reconhecidas. Os autores trazem à atenção a vida e época de Brouillet, bem como o restante de seus outros trabalhos notáveis; dando contexto apropriado à pintura, neurologistas atuais podem compreender melhor a própria história de nossa especialidade e seu impacto cultural.

Beyond 'A Clinical Lesson at La Salpêtrière': a brief assessment of André Brouillet's other paintings on medical subjects, life, and times.

André Brouillet's (1857-1914) famous group tableau 'A Clinical Lesson at La Salpêtrière' (French: Une leçon clinique à la Salpêtrière) is possibly the most celebrated painting in the history of neurology. His depiction of one of Jean-Martin Charcot's legendary "Tuesday Lessons" includes portraits of not only one of the master's most famous patients, but also of his pupils, the heirs to the founder of modern neurology. However, the painter himself has long been neglected, and even his other paintings on medical subjects are little acknowledged. The authors aim to bring attention to Brouillet's life and times, as well as the remainder of his notable works; and in giving a proper context to the famous painting, neurologists today may be able to appreciate better the early history of our field and its cultural impact.

" In the immortal forms of beauty": Frédéric Chopin seen through the eyes of the father of Brazilian neurological semiology.

Aloysio de Castro (1881-1959) is now remembered as one of Brazil's greatest physicians and is considered the father of Brazilian neurological semiology. However, his interests went far beyond the realm of Medicine, and he became one of the most illustrious intellectuals of his time. In 1927, he gave a speech at the São Paulo Society of Artistic Culture on Frédéric Chopin and embarked on a journey across the composer's life and times, discussing the medical issues involving his death, as well as his lovers, his compositions, and the spiritual aspects of musical interpretation. Thus, Castro reinforced the bonds of music and Medicine and provided lessons on Musicology that may very well be as suited to a hospital as they are to a concert hall.

"... In the immortal forms of beauty: Frédéric Chopin seen through the eyes of the father of Brazilian neurological semiology / "... nas imortais formas da beleza: Frédéric Chopin visto pelos olhos do pai da semiologia neurológica brasileira

ABSTRACT Aloysio de Castro (1881-1959) is now remembered as one of Brazil's greatest physicians and is considered the father of Brazilian neurological semiology. However, his interests went far beyond the realm of Medicine, and he became one of the most illustrious intellectuals of his time. In 1927, he gave a speech at the São Paulo Society of Artistic Culture on Frédéric Chopin and embarked on a journey across the composer's life and times, discussing the medical issues involving his death, as well as his lovers, his compositions, and the spiritual aspects of musical interpretation. Thus, Castro reinforced the bonds of music and Medicine and provided lessons on Musicology that may very well be as suited to a hospital as they are to a concert hall.

RESUMO Aloysio de Castro (1881-1959) é hoje lembrado como um dos maiores médicos do Brasil, bem como o pai da semiologia neurológica brasileira. Entretanto, seus interesses iam muito além dos domínios da Medicina, e ele se tornou um dos mais ilustres intelectuais de sua era. Em 1927, Castro deu uma palestra na Sociedade de Cultura Artística de São Paulo sobre Frédéric Chopin, e embarcou em uma jornada pela vida e época do compositor, discutindo o aspecto médico de sua morte, bem como suas amantes, composições e aspectos espirituais da interpretação musical. Assim, Castro reforçou os laços entre música e Medicina e ensinou lições de Musicologia que podem muito bem ser tão aplicadas à Medicina quanto a uma sala de concertos.

The Devil Is in the Details: Neurological Diseases Presenting as Religious Hallucinations in Two Literary Works.

Few authors in the Western literature have acquired such a monumental reputation as Thomas Mann and Fyodor Dostoyevsky; although with different backgrounds and aesthetic peculiarities, their writings converge thematically in their frequent relationship with disease. From Dostoyevsky's struggle with epilepsy to Mann's descriptions of tuberculosis and cholera, many are the examples found in their body of work describing medical afflictions. One noteworthy similarity in their works is the presence of hallucinations with Mephistopheles-like devilish entities, possibly caused by neurological diseases: in Mann's case, concerning the main character of Doctor Faustus, caused by neurosyphilis, while for Dostoyevsky, concerning one of the titular Brothers Karamazov, by delirium tremens. In both cases, the authors leave room for ambiguity, with the characters themselves casting doubts on whether their experiences were indeed caused by their disease or by an actual supernatural being. In this, we may find an interesting intersection between neurology and the literature.

Neurology, psychiatry and the chess game: a narrative review.

The chess game comprises different domains of cognitive function, demands great concentration and attention and is present in many cultures as an instrument of literacy, learning and entertainment. Over the years, many effects of the game on the brain have been studied. Seen that, we reviewed the current literature to analyze the influence of chess on cognitive performance, decision-making process, linking to historical neurological and psychiatric disorders as we describe different diseases related to renowned chess players throughout history, discussing the influences of chess on the brain and behavior.

Neurology, psychiatry and the chess game: a narrative review / Neurologia, psiquiatria e o jogo de xadrez: uma revisão narrativa

Abstract The chess game comprises different domains of cognitive function, demands great concentration and attention and is present in many cultures as an instrument of literacy, learning and entertainment. Over the years, many effects of the game on the brain have been studied. Seen that, we reviewed the current literature to analyze the influence of chess on cognitive performance, decision-making process, linking to historical neurological and psychiatric disorders as we describe different diseases related to renowned chess players throughout history, discussing the influences of chess on the brain and behavior.

Resumo O jogo de xadrez compreende diferentes domínios da função cognitiva, exige grande concentração e atenção e está presente em muitas culturas como instrumento de alfabetização, aprendizado e entretenimento. Ao longo dos anos, muitos efeitos do jogo no cérebro foram estudados. Dessa forma, revisamos a literatura atual para analisar a influência do xadrez no desempenho cognitivo, no processo de tomada de decisão, vinculando-a a distúrbios neurológicos e psiquiátricos históricos ao descrevermos diferentes doenças relacionadas a renomados jogadores de xadrez ao longo da história, discutindo as influências do xadrez no cérebro e no comportamento.

Salomón Hakim: the man behind normal pressure hydrocephalus.

The illustrious Colombian Professor Salomón Hakim provided the annals of neurology with one of the most brilliant and original bodies of research on record, developing the concept of normal pressure hydrocephalus, as well as proving that ventricular shunting is an effective treatment. Thus, Professor Hakim proved that some of the dementias, at that time considered senile, could be successfully treated. Here the authors present an historical review of his main contributions, which continue to influence the study of dementia to this day.

Salomón Hakim: the man behind normal pressure hydrocephalus / Salomón Hakim: o homem por trás da hidrocefalia de pressão normal

ABSTRACT The illustrious Colombian Professor Salomón Hakim provided the annals of neurology with one of the most brilliant and original bodies of research on record, developing the concept of normal pressure hydrocephalus, as well as proving that ventricular shunting is an effective treatment. Thus, Professor Hakim proved that some of the dementias, at that time considered senile, could be successfully treated. Here the authors present an historical review of his main contributions, which continue to influence the study of dementia to this day.

RESUMO O ilustre professor colombiano Salomón Hakim deixou como legado nos anais da neurologia uma das mais brilhantes e originais séries de pesquisa da história, desenvolvendo o conceito de hidrocefalia de pressão normal, bem como introduzindo a derivação ventricular como tratamento efetivo. Assim, Hakim provou que algumas das demências até então consideradas senis tinham possibilidade de tratamento bem-sucedido. Aqui os autores apresentarão uma revisão histórica de suas maiores contribuições, que continuam a influenciar o estudo de demências até os nossos dias.

The Legacy of Charles David Marsden: A Role Model in the Field of Movement Disorders.

David Marsden was one of the most renowned neuroscientists of the twentieth century. His scientific contributions in the specialty of movement disorders are recognized worldwide, particularly in the area of Parkinson's disease and also in hyperkinesias, such as dystonia and myoclonus.

Burnout syndrome: are stroke neurologists at a higher risk?

BACKGROUND: Burnout syndrome is a work-related psychological response, characterized by emotional exhaustion, depersonalization and low professional accomplishment. OBJECTIVE: The study aimed to evaluate the prevalence of burnout syndrome in neurologists in the State of Paraná, Brazil, dividing them into stroke neurologists and non-stroke neurologists. METHODS: We performed a crosssectional observational study, with a quantitative approach, based on the online Maslach Burnout Inventory - Human Services Survey questionnaire. RESULTS: A total of 74 neurologists were evaluated, 44.6% of whom had burnout syndrome, predominantly among females and stroke neurologists. Both the stroke neurologist and non-stroke neurologist groups had medium degrees of emotional exhaustion and depersonalization; however, while stroke neurologists had high professional accomplishment, non-stroke neurologists had mean-to-low scores of professional accomplishment. There was a proportional relationship between age and emotional exhaustion. Female neurologists also reported lower professional accomplishment levels. CONCLUSION: Burnout is prevalent among the neurologists of Paraná, corroborating the results previously reported in other studies. There seems to be no significant difference between those neurologists who work in the emergency stroke care setting compared with those who don't.

Burnout syndrome: are stroke neurologists at a higher risk? / Síndrome de Burnout: neurologistas vasculares apresentam maior risco?

ABSTRACT Background: Burnout syndrome is a work-related psychological response, characterized by emotional exhaustion, depersonalization and low professional accomplishment. Objective: The study aimed to evaluate the prevalence of burnout syndrome in neurologists in the State of Paraná, Brazil, dividing them into stroke neurologists and non-stroke neurologists. Methods: We performed a crosssectional observational study, with a quantitative approach, based on the online Maslach Burnout Inventory - Human Services Survey questionnaire. Results: A total of 74 neurologists were evaluated, 44.6% of whom had burnout syndrome, predominantly among females and stroke neurologists. Both the stroke neurologist and non-stroke neurologist groups had medium degrees of emotional exhaustion and depersonalization; however, while stroke neurologists had high professional accomplishment, non-stroke neurologists had mean-to-low scores of professional accomplishment. There was a proportional relationship between age and emotional exhaustion. Female neurologists also reported lower professional accomplishment levels. Conclusion: Burnout is prevalent among the neurologists of Paraná, corroborating the results previously reported in other studies. There seems to be no significant difference between those neurologists who work in the emergency stroke care setting compared with those who don't.

RESUMO Introdução: A síndrome de burnout é uma resposta psicológica relacionada à profissão, caracterizada por exaustão emocional, despersonalização e redução da realização pessoal. Objetivos: Objetivamos avaliar a prevalência da síndrome de burnout em neurologistas do Paraná, distribuindo a atuação entre neurologistas vasculares e não-vasculares. Métodos: Estudo observacional, transversal, com abordagem quantitativa, realizado a partir do questionário online autoaplicável Maslach Burnout Inventory - Human Services Survey. Resultados: 74 neurologistas foram avaliados, desses 44.6% apresentaram critérios para síndrome de burnout, predominando entre mulheres e stroke neurologists. Ambos os grupos apresentaram médios graus de exaustão emocional e despersonalização. Enquanto os neurologistas vasculares apresentaram alto grau de realização pessoal, os não-vasculares apresentaram médio grau. Houve relação proporcional entre idade e exaustão emocional. Neurologistas do gênero feminino mostraram menores níveis de realização pessoal. Conclusão: A síndrome de burnout é frequente nos neurologistas do Paraná, compatível com a literatura que sugere a especialidade como uma das mais susceptíveis. Não obtivemos contudo, diferenças entre os neurologistas que lidam ou não com a emergência vascular.

Abnormal Findings in Polysomnographic Recordings of Patients with Spinocerebellar Ataxia Type 2 (SCA2).

Spinocerebellar ataxia type 2 (SCA2) is characterized by a progressive cerebellar syndrome, and additionally saccadic slowing, cognitive dysfunction, and sleep disorders. The aim of this study was to assess the frequency of abnormal findings in sleep recordings of patients with SCA2. Seventeen patients with genetically confirmed SCA2 from the Movement Disorders Outpatient group of the Hospital de Clínicas da UFPR were evaluated with a structured medical interview and the Scale for the Assessment and Rating of Ataxia (SARA). Polysomnographic recordings were performed and sleep stages were scored according to standard criteria. There were 10 male subjects and 7 females, aged 24-66 years (mean 47.44). A sex- and age-matched control group of healthy subjects was used for comparison. There was a reduction of rapid eye movement (REM) sleep in 12 (70.58%), increased REM latency in 9 (52.94%), increased obstructive sleep apnea-index in 14 (82.35%), absent REM density (REM density was calculated as the total number of 3-s miniepochs of REM sleep with at least 1 REM per minute) in 13 (76.47%), and markedly reduced REM density in 4 (23.52%). There was an indirect correlation according to the SARA scale and the REM density decrease (r = - 0.6; P = < 0.001); and with a disease progression correlating with a reduction in the REM density (r = - 0.52, P = 0.03). In SCA2, changes occur mainly REM sleep. The absence/decrease of REM sleep density, even in oligosymptomatic patients, and the correlation of this finding with disease time and with the SARA scale were the main findings of the study.

Neurosyphilis and classical music: the great composers and "The Great Imitator".

OBJECTIVE: Throughout history, neurosyphilis has victimized many people, including classical composers, with a wide range of clinical presentations. Six articles with descriptions of composers with possible neurosyphilis were reviewed. METHODS: Neurosyphilis is a possible diagnosis for composers like Beethoven, whose progressive hearing loss influenced his career, culminating in complete deafness. In his autopsy, cochlear nerve atrophy and cochlear inflammation were described. Donizetti developed behavioral changes, as well as headaches, general paresis and seizures. RESULTS: Both Schumann and Wolf suffered from personality changes, persecutory delusions and general paresis. Joplin and Delius also had symptoms attributed to syphilis. Autopsy findings confirmed the diagnosis of Smetana, who developed dementia, deafness and auditory hallucinations with rapid progression. His tinnitus was musically represented in his first String Quartet. CONCLUSION: Neurosyphilis victimized several notorious composers. It can be argued that neurosyphilis was a major source of inspiration as well, being responsible for the genesis of musical masterpieces.

Neurosyphilis and classical music: the great composers and "The Great Imitator" / Neurossífilis e música clássica: os grandes compositores e "A Grande Imitadora"

ABSTRACT Throughout history, neurosyphilis has victimized many people, including classical composers, with a wide range of clinical presentations. Methods: Six articles with descriptions of composers with possible neurosyphilis were reviewed. Results: Neurosyphilis is a possible diagnosis for composers like Beethoven, whose progressive hearing loss influenced his career, culminating in complete deafness. In his autopsy, cochlear nerve atrophy and cochlear inflammation were described. Donizetti developed behavioral changes, as well as headaches, general paresis and seizures. Both Schumann and Wolf suffered from personality changes, persecutory delusions and general paresis. Joplin and Delius also had symptoms attributed to syphilis. Autopsy findings confirmed the diagnosis of Smetana, who developed dementia, deafness and auditory hallucinations with rapid progression. His tinnitus was musically represented in his first String Quartet. Conclusion: Neurosyphilis victimized several notorious composers. It can be argued that neurosyphilis was a major source of inspiration as well, being responsible for the genesis of musical masterpieces.

RESUMO Através da história, a neurossífilis vitimou milhares de pessoas, incluindo compositores clássicos, com uma grande gama de manifestações. Métodos: Seis artigos com descrições de compositores com possível neurossífilis foram revisados. Resultados: Neurossífilis é um diagnóstico possível para compositores como Beethoven, cuja perda auditiva progressiva influenciou sua carreira, culminando com surdez completa. Em sua autópsia foram descritas inflamação e atrofia dos nervos cocleares. Donizetti desenvolveu alterações comportamentais, bem como cefaleias, paresia e convulsões. Tanto Schumann quanto Wolf sofreram com alterações comportamentais, delírios persecutórios e paresia. Joplin e Delius também tiveram sintomas relacionados a sífilis. Achados de autópsia confirmaram o diagnóstico de Smetana, que desenvolveu demência, surdez e alucinações auditivas rapidamente progressivas. Seu tinito foi musicalmente representados em seu Quarteto de Cordas No. 1. Conclusão: Neurossífilis vitimou diversos compositores de destaque. Pode-se argumentar que a doença chegou a ser grande fonte de inspiração e mesmo responsável pela gênese de diversas obras-primas.