RESUMEN
Abstract Introduction Facial nerve palsy results in both functional disability and psychological morbidity. There are several well-established grading scales to quantify the quality of life of these patients. Objective Translate and validate the Facial Clinimetric Evaluation (FaCE) scale and Synkinesis Assessment Questionnaire (SAQ) to Brazilian Portuguese. Methods This study adopted a forward-backward translation method and performed cross-cultural adaptation. A pilot study was conducted to correct any confusing language and to evaluate content validity. A validation study was then performed. Internal consistency of the Brazilian Portuguese version of the FaCE and SAQ items was evaluated by Cronbach's alpha coefficient. Construct validity was assessed by Spear- man's Rank Correlation Coefficient between FaCE and SAQ scores to eFACE, House- Brackmann, Short Form 12 (SF-12) and Facial Disability Index (FDI) (sub)scores. Results A total of 90 patients were included. Cronbach's alpha for total domain scored 0.881 for FaCE and 0.809 for SAQ. FaCE total score correlation to eFACE total and House- Brackmann showed Spearman's r value of 0.537 and -0.538, respectively (p < 0.001). SAQ correlation to eFACE synkinesis subdomain was -0.449 (p < 0.001). No correlation was found between SAQ and HB score. FaCE total score correlations were of 0.301 and 0.547 for SF-12 PCS and MCS, respectively (p < 0.001). Correlation between FaCE total and FDI Physical and Social/well-being functions were 0.498 and 0.567 (p < 0.001). Conclusion Brazilian Portuguese FaCE scale and SAQ versions achieved high validity and reliability in the present study. These translated instruments demonstrated good psychometric properties, being proper to use in clinical practice in Brazil and with Brazilian Portuguese speakers.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Traducción , Encuestas y Cuestionarios , Sincinesia , Parálisis Facial , Calidad de Vida , Índice de Severidad de la Enfermedad , Brasil , Proyectos Piloto , Estudios Prospectivos , Reproducibilidad de los Resultados , Evaluación de la DiscapacidadRESUMEN
ABSTRACT This report documents an unusual phenomenon. A 6-year-old girl with trochlear-oculomotor synkinesis presented with superior oblique and palpebral levator co-contraction. The literature was reviewed and the possibility of classifying this entity as a congenital cranial dysinnervation disorder was speculated.
RESUMO Este relato descreve um fenômeno incomum. Uma menina de 6 anos com sincinesia troclear-oculomotora apresentou co-contração do oblíquo superior e do levantador da pálpebra. A literatura foi revisada e especulou-se a possibilidade de classificar essa desordem como um distúrbio da congenital cranial dysinnervation disorder.
Asunto(s)
Humanos , Femenino , Niño , Trastornos de la Motilidad Ocular/congénito , Nervios Craneales/anomalías , Enfermedades del Nervio Troclear/congénito , Sincinesia/congénito , Músculos Oculomotores/inervación , Trastornos de la Motilidad Ocular/clasificación , Trastornos de la Motilidad Ocular/patología , Enfermedades del Nervio Troclear/clasificación , Enfermedades del Nervio Troclear/patología , Enfermedades Raras , Sincinesia/clasificación , Sincinesia/patología , Párpados/anomalíasRESUMEN
RESUMEN Los trastornos de la motilidad ocular constituyen motivo de consulta periódica en Oftalmología. La regeneración aberrante, trastorno muy poco reportado, es considerada la sincinesia oculomotora de mayor invalidez y complejidad. Diversas condiciones neuroftalmológicas están implicadas en la etiopatogenia de la enfermedad, la mayoría de las cuales puede ocasionar la muerte. El manejo de los síntomas y signos provocados por paradójicos movimientos oculares conjugados es difícil. Se reportó un caso con remisión tardía a neuroftalmología por diagnóstico inicial y evolución desfavorable. La historia psicofísica arrojó diagnóstico definitivo de regeneración aberrante del III nervio craneal secundario, a aneurisma cerebral de la carótida interna bilateral, agravado por reanastomosis quirúrgica. Una rigurosa, obligatoria e impostergable historia neuroftalmológica, se impone ante toda parálisis del III nervio craneal para brindar un diagnóstico etiológico preciso y de esta forma proteger la vida.
ABSTRACT The disturbances in ocular motility are the cause of periodical consultation in Ophthalmology. The aberrant regeneration, a scarcely reported disturbance, is considered the oculomotor synkinesis of highest disability and complexness. Several neuro-ophthalmologic conditions are implicated in the disease ethiopathogeny, and most of them could lead to death. The management of the symptoms and signs caused by paradoxical conjugated ocular movements is difficult. A case is reported of late remission to Neuro-ophthalmology due to unfavorable diagnosis and evolution. The psycho-physical history led to a definitive diagnosis of aberrant regeneration of the III secondary cranial nerve, to cerebral aneurism of the bilateral internal carotid, worsened by surgical re-anastomosis. In front of any paralysis of the III cranial nerve, it is necessary a rigorous, obligatory and immediate neuro-ophthalmological history to arrive to a precise etiological diagnosis, protecting life in that way.
Asunto(s)
Humanos , Femenino , Anciano de 80 o más Años , Imagen por Resonancia Magnética/métodos , Trastornos de la Motilidad Ocular/diagnóstico , Enfermedades del Nervio Oculomotor/diagnóstico , Enfermedades del Nervio Oculomotor/diagnóstico por imagen , Sincinesia/diagnóstico , Diplopía/diagnósticoRESUMEN
Most studies concerning congenital mirror movements (CMMs) have been focused on the motor organization in the distal hand muscles exclusively. To the best of our knowledge, there is no data on motor organization pattern of lower extremities, and a scarcity of data on the significance of forearm and arm muscles in CMMs. Here, we describe the case of a 19-year-old boy presenting mirror movements. In these terms, a 10-year transcranial magnetic stimulation study demonstrated that the motor organization pattern of the arm muscles was different from that of distal hand and forearm muscles even in the same upper extremity, and that the lower extremities showed the same pathways as healthy children. Moreover, in this case, an ipsilateral motor evoked potentials (MEPs) for distal hand muscles increased in amplitude with age, even though the intensity of mirror movements decreased. In the arm muscles, however, it was concluded that the contralateral MEPs increased in amplitude with age.
Asunto(s)
Niño , Humanos , Masculino , Adulto Joven , Brazo , Potenciales Evocados , Potenciales Evocados Motores , Estudios de Seguimiento , Antebrazo , Mano , Extremidad Inferior , Músculos , Tractos Piramidales , Sincinesia , Estimulación Magnética Transcraneal , Extremidad SuperiorRESUMEN
Abstract Introduction Surface electromyographic activity may not be symmetric, even in subjects with no facial paralysis history. Objective To evaluate the contribution of the index of electromyographic (IEMG) activity in the identification of the two extremes of the facial paralysis course. Methods Thirty-four subjects with unilateral peripheral facial paralysis were selected. A control group was composed of volunteers without a history of facial paralysis. The electromyographic assessment of the facial muscle was performed by placing surface electrodes during movements of the forehead, eyes and lips using MIOTEC equipment, such as the MIOTOOL (Miotec, Porto Alegre, Brazil) software. The electromyographic activity was also recorded in other channels during the primary activity to identify the presence of synkinesis. The statistical analysis was performed using the Statistical Package for Social Sciences for Macintosh (SPSS Inc, Chicago, IL, USA). The IEMG activity was obtained from the division of the electromyographic activity root mean square (RMS) values on both sides. Results There was a statistically significant difference among the groups in all the analyzed indexes. The ocular-oral synkinesis in all patients must be correctly identified (with 100% sensitivity and specificity) using an IEMG activity of 1.62 as a cutoff point. The oral-ocular synkinesis must be correctly identified (93.3% sensitivity and 95.9% specificity) using the IEMG activity of 1.79 as a cutoff point. Conclusion The IEMG activity is below the normal scores in patients in the flaccid stage, whereas patients in the sequelae stage can either show normal values or values above or below the normal scores. The IEMG activity was shown to have high sensitivity and specificity in the identification of synkinesis.
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Anciano , Electromiografía , Parálisis Facial/diagnóstico , Parálisis Facial/fisiopatología , Superficie Corporal , Evolución Clínica , Sincinesia/diagnóstico , Músculos Faciales/fisiopatología , Parálisis Facial/complicaciones , Hipotonía Muscular/fisiopatologíaRESUMEN
Vocal fold paralysis (VFP) refers to neurological causes of reduced or absent movement of one or both vocal folds. Bilateral VFP (BVFP) is characterized by inspiratory dyspnea due to narrowing of the airway at the glottic level with both vocal folds assuming a paramedian position. The primary objective of intervention for BVFP is to relieve patients’ dyspnea. Common clinical options for management include tracheostomy, arytenoidectomy and cordotomy. Other options that have been used with varying success include reinnervation techniques and botulinum toxin (Botox) injections into the vocal fold adductors. More recently, research has focused on neuromodulation, laryngeal pacing, gene therapy, and stem cell therapy. These newer approaches have the potential advantage of avoiding damage to the voicing mechanism of the larynx with an added goal of restoring some physiologic movement of the affected vocal folds. However, clinical data are scarce for these new treatment options (i.e., reinnervation and pacing), so more investigative work is needed. These areas of research are expected to provide dramatic improvements in the treatment of BVFP.
Asunto(s)
Toxinas Botulínicas , Cordotomía , Disnea , Terapia por Estimulación Eléctrica , Terapia Genética , Laringe , Parálisis , Traumatismos del Nervio Laríngeo Recurrente , Literatura de Revisión como Asunto , Células Madre , Sincinesia , Traqueostomía , Parálisis de los Pliegues Vocales , Pliegues VocalesRESUMEN
Objective To determine frequency of motor alterations in children with attention deficit hyperactivity disorder (ADHD). Method We evaluated 19 children aged 7-12 years with ADHD classified in three sub-types: Combined (ADHD-C), with Inattention (ADHD-I), and with Hyperactivity (ADHD-H). Controls were age- and gender matched healthy children. We utilized Bruininks-Oseretsky Test of Motor Proficiency (BOTMP) for measuring motor skills. Results We observed differences between children with ADHD and controls in BOTMP general score and in static coordination, dynamic general- and hand- coordination, and in synkinetic movements. We also found differences in dynamic hand coordination between controls and children with ADHD-C; in dynamic general coordination between controls and children with ADHD-H; and in frequency of synkinetic movements between controls and children with ADHD-H. Conclusion Children with ADHD with a major degree of hyperactivity showed greater frequency of motor alterations. .
Objetivo Determinar la frecuencia de alteraciones motoras en niños con trastorno por déficit de atención-hiperactividad (TDAH). Método Evaluamos 19 niños de 7-12 años de edad con TDAH clasificados en tres subtipos: Combinado (TDAH-C), con Inatención (TDAH-I) y con Hiperactividad (TDAH-H). Los controles fueron niños sanos pareados por edad y género. Utilizamos la Prueba de eficiencia motora de Bruininks-Oseretsky (PEMBO). Resultados Observamos diferencias entre los grupos de niños con TDAH en la calificación general de la PEMBO y en: coordinación estática, coordinación dinámica general, coordinación manual y en movimientos sincinéticos. También encontramos diferencias en la coordinación manual entre los controles y los niños con TDAH-C; en la coordinación dinámica general entre los controles y los niños con TDAH-H; y en la frecuencia de movimientos sincinéticos entre los controles y los niños con TDAH-H. Conclusión Los niños con TDAH con una mayor grado de hiperactividad muestran una mayor frecuencia de alteraciones motoras y movimientos sincinéticos. .
Asunto(s)
Niño , Femenino , Humanos , Masculino , Trastorno por Déficit de Atención con Hiperactividad/fisiopatología , Trastornos de la Destreza Motora/fisiopatología , Análisis de Varianza , Estudios de Casos y Controles , Pruebas de Inteligencia , Destreza Motora/fisiología , Pruebas Neuropsicológicas , Valores de Referencia , Encuestas y Cuestionarios , Sincinesia/fisiopatologíaRESUMEN
The facial nerve coursing through the temporal bone provides a challenge to the otologic surgeon. Advances in surgical instrumentation and refinements of surgical strategies enable the otologist to uncover the entire course of the facial nerve safely from brainstem to its exit from temporal bone. The most common cause of facial nerve paralysis is Bell's palsy, followed by traumatic facial paralysis, herpes zoster oticus, and intratemporal tumorous lesion. The surgical approaches to the injured facial nerve depend on its causes. In consideration of selecting surgical technique of facial nerve paralysis reconstruction, clinician must find out thecause, degree and duration of paralysis for the appropriate technique. Although preventing synkinesis is more effective than treating an established one, it has been shown that when patients inevitably has facial sequelae, there is no effective intervention modalities. Numerous facial rehabilitation techniques for such facial problems after facial palsy have been developed to improve cosmesis and function. Recently botulinum toxin A chemical neurectomy has been considered as a best approach in treating facial sequelae. Botulinum toxin injection has shown remarkable results in the disappearance of facial synkinesis within a few days. This procedure helps the patients to recover from lower self-esteem and better quality of life than before.
Asunto(s)
Humanos , Parálisis de Bell , Toxinas Botulínicas , Tronco Encefálico , Nervio Facial , Parálisis Facial , Herpes Zóster Ótico , Parálisis , Calidad de Vida , Regeneración , Instrumentos Quirúrgicos , Sincinesia , Hueso TemporalRESUMEN
Objetivo: estudiar a través del examen neurológico evolutivo, la maduración de los signos cerebelosos, la paratonía y las sincinecias en el niño. Método: se realizó un estudio de tipo transversal, examinándose niños de 4 a 12 años de edad, de ambos sexos, de escuelas públicas de Montevideo, entre octubre 2006-mayo 2008. Resultados: se examinaron 330 niños, 178 varones y 152 niñas. La paratonía en maniobra de caída del brazo, estuvo presente en el 70% a los 4 años y en el 17% a los 11 años. A los 7 años el 100% de los niños lograron el equilibrio estático en un pie y el 92.5% el equilibrio en el salto en un pie. La marcha punta-talón la lograron el 96.2% de los niños de 6 años de edad. El 79% de los niños de 7 años lograron la metría en la maniobra índice-nariz. La diadococinesia en la maniobra palma-dorso fue lograda en el 100% de los niños de 6 años de edad y la maniobra de marionetas en el 100% de los niños de 9 años. Las sincinesias de imitación estuvieron presentes en el 80% a los 4 años y menos del 20% a los 11 años. La maniobra de oposición de dedos al pulgar la lograron el 92,5% de los niños de 8 años de edad. Conclusiones: se constata que el desarrollo motriz presenta etapas secuencialmente ordenadas, donde los cambios más significativos en la maduración de los sistemas motores ocurre entre los 5 y 6 años de edad.
Asunto(s)
Humanos , Masculino , Femenino , Preescolar , Niño , Destreza Motora/fisiología , Enfermedades Musculares/diagnóstico , Examen Neurológico/instrumentación , Sincinesia/diagnóstico , Trastornos Neurológicos de la Marcha/diagnóstico , Trastornos Psicomotores/diagnósticoRESUMEN
PURPOSE: To evaluate clinical features of periorbital spasm and facial asymmetry in the patients who recovered poorly from Bell's palsy and facial trauma and to investigate the effect of Botulinum toxin A as a treatment for periorbital spasm and facial asymmetry. METHODS: Between November 2001 and January 2010, Botulinum toxin injection was performed in 17 patients who had blepharospasm and facial asymmetry following poor recovery from facial palsy. The past history, trauma history, clinical manifestation of blepharospasm, Botulinum toxin A injection dose, injection site, frequency of injection, and duration of effect was evaluated. Data was analyzed using the Mann-Whitney U test, SPSS 12.0. RESULTS: The mean number of injections was 2.7 +/- 2.4 times and the mean dose per injection unit was 12.2 +/- 1.2 units. The Botulinum toxin effect lasted 6.9 +/- 5.5 months in Bell's palsy patients, and 8.0 +/- 4.2 months in trauma patients. There was no significant difference between the 2 groups. Most patients reported improvement of periorbital spasm and facial asymmetry. After treatment, 1 patient complained of epiphora and 1 patient complained of ptosis; conservative treatment was performed for these patients. CONCLUSIONS: Blepharospasm can be treated and a cosmetic improvement in facial symmetry can be achieved by Botulinum toxin A injection in the patients who recover poorly from facial palsy.
Asunto(s)
Humanos , Parálisis de Bell , Blefaroespasmo , Toxinas Botulínicas , Cosméticos , Asimetría Facial , Nervio Facial , Parálisis Facial , Enfermedades del Aparato Lagrimal , Paresia , Espasmo , SincinesiaRESUMEN
The simultaneous and involuntary displacement of the opposite limb during a volitional movement is called mirror movements. They mimic the gesture, partly or wholly. They can be congenital, familiar or installed in various CNS pathologies. We present a 64 years old woman with familiar history of mirror movements that affect their hands, since childhood. At admission presented confused with left hemiplegia and homolateral sensory involvement. The brain CT defined a right ischemic stroke affecting the posterior limb of the internal capsule, lenticular nucleus, sub insular region and par ventricular white matter. The paretic left hand, unable to perform voluntary movements, presented mirror movements during volitional movements of the contra lateral hand. Neurophysiologic studies have suggested that mirror movements are due the activation of the direct corticospinal pathway or simultaneous discharge of both motor cortexes due inhibitory pathways failures. Cortical origin seems unlikely for the movements in this patient, due to the injury of the internal capsule. Our case could be interpreted by the simultaneously brain innervations on both anterior horns, together with a congenital deregulation of the Central Pattern Generator Networks.
Se denomina movimiento en espejo (ME) el desplazamiento involuntario, imitativo y simultáneo de la extremidad opuesta al de un movimiento volitivo. Los ME pueden tener un origen congénito y familiar o generarse por patologías diversas del sistema nervioso central. Se presenta una mujer de 64 años con el antecedente de ME desde la infancia de carácter familiar. Ingresa en estado confusional y presentando una hemiplejía y hemihipoestesia faciobraquicrural izquierda. La tomografía cerebral mostraba compromiso del brazo posterior de la cápsula interna, núcleo lenticular, región subinsular y de la sustancia blanca paraventricular. La mano izquierda pléjica que era incapaz de realizar movimientos voluntarios, se movía en espejo al mover la mano derecha. Esta curiosa manifestación hace necesario una más ajustada interpretación neurofisiológica de los movimientos en espejo. Se ha postulado una activación de la vía corticoespinal directa, o la descarga simultánea de ambas cortezas motoras por fallas en la natural inhibición transcortical. En este caso parece improbable un origen cortical contralateral de los movimientos, debido a la lesión de la cápsula. Tal vez podrían comprenderse los ME de esta paciente, si se demostrara una doble inervación de ambas astas anteriores, asociada a una falla en la inhibición normal por desregulación congénita de los Circuitos Generadores Centrales.
Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Hemiplejía/diagnóstico , Infarto Cerebral/complicaciones , Trastornos del Movimiento/congénito , Trastornos del Movimiento/fisiopatología , Accidente Cerebrovascular/complicaciones , Lateralidad Funcional , Mano/fisiopatología , Reflejo , Sincinesia , Sistema Nervioso Central/fisiopatologíaRESUMEN
OBJETIVO: Correlacionar a auto-avaliação da condição facial do paciente, o grau de incômodo quanto às seqüelas e de prejuízo em atitudes diárias com os dados encontrados na avaliação fonoaudiológica. MÉTODOS: Participaram da pesquisa 29 indivíduos, de ambos os sexos, média de idade de 46 anos, com média de 5,9 sessões de fonoterapia. Realizou-se avaliação fonoaudiológica da simetria e da movimentação da face e verificou-se a presença de sincinesias e contraturas por meio de instrumento publicado e padronizado. Além disso, realizou-se uma entrevista por meio de perguntas fechadas que permitiram a graduação da opinião do paciente quanto à sua própria face e influência desse problema em suas atividades sociais e profissionais. RESULTADOS: Encontrou-se concordância quanto à auto-avaliação do paciente e a avaliação fonoaudiológica (p=0,0029), porém essa correlação não esteve presente em pacientes com menos de três sessões de fonoterapia. Não houve correlação significante entre o grau de sincinesia/contratura e o grau de incômodo referido pelo paciente, assim como o prejuízo em atividades sociais e profissionais não foi associado ao grau de paralisia, sincinesia, ou contratura. No entanto, houve correlação fraca com tendência à significação (r=-0,3250/p=0,085) quando comparou-se a auto-avaliação do paciente com o grau de prejuízo referido. CONCLUSÕES: A autopercepção da condição facial nem sempre é concordante entre o profissional e o paciente, sendo que essa concordância aumenta em pacientes com maior tempo de terapia. Por outro lado, o impacto da condição facial na vida do paciente parece não depender do grau das seqüelas.
PURPOSE: To correlate the self-evaluation of patients with facial paralysis regarding their facial condition, the level of sequelae discomfort and the negative consequences in daily activities, with the results found in clinical assessment. METHODS: Twenty-nine subjects of both genders, with average age of 46 years and an average of 5.9 therapy sessions participated in this study. A clinical evaluation of facial symmetry and movement was carried out, verifying the presence of synkinesis and contractures using a facial grading system proposed by a standardized and published instrument. Moreover, an interview with closed questions was carried out, in order to measure the patient's opinion regarding how his own face influences his professional and social activities. RESULTS: There was an accordance regarding the patient's self evaluation and the clinical assessment (p=0,0029), but this correlation was not found in patients with less than three sessions of the speech therapy. No significant correlation was found between the degree of synkinesis/contracture and the level of discomfort reported by the patient. Furthermore, the negative consequences in professional and social activities were not associated to the degree of facial paralysis, synkinesis or contracture. However, there was a weak correlation tending to significance (r=-0,3250/p=0,085) when the self evaluation was compared to the reported negative consequences. CONCLUSIONS: The patient's self perception of the facial condition is not always in agreement with the professional's assessment, although this agreement increases in patients with longer intervention periods. On the other hand, the impact of the facial condition in the patient's life does not depend on the degree of sequelae.
Asunto(s)
Humanos , Autoevaluación (Psicología) , Logopedia , Parálisis Facial/terapia , Perfil de Impacto de Enfermedad , Calidad de Vida , Sincinesia/psicologíaRESUMEN
OBJECTIVE: To study electrodiagnostic findings and recovery patterns of patients with facial nerve paralysis. MATERIAL AND METHOD: Seventy-six medical records of patients who had a facial paralysis and had their electrodiagnosis performed in a 2-year period were reviewed. Patients were invited for re-evaluation. The patients would be evaluated according to House-Brackmann Facial Nerve Grading Scale (HBFNGS), residual impairment, disability, emotional and social consequences. RESULTS: Complete data were obtained from 50 patients whose mean age was 47.0 +/- 17.9 years. Seventy-two percent were diagnosed as Bell's palsy. There was significant correlation between %CMAP amplitude and HBFNGS (grade I-VI) at r = 0.5; p < 0.01. All cases of Bell's palsy with CMAP amplitude > or = 70% of normal side regained full recovery. Patients with CMAP amplitude > or = 30% had good recovery. Bell's palsy with CMAP amplitude < 10% and with other causes had poor outcome. Nine patients had synkinesis. Most of them were of traumatic cause and had severe nerve degeneration. No evidence showed that electrical stimulation was a factor inducing synkinesis. CONCLUSION: Percent CMAP amplitude could moderately predict the outcome of Bell's palsy better than other causes of facial palsy. The paralysis from traumatic cause with low %CMAP amplitude had more chance to develop synkinesis.
Asunto(s)
Potenciales de Acción , Estudios Transversales , Electrodiagnóstico , Enfermedades del Nervio Facial , Parálisis Facial/diagnóstico , Femenino , Indicadores de Salud , Humanos , Masculino , Persona de Mediana Edad , Pruebas Neuropsicológicas , Recurrencia , Factores de Riesgo , Sincinesia , Tailandia , Insuficiencia del Tratamiento , Resultado del TratamientoRESUMEN
Asunto(s)
Humanos , Párpados , Peste , Rehabilitación , Sincinesia , Donantes de Tejidos , TrasplantesRESUMEN
Purpose: To report and discuss a new form of congenital cranial dysinnervation disorder - ptotic lid elevation during ipsilateral abduction. Ophthalmic evaluation of affected individuals in two related families and of other immediate family members. Two siblings and their cousin exhibit ptosis and abnormal synkinetic lid elevation associated with ipsilateral abduction - one sibling is bilaterally affected and the two other individuals are unilaterally affected. A third sibling has isolated bilateral congenital ptosis. A fourth demonstrates classic Duane syndrome Type I in the right eye. Other family members do not have ophthalmic abnormalities. Conclusions: A familial syndrome of ptotic lid elevation during ipsilateral abduction is described. A unifying mechanism of congenital cranial dysinnervation likely underlies phenotypes of congenital oculomotor and/or abducens nerve abnormalities with or without abnormal synkinesis
Asunto(s)
Humanos , Masculino , Femenino , Blefaroptosis , SincinesiaRESUMEN
Recovery from the laryngeal dysfunction caused by the recurrent laryngeal nerve (RLN) injury is not common. Recently, we have found that PEMS treatment improved the functional recovery rate and shortened the recovery time after RLN transection and reanastomosis in rat. In this study, we compared the morphology of RLN stumps according to their laryngeal functional status to investigate 1) the nerve morphology associated with functional recovery and 2) the possible underlying mechanism of persistent laryngeal dysfunction after RLN injury. We transected left RLN and then performed primary neurorrhaphy in Sprague-Dawley rats (n = 36). They were randomly divided into PEMS and control groups. 19 animals (10 PEMS group, 7 control group and 2 normal control animals) survived until the end of the experiment were included in the morphological analysis. Both the proximal and distal segments of reanastomosed RLN were obtained and the ultrastructural study was done using transmission electron microscope. There is no prominent morphological difference between the PEMS and control groups. In the functional recovery group, the findings suggestive of nerve regeneration were prominent both in the proximal and distal segments. Many regenerating axons were also observed in the proximal segments of RLNs in non-recovery group. But findings such as degenerating axons, infiltration of macrophage and inflammatory cells, increased collagen fibrils were frequently observed in this group. Even in the distal segments of functional non-recovery group, prominent regenerative findings were observed in 9 out of 10 (4 out of 5 PEMS and all control group animals) samples. We could not find any regenerating findings in one case of the PEMS group. Through the above results, failure of the nerve regeneration is unlikely the main cause of functional non-recovery after RLN injury in rat. Possible other causes such as synkinesis or definite but inadequate nerve regeneration should be considered and needs further investigation.
Asunto(s)
Animales , Ratas , Axones , Colágeno , Macrófagos , Regeneración Nerviosa , Ratas Sprague-Dawley , Traumatismos del Nervio Laríngeo Recurrente , Nervio Laríngeo Recurrente , SincinesiaRESUMEN
Botulinum toxin type A(BTA) is well known treatment agent in the treatment of paralytic strabismus in ophthalmological field for more than 15 years. Its therapeutic potential as temporary paralyzing agent was adopted to treat several neurologic, movement disorders. In recent years, BTA is considered as initial medical treatment option in such as blepharospasm, hemifacial spasm, spasmodic torticollis, spasmodic dysphonia. Authors applied BTA injection in cases with spasmosmodic torticollis, hemifacial spasm, facial synkinesis and experienced staisfactory result. So authors present our experience of BTA injection therapy and discuss techniques, advantages and disavantages.
Asunto(s)
Blefaroespasmo , Toxinas Botulínicas , Toxinas Botulínicas Tipo A , Disfonía , Distonía , Espasmo Hemifacial , Trastornos del Movimiento , Estrabismo , Sincinesia , TortícolisRESUMEN
Oculostapedial synkinesis following Bell's palsy, Ramsay Hunt syndrome and traumatic facial nerve paralysis is a rarely reported phenomenon. Oculostapedial synkinesis accompanying with hemifacial spasm also has been reported. We experienced a 51-year-old woman with persistent loud rumbling noise from her left ear related with voluntary left eye closure compatible with oculostapedial synkinesis after Bell's palsy. We objectively proved this oculostapedial synkinesis with impedance audiometry. The patient was successfully treated by transmeatal tenotomy of the left stapedius muscle tendon under local anesthesia.
Asunto(s)
Femenino , Humanos , Persona de Mediana Edad , Pruebas de Impedancia Acústica , Anestesia Local , Parálisis de Bell , Oído , Nervio Facial , Espasmo Hemifacial , Herpes Zóster Ótico , Ruido , Parálisis , Estapedio , Sincinesia , Tendones , TenotomíaRESUMEN
PURPOSE: This case is the first report of unilateral ptosis associated with paradoxical movement on abduction in Korean literature and we report this case with a successful result of operations. METHODS: We examined an 18-year-old male patient who had showed drooping of right eyelid since birth. The ptosis was exaggerated on abduction. He did not have any remarkable history of illness. Corrected visual acuity was 20/20 in both eyes. Ocular motility examination revealed full range of ductions and versions in all fields of gaze. On exophthalmometric examination, no difference was found in any direction of gaze between two eyes. Under the diagnosis of unilateral ptosis associated with paradoxical movement on abduction, levator resection of right upper lid and blepharoplasty of left upper lid were performed. RESULTS: During operation, we noticed unusual finding that thickened tendon sheath was covering the lateral one third of levator aponeurosis. Paradoxical eyelid movement disappeared completely after secondary operation by removal of the previously noticed tendon sheath as much as possible.