Stiff-Person syndrome: A rare neurological disorder

The Stiff-person syndrome (SPS) is an uncommon disorder characterized by progressive rigidity, muscle stiffness, and spasm involving the axial muscles, resulting in severe impairment of ambulation. We present the case of a 49-year-old gentleman with recent onset of progressive asymmetric spastic ataxia, subsequently diagnosed with SPS.

Evaluación funcional de pacientes con osteoartritis que asistieron al Centro de Rehabilitación Integral Especializado Guayaquil / Functional evaluation of patients with osteoarthritis who attended the Guayaquil Specialized Comprehensive Rehabilitation Center / Avaliação funcional de pacientes com osteoartrite atendidos no Centro Especializado de Reabilitação Integral de Guayaquil

La osteoartritis pertenece al grupo de afecciones articulares degenerativa más común y una de las principales causas de discapacidad en el mundo, caracterizada por provocar dolor articular, rigidez, y pérdida de la capacidad funcional. Objetivo. Evaluar la funcionalidad de los pacientes con osteoartritis que asistieron al Centro de Rehabilitación Integral Especializado Guayaquil # 2. Materiales y Métodos. Se realizó un estudio bajo un enfoque cuantitativo, con un alcance descriptivo y de corte transversal, se desarrolló mediante la medición numérica y análisis de datos de las variables en estudio como edad; sexo; patología; ocupación laboral; rigidez y capacidad funcional, mediante opciones de respuestas. La recolección de datos se ejecutó por medio de la técnica de la observación, para el análisis de los resultados obtenidos del Cuestionario Western Ontario and McMasters Universities Osteoarthritis Índex. Resultados. Se determinó que de acuerdo al sexo prevaleció el femenino en un 90%, la mayoría entre 60 y 70 años de edad; el 70% del grupo estudiado fueron las amas de casa; patología frecuente la poliosteoartritis en un 85%; presentaron un 55% muchísimo dolor y el 45% mucho dolor; la rigidez en un 45%; mientras un 10% no disminución de la capacidad funcional en las articulaciones afectadas. Conclusiones. La osteoartritis afectó la condición funcional a la mayoría de las mujeres y se evidenció con el análisis de la evaluación del resultado obtenido, mediante el Cuestionario Womac.

Osteoarthritis belongs to the most common group of degenerative joint conditions and is one of the leading causes of disability in the world, characterized by joint pain, stiffness, and loss of functional capacity. Objective. To evaluate the functionality of patients with osteoarthritis who attended the Centro de Rehabilitación Integral Especializado Guayaquil # 2. Materials and Methods. A study was carried out under a quantitative approach, with a descriptive and cross-sectional scope, developed through numerical measurement and data analysis of the variables under study such as age; sex; pathology; work occupation; stiffness and functional capacity, by means of response options. Data collection was carried out by means of the observation technique, for the analysis of the results obtained from the Western Ontario and McMasters Universities Osteoarthritis Index Questionnaire. Results. It was determined that according to sex, 90% were female, most of them between 60 and 70 years of age; 70% of the group studied were housewives; 85% had polyosteoarthritis; 55% had a lot of pain and 45% a lot of pain; 45% had stiffness; while 10% had no decrease in the functional capacity of the affected joints. Conclusions. Osteoarthritis affected the functional condition of most of the women and this was evidenced by the analysis of the evaluation of the result obtained by means of the Womac Questionnaire.

A osteoartrite pertence ao grupo mais comum de condições articulares degenerativas e é uma das principais causas de incapacidade no mundo, caracterizada pela dor articular, rigidez e perda da capacidade funcional. Objetivo. Avaliar a funcionalidade dos pacientes com osteoartrite que atendem ao Centro de Reabilitação Integral Especializado Guayaquil # 2. Materiais e métodos. Foi realizado um estudo sob uma abordagem quantitativa, com um escopo descritivo e transversal, desenvolvido por meio de medição numérica e análise de dados das variáveis em estudo, tais como idade; sexo; patologia; ocupação do trabalho; rigidez e capacidade funcional, por meio de opções de resposta. A coleta de dados foi realizada por meio da técnica de observação, para a análise dos resultados obtidos das Universidades Western Ontario e McMasters Questionário do Índice de Osteoartrite. Resultados. Foi determinado que, segundo o sexo, 90% eram do sexo feminino, a maioria entre 60 e 70 anos de idade; 70% do grupo estudado eram donas de casa; 85% tinham polioseartrose; 55% tinham muita dor e 45% muita dor; 45% tinham rigidez; enquanto 10% não tinham diminuição da capacidade funcional das articulações afetadas. Conclusões. A osteoartrose afetou a condição funcional da maioria das mulheres e foi evidenciada pela análise da avaliação do resultado obtido por meio do Questionário Womac.

Depressive symptoms and axial motor disorders in individuals with Parkinson's disease: a cross-sectional study / Sintomas depressivos e distúrbios motores axiais em indivíduos com doença de Parkinson: um estudo transversal

Abstract Background Depression is an important nonmotor symptom of Parkinson's disease (PD) and has been associated with the motor symptoms in these individuals. Objectives To determine whether there are relationships between depressive symptoms and abnormalities in axial postural alignment and axial motor deficits, especially postural instability, and trunk rigidity in PD. Methods In this cross-sectional study, 65 individuals were evaluated using the Beck Depression Inventory-II (BDI-II) for the analysis of depressive symptoms and underwent a postural assessment of head, trunk, and hip sagittal alignment through computerized photogrammetry. The MDS-UPDRS was used to assess clinical aspects of PD, the Trunk Mobility Scale was used to assess axial rigidity, and the MiniBESTest to assess balance. To determine the relationship between depressive symptoms and postural alignment, multiple linear regression analysis was performed. Results The participants with depressive symptoms had more severe motor deficits as well as greater trunk rigidity and worse postural instability (p < 0.05). When the postural angles were compared between men and women using Student's t-test, it was found that men had greater flexion angles of the head (p = 0.003) and trunk (p = 0.017). Using multiple linear regression analysis corrected for the age and sex of the participants, we verified that the anterior trunk inclination was significantly larger in the PD population with depressive symptoms (R2 = 0.453, β = 0.116, and p = 0.045). Conclusion PD individuals with depressive symptoms have more severe flexed trunk posture, mainly in older men. Additionally, more severe depressive symptoms are associated with worsening postural instability, trunk rigidity and motor deficits in this population.

Resumo Antecedentes A depressão é um sintoma não motor importante da doença de Parkinson (DP) e tem sido associada aos sintomas motores nesses indivíduos. Objetivos Determinar se existem relações entre sintomas depressivos e anormalidades no alinhamento postural axial e déficits motores axiais, especialmente instabilidade postural e rigidez de tronco na DP. Métodos Neste estudo transversal, 65 indivíduos foram avaliados pelo BDI-II para análise de sintomas depressivos e submetidos à avaliação postural do alinhamento sagital de cabeça, tronco e quadril por meio de fotogrametria computadorizada. A MDS-UPDRS avaliou os aspectos clínicos, TMS avaliou rigidez axial e o MiniBESTest equilíbrio. Para determinar a relação entre sintomas depressivos e alinhamento postural, realizou-se uma análise de regressão linear múltipla. Resultados Os participantes com sintomas depressivos apresentaram déficits motores mais graves, bem como maior rigidez de tronco e pior instabilidade postural (p < 0,05). Quando comparados os ângulos posturais entre homens e mulheres pelo teste t de Student, verificou-se que os homens apresentaram maiores graus de flexão da cabeça (p = 0,003) e do tronco (p = 0,017). Por meio da análise de regressão linear múltipla corrigida para a idade e sexo dos participantes, verificamos que a inclinação anterior do tronco foi significativamente maior nos indivíduos com DP com sintomas depressivos do que sem sintomas depressivos (R2 = 0,453, β = 0,116 e p = 0,045) Conclusão Indivíduos com DP com sintomas depressivos apresentam postura de tronco flexionado mais severa, principalmente em homens mais idosos. Além disso, os sintomas depressivos mais graves pioram significativamente a instabilidade postural, a rigidez do tronco e os déficits motores nessa população.

The polymorphism of metalloproteinases 1 and 13 and posttraumatic elbow stiffness / O polimorfismo das metaloproteinases 1 e 13 e a rigidez articular pós-traumática do cotovelo

ABSTRACT Introduction To evaluate the relationship between the genetic polymorphism of matrix metalloproteinases 1 and 13 and posttraumatic elbow stiffness, as well as the association of other risk factors with this condition. Materials and methods We evaluated 20 patients with posttraumatic elbow stiffness and 12 controls with traumatic elbow disorders without contracture. Deoxyribonucleic acid (DNA) was obtained from buccal mucosa epithelial cells of the volunteers. The MMP-1 and MMP-13 genotypes were determined using PCR-restriction fragment length polymorphism assays. Results We did not find any significant differences in the frequency of genotypes and alleles between the test and control groups for the polymorphism of metalloproteinases 1 and 13. We observed that genotypes 1G/2G and 2G/2G of MMP-1 were present in 65% (13/20) of patients with articular stiffness and 50% (6/12) of controls (p = 0.599). Genotypes A/A and A/G of MMP-13 were obtained in 95% (19/20) of patients and 91.6% (11/12) of controls (p = 0.491). Among the prognostic factors for elbow stiffness, only immobilization time correlated positively. The mean immobilization time for cases and controls were 16 ± 10 days and 7 ± 7 days, respectively (p = 0.017). Conclusion The genetic polymorphism of MMP-1 at position -1607 and MMP-13 at position -77 was not associated with post-traumatic elbow stiffness. Level of Evidence III; Prognosis Study; Case-Control Study.

RESUMO Introdução Avaliar a relação entre o polimorfismo genético das metaloproteinases 1 e 13 da matriz e a rigidez pós-traumática do cotovelo, assim como a associação de outros fatores de risco com essa condição. Material e método Foram avaliados 20 pacientes com rigidez pós-traumática do cotovelo e 12 controles com distúrbios traumáticos do cotovelo sem contratura. O ácido desoxirribonucleico (DNA) de voluntários foi obtido a partir de células epiteliais da mucosa bucal. Os genótipos MMP-1 e MMP-13 foram determinados usando ensaios de polimorfismo de comprimento de fragmento de restrição de PCR. Resultados Não encontramos diferença significativa na frequência de genótipos e alelos entre os grupos teste e controle para o polimorfismo das metaloproteinases 1 e 13. Observamos que os genótipos 1G/2G e 2G/2G de MMP-1 estavam presentes em 65% (13/20) dos pacientes com rigidez articular e 50% (6/12) dos controles (p = 0,599). Os genótipos A/A e A/G da MMP-13 foram obtidos em 95% (19/20) dos pacientes e 91,6% (11/12) dos controles (p = 0,491). Dentre os fatores prognósticos para rigidez de cotovelo, apenas o tempo de imobilização se correlacionou positivamente. O tempo médio de imobilização para casos e controles foi de 16 ± 10 dias e 7 ± 7 dias, respectivamente (p = 0,017). Conclusões O polimorfismo genético de MMP-1 na posição -1607 e MMP-13 na posição -77 não foi associado à rigidez pós-traumática do cotovelo. Nível de Evidência III; Estudos Prognósticos; Estudo de Caso-Controle.

Clinical, skeletal muscle pathological and genetic characteristics of fatal infantile hypertonic myofibrillar myopathy / 中华实用儿科临床杂志

Objective:To investigate the clinical, skeletal muscle pathological, and genetic characteristics of fatal infantile hypertonic myofibrillar myopathy (FIHMM).Methods:The clinical manifestations, laboratory assessments data and gene sequencing results of 10 patients diagnosed with FIHMM in Shenzhen Children′s Hospital from February 2017 to April 2021 were retrospectively analyzed.Magnetic resonance imaging (MRI) of both musculoskeletal system and the brain, and electromyogram (EMG) were performed in 3 cases, while muscle biopsy was performed in 2 cases.Results:Among these 10 cases, 1 case was from Northeast China and 1 case from East China, while the rest 8 cases were from South China.Eight of the 10 patients were male, and the other 2 cases were female.They were all born normal and not related to each other.The age of onset varied from 2 to 12 months.The main clinical manifestations for all the patients were progressive rigidity of the rectus abdominis (8 cases), neck muscles (7 cases), rectus abdominis (2 cases) and intercostal muscles (1 case), resulting in respiratory failure.Mildly to moderately elevated serum creatine kinase level was detected (436-5 804 IU/L) (reference range: 24-229 IU/L). Complex repetitive discharges can be seen in the EMG, without any myotonic potential.Muscle fiber degeneration, necrosis, and vacuolar degeneration were noted in the histopathological examination of the vastus lateralis and rectus abdominis.An abnormal red granular deposit was observed in a portion of the field of the modified Gomory Trichrome staining.Immunohistochemistry showed substantial deposition of desmin.Under the electron microscopy, the sarcomere structure of the muscle fibers was seriously disordered, with the destruction of Z-bands and the presence of granular deposits.The whole-exome sequencing identified the same homozygous variation c. 3G>A, p.Met1? of CRYAB gene in all the patients, but heterozygous variation in their parents. Conclusions:Axial muscles involvement, such as rectus abdominis rigidity, is the main clinical characteristic of FIHMM.c.3G>A, p.Met1? mutation in the CRYAB gene is a hotspot mutation in Chinese children.

Changes in the flexor and extensor muscles at different speeds / Mudanças nos músculos flexor e extensor a diferentes velocidades / Cambios en los músculos flexor y extensor a diferentes velocidades

ABSTRACT Introduction: Centrifugal strength is an important element for strength quality. Developing muscle centrifugal strength can effectively increase the stability of lower limbs and reduce the risk of injury. Objective: To explore the characteristics of contractile force of flexor ahods in strength training and rehabilitation training, and the extensor muscles of the knee joint in athletes with different speeds of centripetal force. Methods: The knee joint muscle group of 8 first-level male high jumpers and 8 second-level male high jumpers were tested by isokinetic centrifugal contraction; the angular test velocity was 60 °/s, 120 °/s, 240 °/s, and the indexes included peak torque, relative peak torque (peak torque/body weight), and the peak torque flexural extension ratio. Results: With the centrifugal contraction of the knee joint muscle group (P < 0.05), the second-level high jumpers should increase the ability of the knee flexor muscle group of the take-off leg. In the case of constant velocity centrifugal contraction (P < 0.01), taking off time must be reduced, that is, taking off speed must be accelerated. Conclusions: The difference in the knee joint muscle isokinetic test results is one of the reasons for the difference in knee joint flexor and extensor muscle contractility under the different speed forces of high jumpers. Level of evidence II; Therapeutic studies - investigation of treatment results.

RESUMO Introdução: A força centrífuga é um elemento importante na qualidade de força. O desenvolvimento da força centrífuga muscular pode, efetivamente, aumentar a estabilidade dos membros inferiores e reduzir o risco de lesões. Objetivo: Explorar as características da força contrátil do músculo flexor no treinamento de força e de reabilitação, e os músculos extensores da junta do joelho em atletas com diferentes velocidades de força centrípeta. Métodos: O grupo muscular da articulação do joelho de oito saltadores de primeiro escalão do sexo masculino, e oito saltadores de segundo escalão do sexo masculino foi testado por contração centrífuga isocinética. A velocidade de teste angular foi de 60 °/s, 120 °/s, 240 °/s, e os índices incluíram torque de pico, torque de pico relativo (torque de pico/peso corporal) e a razão extensão-flexão de torque de pico. Resultados: Na contração centrífuga do grupo muscular da articulação do joelho (P < 0,05), os saltadores de segundo escalão devem aumentar a habilidade do grupo muscular flexor do joelho da perna de arranque. No caso da contração centrífuga de velocidade constante (P < 0,01), o tempo de arranque deve ser reduzido, ou seja, a velocidade de arranque deve ser acelerada. Conclusões: A diferença nos resultados dos testes isocinéticos do músculo da junta do joelho é uma das razões para a diferença na contratilidade muscular flexor e extensor da junta do joelho sob forças de velocidade diferentes em saltadores. Nível de evidência II; Estudos terapêuticos - investigação de resultados de tratamento.

Resumen Introducción: La fuerza centrífuga es un elemento importante en la calidad de fuerza. El desarrollo de la fuerza centrífuga muscular puede efectivamente aumentar la estabilidad de los miembros inferiores y reducir el riesgo de lesiones. Objetivo: Explorar las características de la fuerza contráctil del músculo flexor en el entrenamiento de fuerza y de rehabilitación, y los músculos extensores de la articulación de la rodilla en atletas con diferentes velocidades de fuerza centrípeta. Métodos: Se testó el grupo muscular de la articulación de la rodilla de 8 saltadores de primer escalafón del sexo masculino, y 8 saltadores de segundo escalafón del sexo masculino por contracción centrífuga isocinética. La velocidad de prueba angular fue de 60 °/s, 120 °/s, 240 °/s, y los índices incluyeron torque de pico, torque de pico relativo (torque de pico/peso corporal) y la razón extensión-flexión de torque de pico. Resultados: En la contracción centrífuga del grupo muscular de la articulación de la rodilla (P<0,05), los saltadores de segundo escalafón deben aumentar la habilidad del grupo muscular flexor de la rodilla de la pierna de arrancada. En el caso de la contracción centrífuga de velocidad constante (P<0,01), el tiempo de arrancada debe reducirse, o sea, la velocidad de arrancada debe acelerar. Conclusiones: La diferencia en los resultados de las pruebas isocinéticas del músculo de la articulación de la rodilla es una de las razones para la diferencia en la contractilidad muscular flexor y extensor de la articulación de la rodilla bajo fuerzas de velocidad diferentes en saltadores. Nivel de evidencia II; Estudios terapéuticos - investigación de resultados de tratamiento.

Luxaciones puras de codo en pacientes pediátricos: tratamiento conservador y complicaciones asociadas a una patología poco prevalente. Serie de 4 casos / Isolated elbow dislocation in pediatric patients: non-operative treatment and complications associated with an infrequent pathology. Series of 4 cases

Si bien el codo es la articulación más frecuentemente luxada en niños, representa el 3-6 % de las lesiones en ese sitio. Las luxaciones sin fracturas asociadas son muy raras y son producto de una caída con el codo en extensión. El paciente consulta por dolor, impotencia funcional y deformidad evidente. La finalidad del tratamiento es restaurar la congruencia articular, lograr estabilidad y minimizar los riesgos de posibles lesiones neurovasculares.Se presentan 4 pacientes tratados con manejo conservador con excelentes resultados funcionales, incluso aquel que presentó una neuropraxia del mediano con restitución ad integrum.Según nuestra experiencia, suelen ser lesiones con buena evolución. Se destaca la importancia de un rápido y preciso examen neurovascular, optando, de ser posible, por una conducta expectante ante las lesiones nerviosas. Se resalta la indicación de una inmovilización acotada con movilización temprana que evite rigidez del codo.

Even though the elbow is the most often dislocated joint in children, this injury accounts for 3-6 % of elbow pathology. Dislocations without associated fractures are extremely rare. They result from a fall onto an outstretched hand. The patient is always referred with a painful joint, movement impairment and even clinical deformity. Acute treatment aims to achieve quick reduction and adequate joint stability, avoiding neurovascular injuries.We sought to analyze the functional outcomes and the complications after non-operative treatment. Our 4 patien had excellent functional results at the latest follow-up, and one of them suffered from a median nerve palsy without further consequences.In our experience, these injuries presented excellent outcomes and we would like to highlight the importance of a quick and precise neurovascular examination with the possibility of non-surgical management of nerve injuries. A short period of immobilization with early rehabilitation should be indicated to avoid joint stiffness

Sociodemographic profile and stages of Parkinson's disease associated with predictors of Temporomandibular Disorder / Perfil sociodemográfico e estágios da doença de Parkinson associados com os preditores de disfunção temporomandibular

ABSTRACT Purpose: to analyze the predictors of temporomandibular disorder in people with Parkinson's disease, verifying their associations with sociodemographic aspects and stages of the disease. Methods: a study based on secondary data from research conducted in 2017 with 110 people with Parkinson's disease. They were assessed with the Research Diagnostic Criteria for Temporomandibular Disorders and the Parkinson's disease staging scale. The studied predictive variables for temporomandibular disorder were pain, crepitation, clicking, nighttime and daytime clenching/gnashing, uncomfortable/non-habitual bite, morning rigidity, and tinnitus. The sociodemographic aspects assessed were age, sex, schooling level, marital status, income, and stages 1 to 3 of the disease. The chi-squared odds ratio was used with a 95% confidence interval and significance level at p < 0.05. Results: an association was verified between nighttime clenching/gnashing and income (p = 0.006); tinnitus and income range from ½ to 3 (p = 0.003) and from 4 to 10 minimum wages (p = 0.004); and between tinnitus and stage 1 (p = 0.02). Conclusion: this study verified that the predictors associated with temporomandibular disorder in people with Parkinson's disease were pain, clicking, crepitation, uncomfortable/non-habitual bite, and morning rigidity. It was verified that income and stage 1 of the disease had an association with nighttime clenching/gnashing and tinnitus.

RESUMO Objetivo: analisar os preditores de disfunção temporomandibular em pessoas com doença de Parkinson (DP) verificando suas associações com aspectos sociodemográficos e estágios da doença. Métodos: estudo que utilizou fonte de dados secundários de uma pesquisa realizada em 2017, com 110 pessoas com DP que foram avaliadas pelo questionário para Pesquisa em Disfunção Temporomandibular (RDC/TMD) e pela escala de estadiamento da DP. As variáveis preditoras de DTM estudadas foram: dor, crepitação, estalido, apertamento/rangido noturno e diurno, mordida desconfortável/não habitual, rigidez matinal e zumbido. Os aspectos sociodemográficos avaliados foram: idade, sexo, escolaridade, estado civil, renda e estágios da doença de 1 a 3. Utilizou-se o Odds ratio do Qui-quadrado com intervalo de confiança de 95% e nível de significância de p<0,05. Resultados: verificou-se associação entre os preditores: apertamento/rangido noturno e renda (p=0,006); zumbido e grupos de renda ½ a 3 (p=0,003) e de 4 a 10 salários mínimos (p=0,004). Além da associação do zumbido e estágio 1 (p=0,02). Conclusão: nesse estudo verificou-se que os preditores associados com a DTM em pessoas com DP foram: dor, estalido, crepitação, mordida desconfortável/não habitual e rigidez matinal. E destes verificou-se associação entre renda e estágio 1 da doença com apertamento/rangido noturno e zumbido.

Analysis of the relationship of the vertical extension of mouth opening with muscle rigidity and sociodemographic factors in Parkinson's disease / Análise da relação da extensão vertical da abertura de boca com a rigidez muscular e fatores sociodemográficos na doença de Parkinson

Introduction: Changes caused by the rigidity of Parkinson's Disease (PD) can affect the mandibular musculature. However, few studies have been published about its impact on the oral opening. Objective: To analyze the relationship of the vertical extension of the oral opening with muscular rigidity and sociodemographic factors of the elderly with PD. Material and method: This is a cross-sectional, quantitative study that collected data from a primary study conducted at the Hospital das Clínicas of the Federal University of Pernambuco in 2018. Data were collected from medical records and from the questionnaire, Research Diagnostic Criterion for Temporomandibular Disorders (RDC/TMD). The sample was composed of 81 parkinsonians and characterized using: sociodemographic variables and the presence or absence of muscular rigidity. The measures of vertical extension of the oral opening evaluated were: mouth opening without assistance and without pain (ABASD), and maximum mouth opening without assistance (AMBSA). The Pearson's linear correlation and Spearman's correlation tests were applied to investigate the relationship among the continuous variables. Analyses of association were conducted using simple logistic regression. The level of significance was set at p<0.05. Result: Limitation of the oral opening was not related to age or sex. The greatest level of significance was between mouth opening without assistance and without pain and muscular rigidity (p=0.012), and years of schooling (p=0.038). Conclusion: The limitation of mouth opening in people with PD was shown to be related to muscular rigidity and fewer years of schooling.

Introdução: As alterações causadas pela rigidez na Doença de Parkinson (DP) podem afetar a musculatura mandibular, mas há uma escassez de estudos publicados sobre seu impacto na abertura bucal. Objetivo: Analisar a relação da extensão vertical da abertura de boca com a rigidez muscular e os fatores sociodemográficos de idosos com a DP. Material e método: Trata-se de um estudo transversal, quantitativo, que coletou dados de uma pesquisa primária realizada no Hospital das Clínicas da Universidade Federal de Pernambuco, em 2018. Apanhou-se dados dos prontuários e do questionário: Critério de Diagnóstico de Pesquisa para Disfunções Temporomandibulares (RDC/TMD). A amostra foi composta por 81 parkinsonianos e caracterizadas em: variáveis sociodemográficas e presença ou ausência de rigidez muscular. As medidas de extensão vertical de abertura de boca avaliadas foram: abertura de boca sem auxílio e sem dor (ABASD) e abertura máxima da boca sem auxílio (AMBSA). Aplicou-se os testes de correlação linear de Pearson e de correlação de Spearman para averiguar relação entre as variáveis contínuas. Análises de associações foram realizadas através da regressão logística simples. Nível de significância de p<0,05. Resultado: A limitação de abertura de boca não apresentou relação com a idade e o sexo. O maior nível de significância foi entre a abertura de boca sem auxílio e sem dor e a rigidez muscular (p= 0,012) e a escolaridade (p= 0,038). Conclusão: A limitação de abertura de boca nas pessoas com DP se mostrou relacionada a rigidez muscular e a escolaridade mais baixa.

Pattern and treatment of Parkinson’s disease at different health care levels in Bangladesh: a hospital based survey

Background:Parkinson’s disease (PD) is a condition in which part of the brain becomes progressively damaged over many years. This study represents the pattern of Parkinson’s disease and help to identify various drugs which are being used at different health care levels in Bangladesh.Methods:Cross-sectional technique was applied as study design in this research work. We accessed the patients with formulated questionnaire of the Department of Neuroscience of National Institute of Neurosciences and Hospital, Dhaka Medical College and Hospital and Bangabandhu Sheikh Mujib Medical University (BSMMU) for data collection from January 2017 to August 2019.100 patients were selected in the ages between 25-80 years, among them 66were male and 34females.Results:A total number of 100 Parkinson’s disease patients (male 66%, female 34%) were recruited for this study. Genetic factor (56%) is the main cause of PD found in this study. Among various symptoms, the prominent symptoms were voice disorders (96%), slowness of movement (90%), mask-like face expression (86%), tremor (80%), sensory and sleep difficulties (78%), excessive sweating (60%) and insomnia (56%). It was observed that along with physiotherapy, drugs used to manage PD were levodopa (14%), carbamazepine (12%), quetiapine (12%), haloperidol (11%), pramipexole (10%), trihexyphenidyl HCl (10%), carbidopa (8%), amlodipine (8%)andclonazepam (8%).Conclusions:Disgrace exists in the personal life and social context of the PD patients which also unfavourably affects their psychosocial aspects of life. Our population-based data provide evidence for a protective effect of Parkinson’s disease in our country.

La rigidez cadavérica, el espasmo cadavérico y tipos de fibras musculares / The cadaveric rigidity, the cadaveric spasm and types of muscle fibers

La rigidez cadavérica (rigor mortis) es un proceso no muy bien comprendido por la mayoría de los médicos. El conocimiento de la intimidad del proceso de la rigidez cadavérica es de vital importancia ya que es una de las variables que junto con las livideces (livor mortis) y la temperatura (algor mortis) del cadáver ayudan a determinar el cronotanatodiagnóstico, tanatocronodiagnóstico o intervalo postmortal del período inmediato de la muerte. Para entender el mecanismo de la rigidez y el espasmo cadavérico es preciso hacer un repaso de la contracción muscular fisiológica en el vivo. Hay que tener presente que el tipo de fibra muscular predominante modificará las características de la contracción muscular fisiológica en el vivo, y también la rigidez y el espasmo cadavérico. (AU)

The cadaveric rigidity (rigor mortis) is a process which is not very well understood by the majority of the doctors. The knowledge of the intimacy of the cadaveric stiffness process is of vital importance since it is one of the variables that, as well as the postmortem lividity (livor mortis) and the body temperature post mortem (algor mortis) help determine the chronotanatodiagnostic, tanatochronodiagnostic or postmortal interval of the immediate period of death. In order to understand the mechanism of stiffness and cadaveric spasm, it is necessary to review the physiological muscle contraction in vivo. We should keep in mind that the predominant type of muscle fiber will modify the characteristics of physiological muscle contraction in vivo, as well as stiffness and cadaveric spasm. (AU)

Consecutive nightly measurements are needed for accurate evaluation of nocturnal erectile capacity when the first-night laboratory recording is abnormal / 亚洲男科学杂志(英文版)

Multiple measurements of nocturnal penile tumescence and rigidity (NPTR) are widely accepted as a method to differentiate psychogenic erectile dysfunction (ED) from organic ED. However, direct evidence remains limited regarding the first-night effect on NPTR measurement using the RigiScan. Here, we evaluated the first-night effect on the results of NPTR measurement to validate the necessity of NPTR measurement for two consecutive nights, particularly when abnormal first-night measurements are recorded in a laboratory setting. We retrospectively reviewed 105 patients with a complaint of ED, who underwent NPTR measurement using the RigiScan in the Department of Infertility and Sexual Medicine, the Third Affiliated Hospital of Sun Yat-sen University (Guangzhou, China), for two consecutive nights, during the period from November 2015 to May 2016. NPTR parameters were collected and analyzed. We found that more effective nocturnal erections were detected during the second night than during the first night (P <0.001). Twenty percent of all patients had no effective erection during the first night, but exhibited at least one effective erection during the second night. The negative predictive value of NPTR measurement during the first night was 43.2%; this was significantly lower than that on the second night (84.2%; P = 0.003). Most NPTR parameters were better on the second night than on the first night. The first-night effect might be greater among patients younger than 40 years of age. In conclusion, two consecutive nightly measurements of NPTR can avoid a false-abnormal result caused by the first-night effect; moreover, these measurements more accurately reflect erectile capacity, especially when the first-night record is abnormal in a laboratory setting.

A Case of Paroxysmal Vomiting with Diarrhea Treated by Ryoukeijutsukanto / 日本東洋医学雑誌

The patient was an 80-year-old woman who had been suffering from paroxysmal vomiting with diarrhea about twice a month for 3 years. She had these symptoms at around 11:00 am every time and got over them after a rest for about 20 minutes. Though no abnormality was detected by the inspections in the gastroenterology and the cardiology, the interval of her attacks became short. These symptoms were treated with ryokeijutsukanto. We conclude that the pathogeneses of this case were excessive accumulation of fluids in epigastrium caused by the lack of stomach qi, and that these were ameliorated by the effect of ryokeijutsukanto mainly in nourishing stomach qi and cleaning away excessive accumulation of water. There is a report that the stuffiness and rigidity below the heart shows the presence of excessive accumulation of fluids in epigastrium, and this case suggests the possibility that this symptom of abdominal diagnosis by palpation is seen only during an attack.

Head and neck symptoms as predictors of outcome in tetanus patients

@#Objective: This study aims to investigate which, if any head and neck symptoms (trismus, dysphagia, alterations in speech or facial movements, and dyspnea) might be good predictors of outcomes (mortality, tracheostomy, discharged, decannulated) and prognosis of tetanus patients. Methods: Design: Retrospective Cohort Study Setting: Tertiary National University Hospital Patients: Seventy-three (73) pediatric and adult patients diagnosed with tetanus and admitted at the emergency room of the Philippine General Hospital between January 1, 2013 and December 31, 2017. Demographic characteristics, incubation periods, periods of onset, routes of entry, head and neck symptoms, stage, and outcomes were retrieved from medical records and analyzed. Results: Of the 73 patients included, 53 (73%) were adults, while the remaining 20 (27%) were pediatric. The three most common head and neck symptoms were trismus (48; 66%), neck pain/ rigidity (35; 48%), and dysphagia to solids (31; 42%). Results of multivariate logistic regression analysis showed that only trismus (OR = 3.742, p = .015) and neck pain/ rigidity (OR = 4.135, p = .015) were significant predictors of decannulation. No dependent variable/symptoms had a significant effect in predicting discharge and mortality. Conclusion: Clinically diagnosed tetanus can be easily recognized and immediately treated. Most of the early complaints are head and neck symptoms that can help in early diagnosis and treatment resulting in better prognosis. In particular, trismus and neck pain/rigidity may predict the outcome of decannulation after early tracheotomy, but not of discharge and mortality.

Camillo Negro and his contributions to neurology / Camillo Negro e suas contribuições à Neurologia

ABSTRACT An historical review of the contributions made by Italian professor, Camillo Negro, to neurology. Negro published several books on clinical neurology, was one of the pioneers of scientific films and described numerous neurological diseases. He is best known for describing the cogwheel phenomenon in patients with Parkinson's disease but also described a sign of peripheral facial paralysis.

RESUMO Os autores apresentam uma revisão histórica sobre as contribuições do Professor italiano Camilo Negro para à neurologia. Negro publicou vários livros sobre clínica neurológica e também foi um dos pioneiros na realização de filmes científicos, com a descrição de inúmeras doenças neurológicas. Ele é mais conhecido pela descrição do fenômeno da roda denteada em pacientes com a doença de Parkinson, mas também descreveu um sinal da paralisia facial periférica.

Clinical study of meningitis in children and role of adenosine deaminase in differentiating tubercular from non-tubercular meningitis

Background: In diseases where cell mediated immunity plays an important role like in tuberculosis, CSF (Cerebrospinal fluid) adenosine deaminase (ADA) activity is increased. Hence, this testing is done especially among children with suspected tubercular meningitis. The objective of this was to study role of Adenosine Deaminase in differentiating tubercular from non-tubercular meningitis.Methods: This was a hospital based cross sectional study was carried out among 50 children of proven meningitis for a period of two years. CSF ADA levels were done for all cases. They were divided into tubercular meningitis and non-tubercular meningitis group and compared.Results: Incidence of tuberculous meningitis (TBM) was found high in the age group 5-8 years. Male:female ratio was 2.3:1. Fever was present in all cases followed by vomiting and neck rigidity. CSF AFB was positive in 3 cases (6%). Neuroimaging was done in all cases in which 3 patients had basal exudate in which 2 patients had TBM and 1 patient was Non-TBM. 3 patients had hydrocephalus in MRI in which 2 had TBM diagnosed and 1 had non-TBM. As per ADA levels, 10 were diagnosed as TBM out of which 8 patients had ADA level ?10 IU/L and 2 had ADA level <10 IU/L which was missed by ADA and later confirmed by other modalities.Conclusions: It was concluded that if ADA level in CSF is ?10IU/l the diagnosis of TBM should be considered.

Cholesterol-tuned liposomal membrane rigidity directs tumor penetration and anti-tumor effect

Recently, liposomes have been widely used in cancer therapeutics, but their anti-tumor effects are suboptimal due to limited tumor penetration. To solve this problem, researchers have made significant efforts to optimize liposomal diameters and potentials, but little attention has been paid to liposomal membrane rigidity. Herein, we sought to demonstrate the effects of cholesterol-tuned liposomal membrane rigidity on tumor penetration and anti-tumor effects. In this study, liposomes composed of hydrogenated soybean phospholipids (HSPC), 1,2-distearoyl--glycero-3-phosphoethanolamine--[methoxy(polyethylene glycol)-2000] (DSPE-PEG) and different concentrations of cholesterol were prepared. It was revealed that liposomal membrane rigidity decreased with the addition of cholesterol. Moderate cholesterol content conferred excellent diffusivity to liposomes in simulated diffusion medium, while excessive cholesterol limited the diffusion process. We concluded that the differences of the diffusion rates likely stemmed from the alterations in liposomal membrane rigidity, with moderate rigidity leading to improved diffusion. Next, the tumor penetration and the anti-tumor effects were analyzed. The results showed that liposomes with moderate rigidity gained excellent tumor penetration and enhanced anti-tumor effects. These findings illustrate a feasible and effective way to improve tumor penetration and therapeutic efficacy of liposomes by changing the cholesterol content, and highlight the importance of liposomal membrane rigidity.

A Neuroleptic Malignant Syndrome Without Rigidity

Neuroleptic malignant syndrome (NMS) is an uncommon but potentially lethal idiosyncratic reaction which may emerge in the aftermath of the treatments with neuroleptics demonstrating itself with the symptoms of altered consciousness, high fever, impaired autonomic functions, and muscle rigidity. Although various risk factors have been identified for NMS, its etiology is not completely known. The mortality and morbidity related with NMS could be reduced by early diagnosis, interruption of the neuroleptics used within a short period and aggressive treatment. Our case is different from general NMS cases due to lack of rigidity. A NMS case which developed within a short time in the aftermath of multiple antipsychotic use and wherein no rigidity was observed shall be discussed in this case report.

A mechanical impedance-based measurement system for quantifying Parkinsonian rigidity / 生物医学工程学杂志

At present the parkinsonian rigidity assessment depends on subjective judgment of neurologists according to their experience. This study presents a parkinsonian rigidity quantification system based on the electromechanical driving device and mechanical impedance measurement method. The quantification system applies the electromechanical driving device to perform the rigidity clinical assessment tasks (flexion-extension movements) in Parkinson's disease (PD) patients, which captures their motion and biomechanical information synchronously. Qualified rigidity features were obtained through statistical analysis method such as least-squares parameter estimation. By comparing the judgments from both the parkinsonian rigidity quantification system and neurologists, correlation analysis was performed to find the optimal quantitative feature. Clinical experiments showed that the mechanical impedance has the best correlation (Pearson correlation coefficient = 0.872, < 0.001) with the clinical unified Parkinson's disease rating scale (UPDRS) rigidity score. Results confirmed that this measurement system is capable of quantifying parkinsonian rigidity with advantages of simple operation and effective assessment. In addition, the mechanical impedance can be adopted to help doctors to diagnose and monitor parkinsonian rigidity objectively and accurately.