ABSTRACT
Abstract: Inflammasomes are intracellular multiprotein complexes that comprise part of the innate immune response. Since their definition, inflammasome disorders have been linked to an increasing number of diseases. Autoinflammatory diseases refer to disorders in which local factors lead to the activation of innate immune cells, causing tissue damage when in the absence of autoantigens and autoantibodies. Skin symptoms include the main features of monogenic inflammasomopathies, such as Cryopyrin-Associated Periodic Syndromes (CAPS), Familial Mediterranean Fever (FMF), Schnitzler Syndrome, Hyper-IgD Syndrome (HIDS), PAPA Syndrome, and Deficiency of IL-1 Receptor Antagonist (DIRA). Concepts from other pathologies have also been reviewed in recent years, such as psoriasis, after the recognition of a combined contribution of innate and adaptive immunity in its pathogenesis. Inflammasomes are also involved in the response to various infections, malignancies, such as melanoma, autoimmune diseases, including vitiligo and lupus erythematosus, atopic and contact dermatitis, acne, hidradenitis suppurativa, among others. Inhibition of the inflammasome pathway may be a target for future therapies, as already occurs in the handling of CAPS, through the introduction of IL-1 inhibitors. This study presents a literature review focusing on the participation of inflammasomes in skin diseases.
Subject(s)
Humans , Skin Diseases/immunology , Hereditary Autoinflammatory Diseases/immunology , Inflammasomes/immunology , Immunity, Innate/immunology , Skin Diseases/pathology , Interleukin-1beta/immunologyABSTRACT
Os transtornos mentais na infância são prevalentes e causam prejuízo para o indivíduo, família e sociedade. Informações acerca do custo dos transtornos mentais na infância são úteis para o planejamento do sistema de saúde, para auxiliar tomadas de decisão de gestores acerca de investimentos na área e para determinação de prioridades no orçamento público. No entanto, não há dados sobre o custo dos transtornos mentais na infância no Brasil. Já dados sobre o custo dos transtornos mentais na infância em nível subclínico são inexistentes na literatura nacional e internacional. Os objetivos centrais da presente tese de doutorado, dividida em Estudo I e Estudo 11, foram: estimar a média do custo do transtorno mental na infância em níveis subclínico e clínico, e estimar o custo total destes para o Brasil. O Estudo I teve como desfecho clínico qualquer diagnóstico de transtorno mental na infância; o Estudo 11, o transtorno obsessivo- compulsivo na infância (TOC), ambos em nível subclínico e clínico. A presente pesquisa é uma avaliação econômica de custo de doença de transtornos mentais na infância com utilização de método bottom-up retrospectivo. Foi baseada em dados de prevalência de um estudo populacional transversal de 2.512 estudantes de escolas públicas de Porto Alegre e São Paulo, selecionados por meio de duas técnicas: a) seleção aleatória de crianças e b) seleção de crianças com alto fiSCO para desenvolvimento de transtornos mentais (baseado no histórico psiquiátrico familiar). Para avaliação de transtornos mentais na infância, foi utilizado o instrumento Development and Well Being Assesment (DAWBA). Os custos de doença foram estimados a partir dos seguintes componentes: tratamentos em saúde mental (uso de medicamentos, psicoterapia, hospitalização), uso de serviços sociais (assistência social, conselho tutelar, medidas socioeducativas) e problemas escolares (suspensão, abandono e repetência escolar). A amostra final foi de 2.512 crianças, de 6-14 anos de...
Child mental disorders are prevalent and impairing, negatively impacting families and society. lnformation on child mental disorders costs is important to plan the health system and to show policy makers how plan and prioritize budgets. However, there are no child mental disorders cost studies in Brazil. The main objectives of the present thesis were to estimate the mean costs of subthreshold and clinical mental disorders in children living in Brazil and to estimate its national costs. Outcome of Study I was any child mental disorder, outcome of Study II was child obsessive-compulsive disorder (OCD), both subthreshold and clinical. The present study it is cost-of-illness study of child mental disorders using a retrospective bottom-up methods, based on prevalence data from a cross-sectional study of children registered at public schools in Porto Alegre and Sao Paulo. A total of 8,012 families were interviewed, providing information about 9,937 children. From this pool, two subgroups were further investigated using random- selection (n=958) and high-risk group selection procedure (n=I,514), resulting in a total sample of 2,512 subjects 6-14 years old. Mental disorder assessment was made using the Development and Well-Being Assessment (DAWBA). The cost of child mental disorders was estimated from the following components: use of mental health services, social services and school problems. Costs were estimated for each child and the economic impact at the national levei was calculated. Study I results: subthreshold and clinical disorder showed lifetime mean total cost of $1,750.86 and $3,141.21, respectively. The national lifetime cost estimate of clinical mental disorders in Brazil was $11.65 billion, whereas for subthreshold mental disorder it was $19.92 billion (alI values in PPP, purchasing power parity). Study 11: subthreshold and clínical OCD showed lifetime mean total cost of $1,651.81 and $3,293.38, respectively. The national lifetime cost estimate of...
Subject(s)
Humans , Costs and Cost Analysis , Cross-Sectional Studies , Epidemiology , Obsessive-Compulsive DisorderABSTRACT
Estudo de corte transversal conduzido em comunidade urbana de baixa renda do município de Embu-SP com objetivo de identificar fatores associados a problemas de saúde mental em crianças/adolescentes (PSMCA) em amostra probabilística (N=67, faixa etária 4-17 anos). Foram aplicados instrumentos estruturados às mães: Child Behavior Checklist (PSMCA); WorldSAFE Core Questionnaire (dados sociodemográficos; violência doméstica; embriaguez do pai/padrasto); Self-Report Questionnaire (problemas de saúde mental maternos, ideação suicida materna). Os resultados do estudo apontaram fatores estatisticamente associados aos PSMCA: criança/adolescente ser do sexo masculino e sofrer punição física grave; ideação suicida da mãe e violência conjugal física grave contra a mãe; embriaguez do pai/padrasto. Concluindo, grupos vulneráveis com características individuais/familiares identificadas neste estudo devem ser considerados prioritários em propostas de prevenção/tratamento.
Across-sectional study was conducted in an urban low-income community in the municipality of Embu-SP with the objective of identifying factors associated with mental health problems of children and adolescents (MHPCA) in a probabilistic sample (N=67, aged 4-17 years). Standardized instruments were applied to mothers: Child Behavior Checklist; WorldSAFE Core Questionnaire (demographics, domestic violence, father/stepfather drunkenness); Self-Report Questionnaire (maternal mental health problems and suicide ideation). The results of this study identified factors statistically related to MPHCA: child/adolescent of male gender and severe physical punishment; maternal suicide ideation and severe physical marital violence; father/stepfather drunkenness. As a result, vulnerable groups with individual/family characteristics identified in this study should have priority in prevention/treatment programs.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Adult , Mental Health , Child Abuse/psychology , Domestic Violence/prevention & control , Domestic Violence/psychology , Adolescent Behavior/psychology , Psychopathology , Child Behavior Disorders/psychologyABSTRACT
Estudo de corte transversal conduzido em comunidade urbana de baixa renda do município de Embu-SP com objetivo de identificar fatores associados a problemas de saúde mental em crianças/adolescentes (PSMCA) em amostra probabilística (N=67, faixa etária 4-17 anos). Foram aplicados instrumentos estruturados às mães: Child Behavior Checklist (PSMCA); WorldSAFE Core Questionnaire (dados sociodemográficos; violência doméstica; embriaguez do pai/padrasto); Self-Report Questionnaire (problemas de saúde mental maternos, ideação suicida materna). Os resultados do estudo apontaram fatores estatisticamente associados aos PSMCA: criança/adolescente ser do sexo masculino e sofrer punição física grave; ideação suicida da mãe e violência conjugal física grave contra a mãe; embriaguez do pai/padrasto. Concluindo, grupos vulneráveis com características individuais/familiares identificadas neste estudo devem ser considerados prioritários em propostas de prevenção/tratamento.(AU)
Across-sectional study was conducted in an urban low-income community in the municipality of Embu-SP with the objective of identifying factors associated with mental health problems of children and adolescents (MHPCA) in a probabilistic sample (N=67, aged 4-17 years). Standardized instruments were applied to mothers: Child Behavior Checklist; WorldSAFE Core Questionnaire (demographics, domestic violence, father/stepfather drunkenness); Self-Report Questionnaire (maternal mental health problems and suicide ideation). The results of this study identified factors statistically related to MPHCA: child/adolescent of male gender and severe physical punishment; maternal suicide ideation and severe physical marital violence; father/stepfather drunkenness. As a result, vulnerable groups with individual/family characteristics identified in this study should have priority in prevention/treatment programs.(AU)
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Adult , Mental Health , Domestic Violence/prevention & control , Child Abuse/psychology , Domestic Violence/psychology , Psychopathology , Child Behavior Disorders/psychology , Adolescent Behavior/psychologyABSTRACT
A exposição à violência é um conhecido fator de risco para o comportamento antissocial. O objetivo do estudo foi investigar a associação entre exposição à violência e o desenvolvimento de dois perfis psicopatológicos de comportamento antissocial em adolescentes da periferia de São Paulo. Trata-se de um estudo longitudinal cuja amostra foi composta por 93 adolescentes (11-18 anos) que em 2002 apresentavam problemas de saúde mental e que foram reavaliados em 2007. O desfecho clínico investigado é o comportamento antissocial em nível clínico/limítrofe na escala de externalização do Child Behavior Checklist. Os fatores de risco foram a exposição à violência urbana e doméstica, segundo o questionário de eventos traumáticos. A violência doméstica mostrou-se como único fator relevante para a manutenção do comportamento antissocial, enquanto dois fatores relativos à violência urbana (ter amigo que foi estuprado e/ou ferido com faca) mostraram-se associados ao surgimento do comportamento antissocial. Concluiu-se que o ambiente familiar e uma comunidade violenta parecem ser modelos para a manutenção e o desenvolvimento de comportamentos violentos entre adolescentes.
The exposure to violence is a known risk factor for antisocial behavior. The objective of the study was to investigate the association between exposure to violence and development of two psychopathological profiles of antisocial behavior in adolescents from the outskirts of Sao Paulo. The design of the investigation was a cohort study with a sample composed by 93 adolescents (11-18 years) diagnosed with mental health problems at baseline in 2002 were reassessed in 2007. The investigated clinical outcome was the antisocial behavior at clinical or borderline level scores on the externalizing problem scale of the Child Behavior-Checklist. The risk factors were the exposure to urban and intrafamilial violence, according to the Questionnaire of Traumatic Events. The intrafamilial violence was the only relevant factor which predicted continuation of antisocial behavior at follow-up, while two factors related to urban violence (having a friend who was raped and/or injured with a knife) were associated with the onset of antisocial behavior. It was concluded that the family environment and a violent community are important influences to consider in the continuation and development of violent behavior among adolescents.
La exposição a la violencia es un factor de riesgo conocido para el comportamiento anti-social. El objetivo del estudio foi investigar la asociación entre exposición a la violencia y el desarrollo de dos perfiles psicopatológicos de comportamiento anti-social en adolescentes de la periferia de São Paulo. El diseño de la investigación fue un estudio longitudinal cuya muestra fué formada por 93 adolescentes (11-18 años) que en 2002 presentaban problemas de salud mental y fueron reavaliados en 2007. Los resultados mostraron comportamiento anti-social en nivel clínico/limítrofe en la escala de externalización del Child Behavior Checklist. Los factores de riesgo fueron la exposición a la violencia urbana y doméstica, según el cuestionario de eventos traumáticos. La violencia doméstica se mostró como único factor relevante para el mantenimiento del comportamiento anti-social, mientras que dos factores relativos a la violencia urbana (tener amigo que fué violado y/o herido con arma blanca) se mostraron asociados al surgimiento del comportamiento anti-social. Se concluye que el ambiente familiar y La violencia en la comunidad parecen ser modelos para el mantenimiento y desarrollo de comportamientos violentos entre adolescentes.
Subject(s)
Humans , Male , Female , Adolescent , Adolescent Behavior , Antisocial Personality Disorder , Violence , Parent-Child RelationsABSTRACT
A exposição à violência é um conhecido fator de risco para o comportamento antissocial. O objetivo do estudo foi investigar a associação entre exposição à violência e o desenvolvimento de dois perfis psicopatológicos de comportamento antissocial em adolescentes da periferia de São Paulo. Trata-se de um estudo longitudinal cuja amostra foi composta por 93 adolescentes (11-18 anos) que em 2002 apresentavam problemas de saúde mental e que foram reavaliados em 2007. O desfecho clínico investigado é o comportamento antissocial em nível clínico/limítrofe na escala de externalização do Child Behavior Checklist. Os fatores de risco foram a exposição à violência urbana e doméstica, segundo o questionário de eventos traumáticos. A violência doméstica mostrou-se como único fator relevante para a manutenção do comportamento antissocial, enquanto dois fatores relativos à violência urbana (ter amigo que foi estuprado e/ou ferido com faca) mostraram-se associados ao surgimento do comportamento antissocial. Concluiu-se que o ambiente familiar e uma comunidade violenta parecem ser modelos para a manutenção e o desenvolvimento de comportamentos violentos entre adolescentes.(AU)
The exposure to violence is a known risk factor for antisocial behavior. The objective of the study was to investigate the association between exposure to violence and development of two psychopathological profiles of antisocial behavior in adolescents from the outskirts of Sao Paulo. The design of the investigation was a cohort study with a sample composed by 93 adolescents (11-18 years) diagnosed with mental health problems at baseline in 2002 were reassessed in 2007. The investigated clinical outcome was the antisocial behavior at clinical or borderline level scores on the externalizing problem scale of the Child Behavior-Checklist. The risk factors were the exposure to urban and intrafamilial violence, according to the Questionnaire of Traumatic Events. The intrafamilial violence was the only relevant factor which predicted continuation of antisocial behavior at follow-up, while two factors related to urban violence (having a friend who was raped and/or injured with a knife) were associated with the onset of antisocial behavior. It was concluded that the family environment and a violent community are important influences to consider in the continuation and development of violent behavior among adolescents.(AU)
La exposição a la violencia es un factor de riesgo conocido para el comportamiento anti-social. El objetivo del estudio foi investigar la asociación entre exposición a la violencia y el desarrollo de dos perfiles psicopatológicos de comportamiento anti-social en adolescentes de la periferia de São Paulo. El diseño de la investigación fue un estudio longitudinal cuya muestra fué formada por 93 adolescentes (11-18 años) que en 2002 presentaban problemas de salud mental y fueron reavaliados en 2007. Los resultados mostraron comportamiento anti-social en nivel clínico/limítrofe en la escala de externalización del Child Behavior Checklist. Los factores de riesgo fueron la exposición a la violencia urbana y doméstica, según el cuestionario de eventos traumáticos. La violencia doméstica se mostró como único factor relevante para el mantenimiento del comportamiento anti-social, mientras que dos factores relativos a la violencia urbana (tener amigo que fué violado y/o herido con arma blanca) se mostraron asociados al surgimiento del comportamiento anti-social. Se concluye que el ambiente familiar y La violencia en la comunidad parecen ser modelos para el mantenimiento y desarrollo de comportamientos violentos entre adolescentes.(AU)
Subject(s)
Humans , Male , Female , Adolescent , Antisocial Personality Disorder , Adolescent Behavior , Violence , Parent-Child RelationsABSTRACT
OBJETIVO: Analisar a prevalência, gravidade e interferência funcional de transtornos do movimento (TM) secundários ao uso crônico de ciclosporina A (CsA). MÉTODO: Realizamos um estudo transversal em 60 pacientes (58.3% do sexo masculino) com idade média de 23.1 (3-75) anos, acompanhados pelo Serviço de Transplante de Medula Óssea do Hospital de Clínicas da Universidade Federal do Paraná, usando CsA por pelo menos seis meses. A avaliação incluiu dados clínicos, interferência funcional de possíveis sintomas e exame neurológico incluindo observação e graduação de TM. RESULTADOS: Oito (13.3%) entrevistados relataram tremor no momento da entrevista e 29 (48.3%) em alguma fase do tratamento. O exame neurológico identificou 14 (23.3%) pacientes com TM: 13 (21.6%) tremor de ação simétrico de membros superiores e em 1 (1.7%) parkinsonismo (rigidez e bradicinesia). Nenhum outro TM foi detectado. Os escores médios indicaram quadros leves em todos os casos. Os sintomas foram também considerados subjetivamente leves e sem interferência funcional. CONCLUSÃO: Quase um quarto dos pacientes usando CsA cronicamente apresenta TM, quase sempre tremor de ação leve e transitório, interferindo pouco funcionalmente, não requerendo intervenção na maioria dos casos.
Subject(s)
Adult , Aged , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Bone Marrow Transplantation , Cyclosporine/adverse effects , Dyskinesia, Drug-Induced/epidemiology , Immunosuppressive Agents/adverse effects , Cross-Sectional Studies , Cyclosporine/therapeutic use , Dyskinesia, Drug-Induced/diagnosis , Graft Rejection/prevention & control , Interviews as Topic , Immunosuppressive Agents/therapeutic use , Long-Term Care , Neurologic Examination , Prevalence , Severity of Illness IndexABSTRACT
We describe a female patient with stable Parkinson's disease who has shown a marked worsening of her motor functions following therapy of menopause related symptoms with veralipride, as well as the improvement of her symptoms back to baseline after discontinuation of the drug. We emphasize the anti-dopaminergic effect of veralipride
Subject(s)
Humans , Female , Middle Aged , Dopamine Antagonists/adverse effects , Menopause/drug effects , Parkinson Disease/physiopathology , Sulpiride/adverse effectsABSTRACT
We describe a patient whose initial manifestation of Parkinson's disease was a malfunctioning of a self-winding wristwatch secondary to bradykinesia of his left arm. Andrade and Ferraz reported this sign in 1996, suggesting that it be called the Rolex sign
Subject(s)
Humans , Male , Middle Aged , Parkinson Disease/diagnosis , Hypokinesia/etiology , Parkinson Disease/complicationsABSTRACT
Relatamos dois casos de doença de Lafora que apresentaram distúrbios do movimento, ataxia cerebelar, disartria e fenômeno do "susto exagerado", como manifestaçoes clínicas iniciais. Estes sintomas precederam as convulsoes, mioclonias e a demência progressiva. O diagnóstico foi confirmado pela identificaçao de corpos de inclusao, PAS positivo, na biópsia de pele de ambos os casos. Os pacientes relatados apresentam uma progressao lenta da doença, o que é incomum, com longa sobrevida. A doença de Lafora deve sempre ser incluída entre as causas de ataxia lentamente progressiva associada com epilepsia
Subject(s)
Humans , Male , Female , Adult , Lafora Disease/pathology , Biopsy , Inclusion Bodies/pathology , Lafora Disease/diagnosis , Lafora Disease/drug therapy , Lafora Disease/genetics , Movement Disorders/diagnosisABSTRACT
OBJECTIVE: To describe the relationship between Professor Charcot and Brazil. BACKGROUND: During the XIX century, French Neurology and its most prominent figure, Professor Charcot, dominated the area of nervous system diseases in the world. METHOD: We have reviewed some of the main publications about Charcot's life, the biography of Dom Pedro II, Emperor of Brazil and the development of Neurology in Brazil. RESULTS: Among the most important patients in Charcot's practice was the Emperor of Brazil. Dom Pedro II became a close friend of Charcot and he was a distinguished guest at Charcot's house, particularly at Tuesday soirées on boulevard St. Germain. In 1887, during the visit of Dom Pedro II to France, Charcot evaluated him and made the diagnosis of surmenage. In 1889, Dom Pedro II was deposed and went to Paris, where he lived until his death in 1891. Charcot signed the death certificate and gave the diagnosis of pneumonitis. Charcot had a passionate affection for animals, a feeling shared by Dom Pedro II. Dom Pedro II was affiliated to the French Society for the Protection of Animals. It is conceivable that Charcot's little monkey, from South America, was given to him by Dom Pedro II. The Brazilian Neurological School was founded by Professor A . Austregésilo in 1911, in Rio de Janeiro. At the time, of Charcot's death in 1893, his influence was still very important in the whole world. He and his pupils played a major role in the development of Brazilian Neurology. CONCLUSION: Professor Charcot had a close relationship with the Emperor of Brazil, Dom Pedro II. He was his private physician and they were close friends. The neurological school, created by professor Charcot, contributed significantly, albeit in an indirect way, to the development of Brazilian Neurology, starting in 1911, in Rio de Janeiro, by Professor A . Austregésilo
Subject(s)
History, 19th Century , History, 20th Century , Humans , Neurology/history , Brazil , France , PortraitABSTRACT
OBJECTIVE: To evaluate the efficacy and safety of bilateral pallidotomies in five patients with generalized dystonia. BACKGROUND: Generalized dystonias are frequently a therapeutic challenge, with poor responses to pharmacological treatment. GPi (globus pallidus internus) pallidotomies for Parkinson's disease ameliorate all kinds of dyskinesias/dystonia, and recent studies reported a marked improvement of refractory dystonias with this procedure. METHODS: Five patients with generalized dystonias refractory to medical treatment were selected; one posttraumatic and four idiopathic. The decision to perform bilateral procedures was based on the predominant axial involvement in these patients. Dystonia severity was assessed with the Burke-Fahn-Marsden Dystonia Scale (BFM). Simultaneous procedures were performed in all but one patient, who had a staged procedure. They were reevaluated with the same scale (BFM) by an unblinded rater at 1, 2, 3, 30, 60, 90, 120 and 180 days post-operatively. RESULTS: The four patients with idiopathic dystonia showed a progressive improvement up to three months; the patient with posttraumatic dystonia relapsed at three months. One patient had a marked improvement, being able to discontinue all the medications. A mean decrease in the BFM scores of 52,58 percent was noted. One patient had a trans-operative motor seizure followed by a transient hemiparesis secondary to rack hemorrhage; other was lethargic up to three days after the procedure. CONCLUSIONS: Our results show that bilateral GPi pallidotomies may be a safe and effective approach to medically refractory generalized dystonias; it can also be speculated that the posttraumatic subgroup may not benefit with this procedure
Subject(s)
Humans , Male , Child, Preschool , Adolescent , Adult , Dystonia/surgery , Globus Pallidus/surgery , Stereotaxic Techniques , Severity of Illness Index , Treatment OutcomeABSTRACT
O Professor Antonio Austregésilo foi o pioneiro da neurologia brasileira, criando a primeira escola neurológica no Rio de Janeiro em 1912. Ele foi também o primeiro a estudar os distúrbios do movimento no Brasil, tendo publicado vários artigos nesta área, particularmente nas consagradas revistas "Revue Neurologique" e "L'Encephale", incluindo a descrição de um sinal sucedâneo de Babinski e a primeira descrição mundial de distonia pós-traumática.
Subject(s)
Humans , Movement Disorders/history , Neurology/history , Brazil , PortraitABSTRACT
OBJECTIVE: To describe the relationship between Professor Charcot and Brazil. BACKGROUND: During the XIX century, French Neurology and its most prominent figure, Professor Charcot, dominated the area of nervous system diseases in the world. METHOD: We have reviewed some of the main publications about Charcot's life, the biography of Dom Pedro II, Emperor of Brazil and the development of Neurology in Brazil. RESULTS: Among the most important patients in Charcot's practice was the Emperor of Brazil. Dom Pedro II became a close friend of Charcot and he was a distinguished guest at Charcot's house, particularly at Tuesday soirées on boulevard St. Germain. In 1887, during the visit of Dom Pedro II to France, Charcot evaluated him and made the diagnosis of surmenage. In 1889, Dom Pedro II was deposed and went to Paris, where he lived until his death in 1891. Charcot signed the death certificate and gave the diagnosis of pneumonitis. Charcot had a passionate affection for animals, a feeling shared by Dom Pedro II. Dom Pedro II was affiliated to the French Society for the Protection of Animals. It is conceivable that Charcot's little monkey, from South America, was given to him by Dom Pedro II. The Brazilian Neurological School was founded by Professor A . Austregésilo in 1911, in Rio de Janeiro. At the time, of Charcot's death in 1893, his influence was still very important in the whole world. He and his pupils played a major role in the development of Brazilian Neurology. CONCLUSION: Professor Charcot had a close relationship with the Emperor of Brazil, Dom Pedro II. He was his private physician and they were close friends. The neurological school, created by professor Charcot, contributed significantly, albeit in an indirect way, to the development of Brazilian Neurology, starting in 1911, in Rio de Janeiro, by Professor A . Austregésilo (AU)
Subject(s)
History, 19th Century , History, 20th Century , Neurology/history , France , BrazilABSTRACT
We describe two cases of palatal myoclonus (PM), one essential and another secondary to a stroke. Case 1: a 64 years old female who developed clicking sounds in both ears after a stroke and three years later on noticed a progressive involuntary movement of the throat associated with rhythmic contractions of the soft palate, muscles of tongue and throat. MRI showed an ischemic area in brainstem. The patient had a partial response to the use of sumatriptan 6 mg subcutaneously. Case 2: a 66 years old female who began with ear clicking at left ear that worsed slowly associated with tinnitus and arrhythmic movements of soft palate and an audible click at left ear. Brain MRI was normal; audiometry showed bilateral neurosensory loss. She was prescribed clonazepan 1 mg daily with complete recovery. Primary and secondary palatal myoclonus share the same clinical features but probably have different pathophysiological underlying mechanisms
Subject(s)
Humans , Female , Middle Aged , Anticonvulsants/therapeutic use , Clonazepam/pharmacology , Myoclonus/drug therapy , Serotonin Receptor Agonists/pharmacology , Sumatriptan/pharmacology , Electromyography , Magnetic Resonance Imaging , Myoclonus/diagnosis , Palate, Soft/drug effectsABSTRACT
We describe a female patient with hemimasticatory spasm, a rare movement disorder due to dysfunction of the motor trigeminal nerve of unknown origin. This patient had an excellent response to botulinum toxin therapy
Subject(s)
Humans , Female , Adult , Anti-Dyskinesia Agents , Botulinum Toxins , Hemifacial Spasm , Trigeminal NerveABSTRACT
Charcot contribuiu significativamente no século XIX na descrição de várias enfermidades neurológicas, em particular na área dos distúrbios do movimento. Charcot contribuiu de forma exponencial na descrição clínica minuciosa da doença de Parkinson, além de introduzir o primeiro tratamento farmacológico. Na área das hipercinesias realizou estudos sobre a síndrome de Tourette, o diagnóstico diferencial dos tremores, das coréias e o estudo inicial sobre startle. Marsden, recentemente falecido, destacou-se no século XX com inúmeras publicações na área dos movimentos anormais.São contribuições seminais os estudos sobre a doença de Parkinson, distonias, mioclonias , tremor essencial, a descrição das síndromes "Painful Legs Moving Toes", "Gait Ignition Failure" e o "Tremor Primário da Escrita". As contribuições de Charcot no século XIX e de Marsden no século XX na área dos distúrbios do movimento permitem concluir que ambos foram as figuras mais representativas desta área nos últimos dois séculos.