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BACKGROUND: Benign essential blepharospasm (BEB) is the most common adult-onset focal facial dystonia and its treatment of choice is periodic application of botulinum toxin (BtA). It has a higher incidence in middle and late adulthood, especially in women between 40 and 60 years of age. OBJECTIVE: To carry out the translation and cross-cultural adaptation of the CDQ24 questionnaire in its Spanish version in patients diagnosed with BEB who have been treated with BtA in an ophthalmologic center in Bogotá - Colombia. MATERIALS AND METHODS: Pilot test of validation study and adaptation of a scale assembled in a prospective cohort of the CDQ24 instrument to Spanish in adult patients with primary blepharospasm treated with botulinum toxin in Bogota, Colombia. RESULTS: We obtained a sample of 26 patients to whom the instrument was applied after translation and retranslation of the original document, composed of 19 (73%) women with a median age of 64.5 years; the average time to answer the survey was 4.93 min. The internal consistency of the scale evaluated by Cronbach's Alpha had a total score of 0.78. Criterion validity between the CDQ24 scale and the WHOQOL-BREF quality of life scale was determined by determining correlation between the Emotional Well-Being and Phsychological domains of both scales. CONCLUSIONS: The translation and cross-cultural adaptation of the CDQ-24 scale into Spanish allowed the applicability of the instrument to the Spanish-speaking population during the pilot test, which allows us to continue the relevant studies in the study population.

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RESUMEN Introducción: Las secuelas de la tortícolis muscular congénita en niños tiene un amplio espectro, algunas de las cuales, una vez establecidas pueden requerir complejas y costosas correcciones quirúrgicas. Objetivo: Caracterizar las secuelas de tortícolis muscular congénita según elementos clínicos, radiológicos y epidemiológicos en niños atendidos en Santiago de Cuba en el periodo 2017-2020. Método: Estudio prospectivo-analítico de caso-control en 112 niños con dicha enfermedad, separados en casos (24 niños) y controles (88 niños). El procesamiento de datos implicó análisis de frecuencias, cálculo de Ji-cuadrado, identificación de factores asociados a variable dependiente, cálculo del valor de p y Odds ratio. Resultados: Se obtuvo predominio de población masculina en casos (70,8 %) y controles (68,2 %), con asociación estadística entre edad ≥ 6 meses al momento del diagnóstico y presencia de complicaciones (OR: 2,4-20,4; p=0,00). Existió asociación estadística entre macrosomía al nacer y presencia de complicaciones en 25,0 % de casos y 11,4 % de controles (OR: 1,9-12,5; p=0,02). Hubo asociación estadística entre inicio tardío del tratamiento rehabilitador y complicaciones (OR: 2,86-21,3; p=0,00). Conclusiones: Se observó predominio de complicaciones orgánicas o comorbilidades en varones, siendo más probable el incremento significativo de secuelas en estos y cuando se inicia el tratamiento médico después de los 6 meses de edad. Las complicaciones más frecuentes fueron: asimetría facial, plagiocefalia y asociación de dos o más secuelas.

ABSTRACT Introduction: Sequelae of congenital muscular torticollis in children have a wide spectrum, some of which, set already, may require complex and costly surgical corrections. Objective: To characterize the sequelae of congenital muscular torticollis according to clinical, radiological, and epidemiological elements in children attended in Santiago de Cuba from 2017 to 2020. Method: Prospective-analytical case-control study in 112 children with this disease, separated in cases (24 children) and controls (88 children). Data processing involved frequency analysis, calculation of chi-square, identification of factors associated with dependent variable, calculation of the p-value and OR. Results: The male population predominated in cases (70.8%) and controls (68.2%), with a statistical association between age ≥ 6 months at diagnosis and the presence of complications (OR: 2.4-20.4; p=0.00). There was a statistical association between macrosomia at birth and the presence of complications in 25.0 % of cases and 11.4 % of controls (OR: 1.9-12.5; p=0.02). There was a statistical association between late initiation of rehabilitation treatment and complications (OR: 2.86-21.3; p=0.00). Conclusions: A predominance of organic complications or comorbidities was observed in males, with a significant increase of sequelae in this group and also when medical treatment is started after 6 months of age. The most frequent complications were facial asymmetry, plagiocephaly and association of two or more sequelae.

RESUMO Introdução: As sequelas do torcicolo muscular congênito em crianças têm amplo espectro, algumas das quais, uma vez estabelecidas, podem exigir correções cirúrgicas complexas e onerosas. Objetivo: Caracterizar as sequelas do torcicolo muscular congênito segundo elementos clínicos, radiológicos e epidemiológicos em crianças atendidas em Santiago de Cuba no período 2017-2020. Método: Estudo prospectivo-analítico caso-controle em 112 crianças com essa doença, separadas em casos (24 crianças) e controles (88 crianças). O processamento dos dados envolveu análise de frequência, cálculo do Qui-quadrado, identificação dos fatores associados à variável dependente, cálculo do valor de p e Odss ratio. Resultados: Obteve-se predominância da população masculina nos casos (70,8%) e controles (68,2%), com associação estatística entre idade ≥ 6 meses no momento do diagnóstico e presença de complicações (OR: 2,4-20,4; p=0,00). Houve associação estatística entre macrossomia ao nascimento e presença de complicações em 25,0% dos casos e 11,4% dos controles (OR: 1,9-12,5; p=0,02). Houve associação estatística entre início tardio do tratamento de reabilitação e complicações (OR: 2,86-21,3; p=0,00). Conclusões: Observou-se predominância de complicações orgânicas ou comorbidades no sexo masculino, com aumento significativo de sequelas sendo mais provável nestes e quando o tratamento médico é iniciado após os 6 meses de idade. As complicações mais frequentes foram: assimetria facial, plagiocefalia e associação de duas ou mais sequelas.

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The real-world use of onabotulinumtoxinA and incobotulinumtoxinA for cervical dystonia and blepharospasm treatment was assessed in two separate retrospective studies using identical protocols (TRUDOSE and TRUDOSE II). The studies were conducted in Mexico, Norway, and United Kingdom and designed to evaluate dose utilization of the two botulinum toxins in clinical practice. Eighty-three patients treated with both onabotulinumtoxinA and incobotulinumtoxinA for ≥2 years for each botulinum toxin were included, (52, cervical dystonia; 31, blepharospasm). All patients switched from onabotulinumtoxinA to incobotulinumtoxinA for administrative/financial reasons. A range of dose ratios (incobotulinumtoxinA to onabotulinumtoxinA) was reported; with the majority of dose ratios being >1. The mean dose ratio was >1 regardless of the study site or underlying clinical condition. The inter-injection interval was significantly longer for onabotulinumtoxinA versus incobotulinumtoxinA when assessed for all patients (15.5 vs. 14.3 weeks; p = 0.006), resulting in fewer onabotulinumtoxinA treatments over the study time period. Consistent with product labeling, no single fixed-dose ratio exists between incobotulinumtoxinA and onabotulinumtoxinA. The dosage of each should be individualized based on patient needs and used as per product labeling. These real-world utilization data may have pharmacoeconomic implications.

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Botulinum toxin-A (BoNT-A) is an effective treatment for cervical dystonia (CD) and spastic paresis (SP), but it requires in-depth knowledge of anatomy and injection techniques. The Ixcellence Network® is an educational programme to provide neurology, neuropaediatrics, and physical medicine and rehabilitation (PMR) specialists with access to best clinical practices and innovations regarding SP and CD management with BoNT-A. To assess the benefits of such educational programmes and identify unmet needs, a multidisciplinary scientific committee designed INPUT (INjection Practice, Usage & Training), an international multicentric survey describing training and practices among this trained and experienced population. A self-completed questionnaire was sent online to 553 trainees and 14 trainers from the Ixcellence Network®. Among the 131 respondents, 92% specialized in PMR (48%) or neurology (44%), with a mean experience of 15.5 years in their clinical fields and 10.9 years of BoNT-A injection. Most of them (98%) reported having received training before performing their first BoNT-A injection and attending specific courses on how to perform it without any instrumental guidance (76%), and with ultrasound (73%), electrical stimulation (44%) or electromyography (41%). In terms of practices, 92% of respondents reported using at least one guidance technique while injecting, with ultrasound being the most used technique (48%). Attending specific courses was significantly associated with greater self-confidence and use, e.g. for injection with ultrasound, mean self-confidence, on a scale from 1 (not confident) to 10 (fully confident), was 7.9 for trained respondents (vs 4.0 for untrained respondents, p < 0.001) of whom 70% stated that they used this technique regularly or systematically (vs. 11% of untrained healthcare professionals (HCPs), p < 0.0001). Moreover, 84% of respondents reported having trained colleagues, residents or fellows through theoretical (70%) or practical teaching in individuals (80%) or in small groups (65%). Overall, 86% of respondents reported a notable increase over the past 5 years of the number of patients treated with BoNT-A. INPUT is the first international survey describing training and practices in SP and CD management of physicians who attended a dedicated educational programme. The results highlighted the importance of training for self-confidence, and the use of specific techniques and new approaches.

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Chronic nonunion cervical fracture leading to cervical dystonia (CD) is very rare. This study reports a 72-year-old man who presented with 9-month history of progressively worsening neck tilting, neck tightness, neck pain, headache, and difficulty with swallowing. The patient was referred to speech therapy and confirmed to have dysphagia on modified barium swallow study. A cervical spine radiograph showed a chronic C2 nonunion fracture. Subsequent cervical spine magnetic resonance imaging confirmed chronic C2 nonunion fracture with kyphotic deformity of the cervical canal with associated cord compression at C1-C2 and severe central canal stenosis. Needle electromyography revealed dystonic or spasmodic neck muscles, consistent with diagnosis of CD. Botulinum toxin injection resulted in marked clinical improvement. The patient finally underwent occipital to C4 posterior segmental fusion. No recurrence of CD had occurred 12 months after botulinum toxin injection and surgery, which supports the conclusion that chronic C2 nonunion fracture is most likely responsible for CD in this case. The authors suggest that all patients with CD receive dysphagia evaluation and more importantly cervical spine imaging to rule out chronic C2 nonunion fracture.

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BACKGROUND: Craniocervical dystonia is a focal or segmental dystonia in its distribution, classically known as spasmodic torticollis when in its pure cervical presentation. Although craniocervical dystonia has been recognized as a possible cause of headache since the publication of the second version of International Classification of Headache Disorders, there are few studies about this entity. METHOD: This was a narrative review. RESULTS: Craniocervical dystonia was associated with muscle pain in 67-89% of the cases. Headaches of any kind affected approximately 60% of patients with craniocervical dystonia, and were located mainly in the occipital and cervical regions. Headache attributed to craniocervical dystonia specifically was rarely found, and it was described in only one patient out of 80 in one study. Treatment with botulinum neurotoxin is considered to be the first-line treatment for focal dystonias, including craniocervical dystonia, and besides reducing clinical severity, impairment, and pain scores among the patients with craniocervical dystonia, there were also descriptions of improvements in headaches attributed to craniocervical dystonia and other headaches associated with this dystonia. CONCLUSIONS: Headache attributed to craniocervical dystonia has been poorly studied. There is a need for more studies to evaluate its characteristics and treatment.

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Bilateral thalamic infarctions are usually caused by occlusion of the "Artery of Percheron" (AoP). Thalamopeduncular syndrome is among the most common presentations of AoP occlusion. A 59-year-old male presented abrupt decreased level of consciousness. After several weeks, on regaining consciousness, he exhibited oculomotor abnormalities, ataxic gait, cervical dystonia, and cognitive and behavioral changes. Magnetic resonance imaging disclosed thalamic, subthalamic, mammillary and midbrain infarction. Clinical features suggestive of bilateral thalamopeduncular syndrome were identified. Besides the presence of cognitive impairment and behavioral symptoms, cervical dystonia was evident, possibly resulting from interruption of the interconnections among basal ganglia, thalamus, subthalamus, midbrain and cerebellum.

Infartos talâmicos bilaterais são em geral ocasionados por oclusão da "Artéria de Percheron" (AdP). A síndrome talamopeduncular está entre as apresentações clínicas mais comuns da oclusão da AdP. Um homem de 59 anos apresentou rebaixamento abrupto do nível de consciência. Após algumas semanas, ao recobrar a consciência, apresentava anormalidades oculomotoras, marcha atáxica, distonia cervical e alterações cognitivas e comportamentais. A imagem por ressonância magnética evidenciou infartos talâmico, subtalâmico, mamilar e mesencefálico. O quadro clínico foi sugestivo de síndrome talamopeduncular. Além da presença de comprometimento cognitivo e transtornos de comportamento, estava presente distonia cervical, que pode resultar da interrupção das interconexões entre gânglios da base, tálamo, subtálamo, mesencéfalo e cerebelo.

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Dystonia is a neurological disorder characterized by intermittent or sustained muscle contractions that cause abnormal, usually repetitive, movements and postures. Dystonic movements can be tremulous and twisting and often follow a pattern. They are frequently associated with overflow muscle activation and may be triggered or worsened by voluntary action. Most voluntary muscles can be affected and, in the case of the neck muscles, the condition is referred to as cervical dystonia (CD), the most common form of dystonia. The high incidence of pain distinguishes CD from other focal dystonias and contributes significantly to patient disability and low quality of life. Different degrees of pain in the cervical region are reported by more than 60% of patients, and pain intensity is directly related to disease severity. Botulinum toxin (BoNT) is currently considered the treatment of choice for CD and can lead to an improvement in pain and dystonic symptoms in up to 90% of patients. The results for BoNT/A and BoNT/B are similar. The complex relationship between pain and dystonia has resulted in a large number of studies and more comprehensive assessments of dystonic patients. When planning the application of BoNT, pain should be a key factor in the choice of muscles and doses. In conclusion, BoNT is highly effective in controlling pain, and its analgesic effect is sustained for a long time in most CD patients.

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La distonía cervical es la forma más común de distonía focal. La aplicación de toxina botulínica a través de la quimiodenervación produce parálisis muscular temporal y de forma reversible. Se presenta un caso clínico relacionado con la distonía cervical la aplicación terapéutica de toxina botulínica y la producción de disfagia secundariamente.

Cervical dystonia is the most common form of focal dystonia. The application of botulinum toxin through chemodenervation produces temporary muscular paralysis and reversibly. A case associated with cervical dystonia therapeutic application of botulinum toxin and dysphagia secondary production is presented.

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