Urrets-Zavalia Syndrome after Penetrating Keratoplasty: A Case Report

Urrets-Zavalia syndrome is a dreaded complication of anterior segment surgery and the most common in patients undergoing corneal transplantation. Avoiding the prescription of mydriatic eye drops during the postoperative period is the mainstay of its therapy, which focuses on prevention. One month after having keratoplasty in the left eye, a 42-year-old man presented with Urrets-Zavalia syndrome and ocular hypertonia at 35 mmgh. Medical and surgical management were required in this situation.The purpose of our presentation is to describe a real-world instance of Urrets-Zavalia syndrome.

Desafió diagnóstico-Diciembre / Diagnostic Challenge- December

Recién nacido a término adecuado para la edad gestacional, producto #3 de madre de 26 años la cual se realizó #6 controles prenatales. Nace vía parto vaginal eutócico, sin complicaciones. Durante el examen físico de rutina, se evidencia hipertonía de miembros superiores e inferiores, hiperextensión del cuello y microcefalia.

Newborn at term suitable for gestational age, product # 3 of a 26-year-old mother who underwent # 6 prenatal controls. It is born via eutocic vaginal delivery, without complications. During the routine physical examination, hypertonia of the upper and lower limbs, hyperextension of the neck, and microcephaly are evident

Consideraciones clínicas en un paciente con hipertonicidad muscular de mentón. Reporte de caso / Clinical considerations in a patient with mint muscle hi-pertonicity. Caso report / Gestão de pacientes adultos com mordida aberta anterior por deglução atípica. Relato de caso

En ortodoncia es de relevante importancia crear una oclusión estéticamente agradable y funcional, además de lograr y preservar el óptimo atractivo facial; es mandatorio realizar un examen facial minucioso porque el equilibrio y la armonía de las diferentes partes de la cara está determinada por los tejidos du-ros y blandos. El objetivo del presente artículo es exponer la importancia de un diagnóstico integral al momento de la plani-ficación del tratamiento. El presente caso, relata el manejo ortodóntico con filosofía Roth de una paciente de 13 años de edad, raza latina, diagnosticada con relación esquelética clase II, proinclinación excesiva de los incisivos en ambos arcos, perfil convexo e hi-pertonicidad del mentón. El plan de tratamiento se planificó para corregir la y la clase II esquelética mediante extracciones de los 4 primeros premolares. Como resultado se eliminó la hipertonicidad muscular, obteniendo una competencia labial adecuada y se mejoró el perfil facial en un tiempo de 18 meses. El mane-jo de este caso resulta exitoso gracias a un diagnóstico correcto y la planifi-ca-ción del caso, permite demostrar que no solo son objetivos ortodónticos las es-tructuras óseas y dentales, sino también aquellos que ayudan a mejorar el per-fil facial del paciente. Fue necesario evaluar la relación de los labios y la barbi-lla ya que estos pueden alterarse con el tratamiento de ortodoncia.

In orthodontics It's of relevant importance to create an aesthetically pleasing and functional occlusion, in addi-tion to achieving and preserving the optimal facial attractiveness; It is mandatory to perform a thorough facial exam because the balance and harmony of the different parts of the face is determined by soft and soft tissues. The objective of this article is to expose the importance of a comprehensive diagnosis at the time of treatment planning. The present case relates the orthodontic management with Roth philosophy of a 13-year-old Latina patient, diagnosed with a class II skeletal relationship, excessive incision of the incisors in both arches, convex profile and chin-hypertonicity. The treatment plan was planned to correct the skeletal class II by extractions of the first 4 premolars. As a result, muscular hypertonicity was eliminated, obtaining adequate lip competence and the facial profile was improved in a period of 18 months. The management of this case is successful thanks to a correct diagnosis and the planning of the case, allows to demonstrate that not only are the bone and dental structures orthodontic objectives, but also those that help improve the patient's facial profile. It was necessary to evaluate the relationship of the lips and chin since these can be altered with orthodontic treatment.

A mordida aberta anterior é uma má oclusão na qual um ou mais dentes não atingem o plano oclusal e não é feito contato com seus antagonistas. Seu tratamento é difícil e há controvérsia na necessidade de uma abord-agem cirúrgica. O presente caso refere-se ao tratamento de uma paciente de 61 anos de idade, atendida na clínica do Instituto Mexicano de Ortodontia, que veio pela "separação dos seus dentes". A avaliação clínica e radiográfica apresentou classe esquelética biprotrusiva I, com sobremordida de -30% com sorriso baixo e não consoante, deglutição atípica como fator etiológico que permitiu o diagnóstico de mordida aberta anterior. O tratamento consistiu na colocação de suportes de prescrição Roth .022 com tubos até os terceiros molares superiores e inferiores, colocando os suportes mais voltados para a gengiva em relação ao centro da coroa anatômica de ambos os arcos para obter extrusão e fechamento da mordida e arco aberto de sorriso; nas centrais a 1,5 mm, laterais a 1 mm e caninos a 0,5 mm. O uso de topes oclusais posteriores e também de elásticos anteriores curtos e leves desde o primeiro dia de tratamento foi essencial. A correção da posição dos dentes foi realizada em um período de 18 meses, o que também induziu uma alteração no padrão da deglutição. Através do plano de tratamento proposto, demonstrou-se que a estabilidade da oclusão pode ser dada e que nem todo paciente com mordida aberta é candidato ao tratamento cirúrgico para correção dessa má oclusão.

El bruxismo en estudiantes de preuniversitario y su repercusión en el sistema estomatognático / Bruxism in pre-university students and its effects on the stomatognathic system

RESUMEN Introducción: el bruxismo es un hábito para funcional que se caracteriza por la acción incesante de rechinar y/o apretar los dientes de manera inadecuada, lo cual provoca alteraciones del sistema estomatognático. Objetivo: describir el comportamiento del bruxismo en estudiantes del preuniversitario Isabel Rubio Díaz. Métodos: se realizó un estudio descriptivo de corte transversal en jóvenes matriculados en el preuniversitario Isabel Rubio Díaz, municipio San Juan y Martínez, Pinar del Río, de septiembre de 2017 a junio de 2018. El universo estuvo constituido por 570 alumnos inscritos en el curso diurno de ese plantel escolar, de ambos sexos, cuyas edades estuvieran comprendidas en un rango entre 16 a 18 años cumplidos, seleccionándose de ellos 198 pacientes con características clínicas que los avalaron como bruxópatas. Se procesaron los datos manualmente y se presentaron en tablas estadísticas. Se utilizó estadística analítica para cada variable y la información fue expresada en frecuencias absoluta y relativa con el propósito de resumir la misma. Resultados: el mayor número de pacientes afectados correspondió al sexo femenino con predominio del grupo de 18 años. Los signos y síntomas más frecuentemente encontrados fueron la cefalea y las alteraciones dentarias. El factor de riesgo que estuvo más relacionado con esta enfermedad fue el estrés. Conclusiones: se describió el comportamiento del bruxismolo que posibilita proponer nuevas investigaciones sobre factores relacionados con el origen y prevención de dicha enfermedad para así lograr controlar sus riesgos y daños a la salud.

ABSTRACT Introduction: bruxism is a parafunctional habit characterized by the incessant action of grinding and/or clenching teeth inappropriately, which causes alterations in the stomatognathic system. Objective: to describe the behavior of bruxism in pre-university students belonging to the health area of San Juan y Martínez municipality. Methods: a descriptive and cross-sectional study was carried out on young people enrolled at Isabel Rubio Diaz pre-university, San Juan y Martínez municipality, Pinar del Río province, during September 2017 to June 2018. The target group consisted of 570 students of both sexes enrolled in the morning session of the institute, with ages between 16 and 18 years. Selecting students with the clinical characteristics as bruxism (n=198). Data were processed through analytical statistics for each variable and the information was expressed in absolute and relative frequencies. Results: the highest number of patients affected belonged to female sex, predominantly in the 18-year-old group. The most frequently signs and symptoms found were headache and dental alterations, being stress the most associated risk factor with this disorder. Conclusions: the behavior of bruxism was described, which made it possible to propose new research on factors related to the origin and prevention of this disorder in order to control its associated risks and the harm of health.

Hypertonia-linked protein Trak1 functions with mitofusins to promote mitochondrial tethering and fusion

Hypertonia is a neurological dysfunction associated with a number of central nervous system disorders, including cerebral palsy, Parkinson's disease, dystonia, and epilepsy. Genetic studies have identified a homozygous truncation mutation in Trak1 that causes hypertonia in mice. Moreover, elevated Trak1 protein expression is associated with several types of cancers and variants in Trak1 are linked to childhood absence epilepsy in humans. Despite the importance of Trak1 in health and disease, the mechanisms of Trak1 action remain unclear and the pathogenic effects of Trak1 mutation are unknown. Here we report that Trak1 has a crucial function in regulation of mitochondrial fusion. Depletion of Trak1 inhibits mitochondrial fusion, resulting in mitochondrial fragmentation, whereas overexpression of Trak1 elongates and enlarges mitochondria. Our analyses revealed that Trak1 interacts and colocalizes with mitofusins on the outer mitochondrial membrane and functions with mitofusins to promote mitochondrial tethering and fusion. Furthermore, Trak1 is required for stress-induced mitochondrial hyperfusion and pro-survival response. We found that hypertonia-associated mutation impairs Trak1 mitochondrial localization and its ability to facilitate mitochondrial tethering and fusion. Our findings uncover a novel function of Trak1 as a regulator of mitochondrial fusion and provide evidence linking dysregulated mitochondrial dynamics to hypertonia pathogenesis.

Técnica para redução do tônus e alongamento muscular passivo: efeitos na amplitude de movimento de crianças com paralisia cerebral espástica / Technique to reduce tonus and passive muscle stretching: effects on range of motion in children with spastic cerebral palsy

INTRODUÇÃO: A paralisia cerebral (PC) é resultante de lesão no encéfalo em fase de maturação, acarretando em disfunções motoras. A espasticidade, forma mais comum de acometimento, gera prejuízos funcionais intensificados pela diminuição da mobilidade. OBJETIVO: Analisar os efeitos do alongamento passivo lento do músculo tríceps sural e de técnica para diminuir o tônus do conceito Bobath na amplitude de movimento (ADM) de dorsiflexão do tornozelo de crianças com PC espástica. MÉTODOS: Participaram 18 crianças atendidas no Hospital Estadual Mario Covas de Santo André e na Santa Casa de Diadema. O grau da hipertonia do músculo tríceps sural foi determinado pela Escala de Aswhorth Modificada e a ADM de dorsiflexão foi medida pela goniometria. Esses dois procedimentos foram realizados antes e após as seguintes situações: 1) aplicação de um protocolo de alongamento muscular passivo; 2) protocolo com uma técnica para diminuir o tônus do conceito Bobath; e 3) emprego associado dos dois protocolos. RESULTADOS: O grau de hipertonia não se modificou após o protocolo 1, porém os protocolos 2 e 3 diminuíram a espasticidade de maneira semelhante, conforme a Escala de Ashworth Modificada. Já o ângulo de dorsiflexão aumentou após aplicação dos três protocolos: 1 (p=0,176); 2 (p=0,008); e 3, com o aumento mais significativo (p=0,003). CONCLUSÃO: A técnica para redução do tônus mostrou efeito positivo na redução da espasticidade, segundo a Escala de Ashworth Modificada, e no aumento da ADM de crianças espásticas. A execução subsequente do alongamento muscular aumentou sua efetividade.

INTRODUCTION: Cerebral palsy (CP) is consequent of brain injury in the maturation phase, causing motor dysfunctions. The decreased mobility is intensified by spasticity, most common form of attack, generating functional impairment. OBJECTIVE: To analyze the effects of slow passive stretching of triceps sure muscle and technique for decreasing the tonus of Bobath concept on the range of motion (ROM) of ankle dorsiflexion in children with spastic CP. METHODS: 18 children seen at Hospital Estadual Mario Covas, in Santo André, and Santa Casa, in Diadema, participated in this study. The hypertonia rate of triceps sure muscle was determined by Modified Ashworth Scale and the ROM dorsiflexion by goniometer. Both procedures were realized before and after the following situations: 1) application of a passive muscle stretching protocol; 2) protocol with technique for decreasing the tonus of Bobath concept; and 3) use associated to two protocols. RESULTS: The hypertonia rate did not modify after protocol 1; however, protocols 2 and 3 decreased the spasticity similarly, according to Modified Ashworth Scale. The dorsiflexion angle increased after all protocols: protocol 1 (p=0.176); protocol 2 (p=0.008); and protocol 3, with the most significant increase of ROM (p=0.003). CONCLUSION: The technique to reduce tonus shows positive effects on decreasing in hypertonia rate and increase of ROM in spastic children. The subsequent execution of muscle stretching increased its effectiveness.

Treatment of severe, disabling spasticity with continuous intrathecal baclofen therapy following acquired brain injury: the experience of a tertiary institution in Singapore

<p><b>INTRODUCTION</b>Intrathecal baclofen (ITB) therapy is a proven, effective treatment for disabling cortical spasticity. We describe the first local series of five patients with acquired brain injury (ABI) who received ITB and were followed up for 63.8 months.</p><p><b>METHODS</b>A retrospective review of medical and rehabilitation records of patients who received ITB therapy was carried out. Data studied included baseline demographic and injury variables, implantation data, spasticity and function, ITB dosage over time and complications.</p><p><b>RESULTS</b>From 2006 to 2010, a total of five patients received ITB therapy via implanted pumps about 39.4 months after ABI. Four out of five patients experienced significant reductions in their lower limb spasticity scores and improvements in global function and dependency. One patient had minor adverse events associated with baclofen-related sedation. The mean ITB dose at one year was 182.7 ± 65.6 mcg/day.</p><p><b>CONCLUSION</b>Our preliminary study showed encouraging long-term outcomes and safety for ITB therapy after ABI-related intractable spasticity. Individual ITB responses over time were variable, with gender differences. The outcomes experienced by our centre were comparable to those in the general ABI population, supporting the efficacy of ITB therapy for chronic disabling spasticity.</p>

Implementation and clinical application of hypertonia assessment tool / 中华实用儿科临床杂志

As the urgent need of both clinic and research,the identification of hypertonia subtypes is becoming more and more important. Hypertonia assessment tool is a standardized discriminative measure with good reliability and validity. Hypertonia assessment tool can identify paediatric hypertonia subtypes. For its easily learning,it can be easily generalized in clinician.

Morfología de la cadera y media de contracción muscular en bipedestación en la parálisis cerebral / Morphologie de la hanche et moyenne de contraction musculaire en position bipède dans la paralysie cérébrale / Hip morphology and average muscle contraction when standing in cerebral palsy

Introducción: en la parálisis cerebral la articulación de la cadera es una de las más afectadas; sus alteraciones conllevan complicaciones importantes. Aunque su desarrollo se relaciona con trastorno del tono muscular y desequilibrio entre el grupo abductor y el adductor no existen datos objetivos que permitan cuantificar la proporción exacta entre las contracciones de ambos grupos musculares, a partir de la cual esta articulación se encuentra en riesgo. Objetivos: mostrar en una población con parálisis cerebral la media global del porcentaje de contracción de los grupos abductores (µ por ciento Abd) y adductores (µ por ciento Add) de cadera en bipedestación y compararla con los distintos grados de alteración de dicha articulación. Métodos: se midió la actividad muscular en bipedestación de los grupos abductor y adductor en 19 caderas, de 10 sujetos con parálisis cerebral utilizando un electromiógrafo de superficie. Los resultados se expresaron en porcentaje respecto a la contracción máxima isométrica obtenida previamente en los mismos grupos musculares. Las caderas se valoraron utilizando estudios radiológicos. Resultados: la media del porcentaje de contracción del grupo adductor supera a la del grupo abductor en las caderas subluxadas y luxadas. En el estudio por casos, al comparar resultados individuales con la media global para nuestra población no se consigue una asociación significativa. Conclusiones: se intuye que aunque el grupo adductor es un factor importante en el desarrollo de la patología coxofemoral, no es útil como indicador del estado articular aun utilizando medias de población con alteraciones neurológicas(AU)

Introduction: the hip joint is one of the most affected joint in cerebral palsy and its alterations are one of the most important complications. Although its development is related to muscle tone disorder and imbalance between abductor and adductor groups, there are no objective data that allow us to quantify the exact proportion between contractions of both muscle groups from which this joint is at risk. Objective: show, in a population with cerebral palsy, the mean of contraction percentage of hip abductors (µ pecent Abd) and adductors (µ percent Add) groups when standing and compare it with the different degrees of alteration of this joint. Methods: muscle activity of the abductors and adductors groups when standing was measured in 19 hips of 10 subjects with cerebral palsy using a surface electromyography. Results were expressed in percentage regarding the maximum isometric contraction previously obtained in the same muscle groups. Hips were assessed using radiological studies. Results: the mean percentage of contraction of the adductor group exceeds the adductor group in subluxated and luxated hips. In these case studies, a significant association when comparing individual results with the overall mean for our population is not achieved. Conclusions: although the adductor group is an important step in the development of hip pathology factor, it is not useful as an indicator of this joint stage, even using population means suffering neurological disorders(AU)

Introduction: l'articulation de la hanche est l'une des structures les plus souvent touchées dans la paralysie cérébrale; son altération provoque des complications importantes. Bien que son évolution soit associée à un trouble du tonus musculaire et de l'équilibre entre le groupe abducteur et le groupe adducteur, il n`y a pas de données objectives permettant de calculer la proportion exacte entre les contractions de tous ces deux groupes musculaires, à partir de laquelle cette articulation est en risque. Objectifs: le but de ce travail est de montrer la moyenne totale du pourcentage de contractions du groupe abducteur (µ pourcent Add) de la hanche en position bipède dans une population atteinte de paralysie cérébrale, et de la comparer avec les différents grades d'altération de cette articulation. Méthodes: on a mesuré l'activité musculaire en position bipède des groupes abducteur et adducteur de 10 patients (19 hanches), atteints de paralysie cérébrale, en utilisant un électromyographe de surface. Les résultats sont exprimés en pourcentage par rapport à la contraction maximale isométrique obtenue préalablement dans les mêmes groupes musculaires. On a évalué les hanches en faisant des examens radiologiques. Résultats: la moyenne du pourcentage de contraction du groupe adducteur supère celle du groupe abducteur dans les hanches subluxées et luxées. Dans l'étude des cas, on n'a pas obtenu une association significative en comparant les résultats individuels avec la moyenne totale de notre population. Conclusions: bien que le groupe adducteur soit un facteur clé dans l'évolution de la pathologie coxofémorale, il n'est pas utile comme indicateur de l'état articulaire, même en utilisant des moyennes de populations atteintes d'altérations neurologiques.

Anesthetic management in pediatric patient with Angelman syndrome: A case report / 대한마취과학회지

The Angelman syndrome is characterized by an abnormality of chromosome 15, where a subunit of the gamma amino-butyric acid receptor is coded. The clinical features are developmental delay, microcephaly, wide mouth, prognathia which usually do not have problem with intubation. But, muscular atrophy may induce delayed recovery from neuromuscular blockade. Moreover, there are case reports that vagal hypertonia such as severe bardycardia or asystole occurred during anesthesia. We present a 5-year-9-month-old male Angelman syndrome patient who underwent a left and right rectus ophthalmicus muscle recession. We gave him prophylactic glycopyrrolate before anesthetic induction and induced and maintained anesthesia with sevoflurane and oxygen. After that we monitored train-of-four ratio for evaluation of neuromuscular blockade. There is no complication during operation and recovery from anesthesia.

Intercellular adhesion molecule-1 polymorphisms in Korean patients with Behcet's disease

Intercellular adhesion molecule-1 (ICAM-1) is expressed on vascular endothelial cells and its expression increases during the inflammatory response in patients with active Behcet's disease (BD). The ICAM1 gene mutations are associated with BD in Caucasians, but clinical features of the mutation phenotype are unknown. We analyzed ICAM1 polymorphisms in Korean BD patients to determine if there was an association between particular mutations and clinical symptoms. The prevalence of ICAM1R241G and ICAM1K469E polymorphisms was determined among 197 patients with BD and 248 healthy controls using BsrG1 and BstU1 PCR-RFLP. The frequency of both genotypes ICAM1469 * K/ * E and ICAM-1469 * E/ * E was significantly higher in BD patients compared with controls (66.0% vs 52.4%, p=0.004, OR=1.28, 95% CI 1.08-1.50) and the allele frequency of ICAM1469 * E was higher in patients with skin lesions (0.41), genital ulcers (0.41), vasculitis (0.43), ocular lesions (0.41) and arthritis (0.39) than in controls (0.31). Only one heterozygote, ICAM1241G/R, was detected in BD patients but the ICAM1241 * R mutation was not found in any of the 248 healthy controls. These results show that the ICAM1 mutation is associated with BD susceptibility, and is another genetic risk factor for BD among the Korean population.

A Case Report of Congenital Hyperekplexia in Twin

Hyperekplexia or startle disease is a hereditary neurological disorder characterized by an abnormally exaggerated startle response to tactile, auditory and visual stimuli, together with a global muscular hypertonia and hyperactive tendon reflexes. This disease is a rare, genetically determined disorder, with an autosomal dominant inheritance with variable expression, first described by Suhren, et al. We report two cases of familial hyperekplexia, who developed hypertonia and pathologic startle response to tactile stimulation in the immediate neonatal period. The infant showed a marked improvement of the startle response and muscular hypertonia with low-dose clobazam.

Effect of Shujin Granules on Rats with Muscular Hypertonia After Stroke / 广州中医药大学学报

0.05).Muscular tension improvement in SG group and baclofen group differed from that in the model group(P0.05).GABA expression in the spinal dorsal horn was decreased in SG group and baclofen group as compared with the model group(P0.05).Conclusion The therapeutic mechanism of SG for the treatment of muscular hypertonia after stroke is probably related with the spinal expression of GABA.

The Effect of Hot Bath and Infrared Radiation on Hypertomia in the Affected Upper Extremity of Hemiplegic Patients. Changes in surface electromyogram / 日本温泉気候物理医学会雑誌

We investigated the effect of hot bath and infrared radiation on tonic muscle discharges due to hypertonia in the affected upper extremity of hemiplegic patients using surface electromyography (EMG). Subjects were 15 hemiplegic patients with cerebrovascular diseases. The subjects were selected on the basis of the presence of considerable rigidospasticity in the biceps brachii muscle (BBM). Hot bath (42°C) was applied for more than 3 minutes in the supine position with the cubital joint fixed in flexion in 11 subjects. Infrared radiation (300 watts) was applied to the flexor side of the affected upper extremity in the same posture in 13 subjects. Surface EMGs of the BBM and triceps brachii muscle (TBM) were recorded bipolarly with waterproof disc electrodes before, during, and after the trials of hot bath and infrared radiation. The recorded EMG was rectified and integrated, and then converted into sequential pulses. The amount of EMG was calculated as the number of pulses. The EMG of the affected side was normalized as a percentage of the amount versus that of the unaffected side in maximum voluntary contraction. The EMGs of the BBM and TBM showed tonic muscle discharges in all subjects in the resting state. The EMGs of the BBM and TBM averaged 6.4% and 1.4% before hot bath and 3.3% and 1.2% before infrared radiation. The EMG of the BBM in the third 1 minute during hot bath decreased by 58.8% (p<0.01) and increased slightly after the trial. The EMG of the TBM during hot bath increased insignificantly, and decreased by 25.3% (p<0.05) in the second 1 minute after the trial. The EMGs of the BBM and TBM in the third 1 minute during infrared radiation decreased by 31.9% (p<0.01) and 9.3% (p<0.05), and the decrease persisted after the trial. The results demonstrated that thermotherapy, especially hot bath, decreases the tonic muscle discharges due to rigidospasticity.