Facial Clinimetric Evaluation Scale and Synkinesis Assessment Questionnaire Translation into Brazilian Portuguese: A Validation Study

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.

Trochlear-oculomotor synkinesis: another congenital cranial dysinnervation disorder? / Sincinesia troclear-oculomotora: outro distúrbio congênito de denervação craniana?

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.

Regeneración aberrante del III nervio craneal por aneurisma cerebral bilateral. Reporte de caso / Aberrant regeneration of the III cranial nerve due to bilateral cerebral aneurism

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.

The Contribution of Surface Electromyographic Assessment for Defining the Stage of Peripheral Facial Paralysis: Flaccid or Sequelae Stage

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.

Differential motor alterations in children with three types of attention deficit hyperactivity disorder / Alteraciones motoras diferenciales en niños con tres tipos de trastorno por déficit de atención-hiperactividad

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. .

Evolución de los signos cerebelosos, la paratonía y las sincinesias en el niño / Evolution of cerebellar signs, the paratonia and the child synkinesias

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.

Persistencia de movimientos en espejo congénitos en una hemiplejía por infarto cerebral / Persistance of congenital mirror movements in the hemiplejic side after ischemic stroke

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.

Grau de percepção e incômodo quanto à condição facial em indivíduos com paralisia facial periférica na fase de seqüelas / Degree of perception and discomfort regarding facial condition in subjects with peripheral facial paralysis in sequelae stage

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.