New-Onset Movement Disorders Associated with COVID-19.

Introduction: Movement disorders are increasingly described in hospitalized and milder cases of SARS-CoV-2 infection, despite a very low prevalence compared to the total patients. Methods: We reviewed the scientific literature published in English, spanning from the initial descriptions of COVID-19 until January 25, 2021, in the PubMed/MEDLINE database. Results: We identified 93 new-onset movement disorders cases (44 articles) from 200 papers screened in the database or reference lists. Myoclonus was present in 63.4% (n = 59), ataxia in 38.7% (n = 36), action/postural tremor in 10.8% (n = 10), rigid-akinetic syndrome in 5.38% (n = 5), oculomotor abnormalities in 20.4% (n = 19), catatonia in 2.1% (n = 2), dystonia in 1.1% (n = 1), chorea in 1.1% (n = 1), functional (psychogenic) movement disorders in 3.2% (n = 3) of the reported COVID-19 cases with any movement disorder. Encephalopathy was a common association (n = 37, 39.78%). Discussion: Comprehensive neurophysiological, clinical, and neuroimaging descriptions of movement disorders in the setting of SARS-CoV-2 infection are still lacking, and their pathophysiology may be related to inflammatory, postinfectious, or even indirect mechanisms not specific to SARS-CoV-2, such as ischemic-hypoxic brain insults, drug effects, sepsis, kidney failure. Cortical/subcortical myoclonus, which the cited secondary mechanisms can largely cause, seems to be the most common hyperkinetic abnormal movement, and it might occur in association with encephalopathy and ataxia. Conclusion: This brief review contributes to the clinical description of SARS-CoV-2 potential neurological manifestations, assisting clinical neurologists in identifying features of these uncommon syndromes as a part of COVID-19 symptomatology. Highlights: - Movement disorders are probably uncommon neurological manifestations in SARS-CoV-2 infection;- Myoclonus is the most reported movement disorder associated with COVID-19, its clinical complications or pharmacological management;- The pathophysiology is yet not well-understood but can include systemic inflammation, autoimmune mechanisms, or hypoxia.

Beyond tics: movement disorders in patients with Tourette syndrome.

BACKGROUND: Tics are the hallmark of Tourette syndrome (TS). However, TS patients may have a particular vulnerability to develop other movement disorders (MDs), such as dystonia, chorea, stereotypy, and other hyperkinetic disorders that may be wrongly attributed to tics. MATERIALS AND METHODS: We studied a cohort of 201 patients with motor and phonic tics associated with TS to determine if they have additional, co-existent, MDs. RESULTS: There were 67 (33.3%) patients with comorbid non-tic MDs. Phenomenology-wise, piano-playing movements resembling chorea or myoclonus, were the most common non-tic movement, observed in 11% of cases, followed by stereotypies (8.0%), tremor, dystonia and parkinsonism, 5.0% each. Drug-induced was the most common etiology (6.0%), followed by functional movement disorders (5.0%) and tardive phenomena (5.0%). No clear etiology was identified in most patients. Piano-playing movements, were associated with a younger age at onset (P = 0.004) and younger age at presentation (P < 0.001). Patients with drug-induced movements and tardive phenomena had a lower frequency of craniofacial tics. FMDs, and idiopathic MDS showed no specific associations with TS. Tic severity was not a predictor of any co-existent MD. CONCLUSION: About a third of patients with TS present with comorbid MDs which should be differentiated and distinguished from tics as their etiopathogenesis and treatment may be different.

Lamotrigine-Associated Movement Disorder: A Literature Review.

BACKGROUND: Lamotrigine (LMT) is a phenyltriazine derivative that was originally described as an antiepileptic drug. OBJECTIVE: This literature review aims to evaluate the clinical epidemiological profile, pathological mechanisms, and management of lamotrigine-associated movement disorders. METHODS: Relevant reports in six databases were identified and assessed by two reviewers without language restriction. Reports that the individuals only developed tremor or ataxia after LMT use were not included. RESULTS: In total 48 reports of 108 cases from 19 countries were assessed. The movement disorders associated with LMT found were 29 tics, 21 dyskinesias, 14 myoclonus, 13 parkinsonism, 10 dystonia, and 1 stuttering. The not clearly defined cases included 10 akathisia, 4 myoclonus, 4 cerebellar syndromes, 1 hypertonia, 1 dyskinesia, and an unknown number of dystonia cases. The mean reported age was 33.34 years (range: 1.574 years). The male was the predominant sex and the most common LMT indication was epilepsy. The mean LMT-dose at the movement disorder onset was 228 mg. The time from LMT start to the onset of movement disorder was within 6 months in 81%. The time from LMT withdrawal to complete recovery was within 1 month in 83%. The most common management was LMT withdrawal. CONCLUSIONS: In the literature, the majority of the cases did not give a clear picture of the individual, and the times of movement disorder onset and recovery are not described. We believe that before withdrawal LMT, a dose adjustment based on the benefits and adverse events with careful evaluation case-by-case can be done.

Trastorno paroxístico no epiléptico en paciente referido por sospecha de epilepsia / Paroxysmal non-epileptic events in patients referred for suspicion of epilepsy

Los Trastornos Paroxísticos No Epilépticos (TPNE), son diagnóstico diferencial de crisis epilépticas. En Chile no existen reportes de frecuencia. OBJETIVO: Determinar frecuencia de TPNE en pacientes derivados por sospecha de epilepsia a Unidad de Electroencefalografía, Hospital Roberto del Río. METODOLOGÍA: Estudio observacional, transversal. Se revisó registros clínicos de pacientes derivados por sospecha de epilepsia(2012- 2014). Inclusión: paciente con TPNE, >1 mes, sin epilepsia previa. Caracterización: sexo, edad, tipo/subtipo TPNE, comorbilidades, electroencefalograma (EEG), uso fármaco antiepiléptico (FAE). Aprobado por comité de ética. RESULTADOS: Derivados 913 pacientes por sospecha de epilepsia. 36% TPNE (2,3% con epilepsia concomitante), 22% epilepsia aislada. TPNE más frecuente: escolares (31%), adolescentes (29%), femenino (52%). 30,1% hipoxia cerebral (síncope, Espasmo Sollozo); 22,4% trastornos del comportamiento (Descontrol Episódico, Crisis Psicógena no Epiléptica). 32,8% con comorbilidades no epilépticas (Trastornos psiquiátricos/neurodesarrollo). 4,3 % recibieron FAE. CONCLUSIONES: La frecuencia de TPNE en niños/adolescentes supera a la de epilepsia. Es fundamental evaluación multidisciplinaria.

Paroxysmal non-epileptic events (PNE) are differential diagnosis of epileptic seizures. In Chile, there are no reports on its frequency. OBJECTIVE: To determine the frequency of PNE in patients referred for suspicion of epilepsy to the Electroencephalography Unit of Roberto del Río Hospital. METHODOLOGY: Observational, cross-sectional study. Clinical records of patients referred for suspicion of epilepsy (2012-2014) were reviewed. Inclusion: Patient with PNE, >1 month, without previous epilepsy. Characterization: gender, age, PNE type/subtype, comorbidities, electroencephalogram, use of antiepileptic drug (AED). Approved by the ethics committee. RESULTS: 913 patients were referred for suspected epilepsy: 36% PNE (2,3% with concomitant epilepsy), 22% isolated epilepsy. PNE were more frequent in children (31%) adolescents (29%), and in females (52%). 30.1% Cerebral hypoxia (syncope, breathholding-spells); 22,4% Behavioral disorders (Episodic loss of control, Non-Epileptic Psychogenic Seizures). 32.8% non-epileptic comorbidities (Psychiatric/ neurodevelopmental disorders). 4.3% received AEDs. CONCLUSIONS: The frequency of PNE in children and adolescents exceeds that of epilepsy. A multidisciplinary medical evaluation is of the outmost importance. Keywords: Paroxysmal Non-Epileptic Disorder, Non-epileptic episodes, Epilepsy.

Motor function in children with congenital Zika syndrome.

AIM: To evaluate gross motor function and associated factors in children with congenital Zika syndrome (CZS). METHOD: Fifty-nine children (30 males, 29 females) with CZS at a mean (SD) age of 14.7 (3.9), months (range 5-29mo) were evaluated using the Gross Motor Function Measure (GMFM) and classified according to the Gross Motor Function Classification System (GMFCS). Neurological damage was evaluated by neuroimaging. The mothers' sociodemographic characteristics and general data on the children were obtained from interviews with the mothers and from the children's medical records. Correlational and multiple regression analyses were performed to identify factors associated with these children's motor function. RESULTS: In 81% of the children, motor function impairment was severe, classified as GMFCS level V. The overall GMFM score ranged from 5 to 210 (median 18; interquartile range 11), with only four children receiving scores in the D and E dimensions. The factors found to affect motor function were the presence of severe malformations of cortical development and small head circumference at birth. INTERPRETATION: Although motor impairment may be mild in some children, it is generally severe. Severe malformations of cortical development and small head circumference at birth were factors associated with poorer motor function, reflecting the greater severity of brain damage. WHAT THIS PAPER ADDS: Motor impairment is severe in most children with congenital Zika syndrome (CZS). Motor skills are adequate or close to adequate for age in 7% of children with CZS. Severe malformations of cortical development are associated with poor motor control. Small head circumference at birth is also associated with poor motor control.

FUNCIÓN MOTORA EN NIÑOS CON SÍNDROME DE ZIKA CONGÉNITO: OBJETIVO: Evaluar la función motora gruesa y los factores asociados en niños con síndrome de Zika congénito (CZS). MÉTODO: Cincuenta y nueve niños (30 varones, 29 mujeres) con CZS a una edad media (DE) de 14,7 (3,9), meses (rango 5-29 meses) se evaluaron utilizando la Medida de la función motora gruesa (GMFM) y se clasificaron de acuerdo con el Sistema de Clasificación de la Función Motora Gruesa (GMFCS). El impacto estructural neurológico se evaluó mediante neuroimagen. Las características sociodemográficas de las madres y los datos generales de los niños se obtuvieron en entrevistas con las madres y de los registros médicos de los niños. Se realizaron análisis de regresión correlacional y múltiple para identificar los factores asociados con la función motora de estos niños. RESULTADOS: En el 81% de los niños, el deterioro de la función motora fue grave, clasificado como nivel V de GMFCS. El puntaje general de GMFM varió de 5 a 210 (mediana 18; rango intercuartil 11), y solo cuatro niños recibieron puntajes en las dimensiones D y E. Los factores que afectaron la función motora fueron la presencia de malformaciones graves del desarrollo cortical y la circunferencia de la cabeza (microcefalia) al nacer. INTERPRETACIÓN: Aunque el deterioro motor puede ser leve en algunos niños, generalmente el impacto de CZS es grave. Las malformaciones graves del desarrollo cortical y la microcefalia al nacer fueron factores asociados con una función motora más limitada, lo que refleja la mayor gravedad del daño cerebral.

FUNÇÃO MOTORA EM CRIANÇAS COM SÍNDROME CONGÊNITA DE ZIKA: OBJETIVO: Avaliar a função motora grossa e fatores associados em crianças com síndrome congênita de Zika (SCZ). MÉTODO: Cinquenta e nove crianas (30 do sexo masculino, 29 do sexo feminino) com SCZ com uma média (DP) de idade de 14,7 (3,9) meses (variação 5-29m) foram avaliadas usando a Medida da Função Motora Grossa (GMFM) e classificadas de acordo com o Sistema de Classificação da Função Motora Grossa (GMFCS). O dano neurológico foi avaliado por neuroimagem. As características sócio-demográficas da mãe e dados gerais sobre as crianças foram obtidos em entrevistas com as mães e a partir dos prontuários medicos. Análises de correlação e de regressão múltipla foram realizadas para identificar fatores associados com a função motora destas crianças. RESULTADOS: Em 81% das crianças, o comprometimento da função motora era severo, classificao como nível GMFCS V. O escore geral da GMFM various de 5 a 210 (mediana 18; intervalo interquartil 11), com apenas quatro crianças recebendo pontuações nas dimensões D e E. Os fatores que afetaram a função motora grossa foram a presença de malformações severas no desenvolvimento cortical, e o pequeno perímetro cefálico ao nascimento. INTERPRETAÇÃO: Embora a deficiência motora possa ser leve em algumas crianças, em geral ela é severa. Malformações severas no desenvolvimento cortical e o pequeno perímetro cefálico foram fatores associados com pior função motora, refletindo a maior severidade do dano cerebral.

[Primary central nervous system lymphoma: clinical experience in a neurological center]. / Linfoma primario del sistema nervioso central: experiencia clinica en un centro neurologico.

INTRODUCTION: Primary lymphoma of the central nervous system is a variety of non-Hodgkin's lymphoma that accounts for 4-5% of intracranial tumours and 5% of all lymphomas. It has its origin in the brain, the eyes, the leptomeninges and the spinal cord with no systemic evidence of lymphomatoid activity; the subtype of lymphoma is predominantly of B-type cells. PATIENTS AND METHODS: We conducted a descriptive study of the patients diagnosed with primary brain lymphoma who were attended to at third-level centres in Mexico between the years 1980 and 2016. Patients who had been screened for systemic lymphoma were included. The results were analysed by means of simple frequencies, and disease-free and overall survival time was analysed by Kaplan-Meier curves; the differences among curves were analysed by means of log rank. RESULTS: Of a total of 215 patients, there were only 74 cases. By sex, 45% were females and 55% were males. Regarding age, 36.7% were over 60 years old. The most frequent clinical manifestations were motor loss (60%) and cognitive disorders (52%). Most patients received some form of chemotherapy (89%). The only significant factor for radiological response and clinical prognosis was the combined use of radiochemotherapy (p = 0.04493). CONCLUSION: Lymphoma is a tumorous condition with a high clinicoradiological response to treatment, although the response is not long-lasting. Its early identification and multidisciplinary management are essential for a more favourable prognosis in these patients.

TITLE: Linfoma primario del sistema nervioso central: experiencia clinica en un centro neurologico.Introduccion. El linfoma primario del sistema nervioso central es una variedad de linfoma no Hodgkin que representa el 4-5% de los tumores intracraneales y el 5% de todos los linfomas. Se origina en el encefalo, los ojos, la leptomeninge y la medula espinal sin evidencia sistemica de actividad linfomatoide; el subtipo de linfoma mayoritariamente es de celulas de tipo B. Pacientes y metodos. Estudio descriptivo de los pacientes diagnosticados con linfoma cerebral primario que fueron atendidos en centros de tercer nivel en Mexico entre los años 1980 y 2016. Se incluyo a los pacientes que contaran con cribado para busqueda de linfoma sistemico. Los resultados se analizaron mediante frecuencias simples; en el caso del tiempo libre de enfermedad y supervivencia global, mediante curvas de Kaplan-Meier, y las diferencias entre curvas, mediante log rank. Resultados. En un total de 215 pacientes solo hubo 74 casos. El 45% fueron mujeres y el 55%, hombres. El 36,7% eran mayores de 60 años. Las manifestaciones clinicas mas frecuentes fueron deficit motor (60%) y alteraciones cognitivas (52%). La mayoria recibio alguna forma de quimioterapia (89%). El unico factor significativo para respuesta radiologica y pronostico clinico era el uso combinado de radioquimioterapia (p = 0,04493). Conclusion. El linfoma representa una patologia tumoral con alta respuesta clinicorradiologica al tratamiento, aunque la respuesta no es duradera. Es fundamental su identificacion temprana y el tratamiento multidisciplinario para el mejor pronostico de estos pacientes.

Social Adversity and Cognitive, Language, and Motor Development of Very Preterm Children from 2 to 5 Years of Age.

OBJECTIVE: To assess the extent to which social and family factors explain variability in cognitive, language, and motor development among very preterm (<30 weeks of gestation) children from 2 to 5 years of age. STUDY DESIGN: As part of a longitudinal study, very preterm children recruited as neonates were assessed at 2 (n = 87) and 5 (n = 83) years of age using standardized tests of cognitive, language, and motor ability alongside demographically matched full term (FT) children (n = 63). For very preterm children, developmental change scores were calculated for each domain to assess within-individual variability to 5 years of age. Multivariate regression and mixed-effect models examined social risk index, parenting stress, family functioning, and maternal intellectual ability as predictors of developmental variation among very preterm children. RESULTS: Very preterm children demonstrated poorer cognitive, language, and motor abilities than FT children at 2 (P ≤ .001) and 5 (P < .002) years of age. Social adversity was associated with cognitive (P < .001) and language (P < .001) outcomes at both ages, with parenting stress also related to cognitive outcomes (P = .03). Infant medical risk was associated with motor outcome at 5 years (P=.01). Very preterm children showed considerable within-individual variation between assessments. Among very preterm children, neonatal white matter abnormalities predicted worsening cognitive (P=.04) and motor development (P = .01). Social risk index predicted worsening language development (P = .04), but this association was subsequently explained by dysfunctional maternal affective involvement (P = .01) and lower maternal intellectual ability (P = .05). CONCLUSIONS: Both clinical and socioenvironmental factors are associated with cognitive, language, and motor developmental variation among very preterm children from infancy to early school age.

Prevalence of functioning difficulties and disability in Mexican adolescent women and their populational characteristics.

OBJECTIVE: Report prevalence of functioning difficulties and disabilities among Mexican adolescent women 15-17 years old and identify differences in characteristics of those with and without a functioning difficulty or disability. MATERIALS AND METHODS: Using data from the National Survey of Boys, Girls and Women in Mexico 2015 we estimated prevalence of functioning difficulties and disability and used chi square tests for independence and logistic regression to explore associations between this condition and various characteristics. RESULTS: Of Mexican adolescent women 15-17 years old, 11.1% had a functioning difficulty or disability. The group of domains of functioning difficulty and disability with by far the highest prevalence was socio-emotional and behavioral functioning difficulties or disability with 8.6%. Being employed, rural residence and self-reported depression symptoms were associated with having functioning difficulties or disability. CONCLUSIONS: This survey constitutes an important initial step in collecting data on functioning difficulty and disability in Mexico although larger samples should be studied.

Prevalence of functioning difficulties and disability in Mexican adolescent women and their populational characteristics / Prevalencia de dificultades de funcionamiento y discapacidad en adolescentes mexicanas y sus características poblacionales

Abstract: Objective: Report prevalence of functioning difficulties and disabilities among Mexican adolescent women 15-17 years old and identify differences in characteristics of those with and without a functioning difficulty or disability Materials and methods: Using data from the National Survey of Boys, Girls and Women in Mexico 2015 we estimated prevalence of functioning difficulties and disability and used chi square tests for independence and logistic regression to explore associations between this condition and various characteristics. Results: Of Mexican adolescent women 15-17 years old, 11.1% had a functioning difficulty or disability. The group of domains of functioning difficulty and disability with by far the highest prevalence was socio-emotional and behavioral functioning difficulties or disability with 8.6%. Being employed, rural residence and self-reported depression symptoms were associated with having functioning difficulties or disability. Conclusions: This survey constitutes an important initial step in collecting data on functioning difficulty and disability in Mexico although larger samples should be studied.

Resumen: Objetivo: Reportar la prevalencia de dificultades de funcionamiento y discapacidad entre mujeres adolescentes mexicanas de 15-17 años e identificar diferencias en características entre las que tienen tal condición y las que no. Material y métodos: Basado en la Encuesta Nacional de Niños, Niñas y Mujeres 2015, se estimaron prevalencias de problemas de funcionamiento y discapacidad y se exploró la relación con varias características con ji cuadrada y regresión logística. Resultados: El 11.1% de las mujeres adolescentes mexicanas de 15-17 años tuvieron dificultades de funcionamiento o discapacidad. El grupo de ámbitos de dificultades de funcionamiento y discapacidad con la mayor prevalencia fue socioemocional y comportamiento con 8.6%. Tener empleo, residencia rural y síntomas de depresión autoreportadas están asociadas con tener dificultades de funcionamiento y discapacidad. Conclusiones: Esta encuesta constituye un primer paso importante en la generación de datos sobre dificultades de funcionamiento y discapacidad en México, aunque se requiere estudiar el tema en muestras mayores.

Juvenile Huntington disease in Argentina / Doença de Huntington juvenil na Argentina

ABSTRACT We analyzed demographic, clinical and genetic characteristics of juvenile Huntington disease (JHD) and it frequency in an Argentinean cohort. Age at onset was defined as the age at which behavioral, cognitive, psychiatric or motor abnormalities suggestive of JHD were first reported. Clinical and genetic data were similar to other international series, however, in this context we identified the highest JHD frequency reported so far (19.72%; 14/71). Age at onset of JHD is challenging and still under discussion. Our findings reinforce the hypothesis that clinical manifestations, other than the typical movement disorder, may anticipate age at onset of even many years. Analyses of JHD cohorts are required to explore it frequency in populations with different backgrounds to avoid an underestimation of this rare phenotype. Moreover, data from selected populations may open new pathways in therapeutic approaches and may explain new potential correlations between HD presentations and environmental or biological factors.

RESUMO Foram analisadas as características demográficas, clínicas e genéticas de doença de Huntington juvenil (JHD) e na freqüência em uma coorte argentino. A idade de início foi definida como a idade em que distúrbios comportamentais, cognitivos, psiquiátricos ou anormalidades motoras sugestivas de JHD foram relatada pela primeira vez. Os dados clínicos e genéticos foram semelhantes aos de outras séries internacionais, no entanto, neste contexto identificamos a maior freqüência de JHD relatados até agora (19,72%; 14/71). A idade de início de JHD é um desafio ainda em discussão. Nossos resultados reforçam a hipótese de que as manifestações clínicas, além do transtorno de movimento típico, pode antecipar a idade de início em muitos anos. As análises de coortes de JHD são obrigados a explorar frequências em populações com diferentes formações, para evitar uma subestimação deste fenótipo raro. Além disso, os dados de populações selecionadas podem abrir novos caminhos em abordagens terapêuticas e pode explicar novas correlações potenciais entre apresentações de HD e fatores ambientais ou biológicas.

Juvenile Huntington disease in Argentina.

We analyzed demographic, clinical and genetic characteristics of juvenile Huntington disease (JHD) and it frequency in an Argentinean cohort. Age at onset was defined as the age at which behavioral, cognitive, psychiatric or motor abnormalities suggestive of JHD were first reported. Clinical and genetic data were similar to other international series, however, in this context we identified the highest JHD frequency reported so far (19.72%; 14/71). Age at onset of JHD is challenging and still under discussion. Our findings reinforce the hypothesis that clinical manifestations, other than the typical movement disorder, may anticipate age at onset of even many years. Analyses of JHD cohorts are required to explore it frequency in populations with different backgrounds to avoid an underestimation of this rare phenotype. Moreover, data from selected populations may open new pathways in therapeutic approaches and may explain new potential correlations between HD presentations and environmental or biological factors.

Movement disorders in spinocerebellar ataxias in a cohort of Brazilian patients.

BACKGROUND: Movement disorders (MDs) are well recognized in all subtypes of spinocerebellar ataxias (SCA), but phenomenology and frequency vary widely. METHODS: Three hundred seventy-eight patients, from 169 Brazilian families, with SCAs were assessed with neurological examination and molecular genetic testing. RESULTS: Dystonia was the most common movement disorder, found in 5.5% of all patients, particularly in SCA3. We observed Parkinsonian features in 6.6% of SCA3 patients, and myoclonus in two patients of our cohort. CONCLUSIONS: Our study demonstrated that MDs are major extracerebellar manifestations of SCA. The observed phenotypes in addition to ataxia may provide significant clues for a particular SCA genotype.

Neurological events after liver transplantation: a single-center experience.

The aim of this study was to identify potential risk factors linked to neurologic events (NE) occurring after liver transplantation (LT) and use them to construct a model to predict such events. From odds ratios (OR) of risk factors, a scoring system was assessed using multivariate regression analysis. Forty-one of 307 LT patients presented NE (13.3%), with prolonged hospital stay and decreased post-LT survival. On multivariate analysis, factors associated with NE included: severe pre-LT ascites OR 3.9 (1.80-8.41; P = 0.001), delta sodium ≥12 mEq/l OR 3.5 (1.36-8.67; P = 0.01), and post-LT hypomagnesemia OR 2.9 (1.37-5.98; P = 0.005). Points were assigned depending on ORs as follows: ascites 4 points, and hypomagnesemia and delta sodium ≥12 mEq/l, 3 points each (score range = 0-10 points). ROC curve analysis suggested good discriminative power for the model, with a c-statistic of 0.72 (CI 0.62-0.81; P < 0.0001), best performance for a cutoff value >3 points (71% sensitivity, 60% specificity). NE risk increased progressively from 6.4%, to 10.3%, 12.8%, 31.5% and 71.0% as scores rose from 0 to 3, 4, 6-7 and 10 cumulative points, respectively. The score described helps to identify patients potentially at risk for neurologic events, and its prevention would decrease morbidity and mortality after LT.

Opciones terapéuticas actuales para los trastornos del movimiento en la enfermedad de Parkinson / New therapeutic options for Disorders Movement in Parkinsons disease

Los trastornos del movimiento como la enfermedad de Parkinson, temblor y distonías, son algunas de las más comunes condiciones neurológicas que afectan a millones de pacientes. Aunque la medicación se ha mantenido como terapia para esta enfermedad, la neurocirugía ha jugado un papel importante en su manejo en los pasados 50 años. La cirugía es ahora una viable y segura opción para pacientes con enfermedad de Parkinson medicamente intratable, temblor esencial y distonías. El campo de la Neurocirugía Funcional ha sido ampliado y renacido en los últimos 20 años. Este desarrollo ha sido impulsado por el progreso en la neurobiología de los trastornos del movimiento, avances en las técnicas quirúrgicas, invención, desarrollo terapéutico y abordajes innovadores. El crecimiento en nuestro conocimiento de los circuitos neurales de la enfermedad ha determinado y refinado nuestros blancos quirúrgicos y nos guiará al descubrimiento de dianas adicionales para la exploración quirúrgica e investigaciones clínicas. La evolución de las herramientas y técnicas de la neurocirugía estereotáxica han facilitado abordajes seguros y mínimamente invasivos que permiten a los neurocirujanos llegar a varias estructuras cerebrales con fiel exactitud. Esto, unido a los rápidos avances en la capacidad y tecnologías de las imágenes, jugarán un importante papel en mejorar nuestra capacidad para visualizar estructuras cerebrales con incomparable resolución. Las lesiones aprendidas desde la experiencia quirúrgica de los trastornos del movimiento están ya siendo aplicadas al tratamiento quirúrgico de trastornos psiquiátricos y otros trastornos neurológicos crónicos(AU)

Movement Disorders, such as Parkinsons disease, tremor, and dystonia, are among the most common neurological conditions and affect millions of patients. Although medications are the mainstay of therapy for movement disorders, neurosurgery has played an important role in their management for the past 50 years. Surgery is now a viable and safe option for patients with medically intractable Parkinsons disease, essential tremor, and dystonia. The field of functional neurosurgery has witnessed a renaissance over the past 20 years. This development has been fueled by progress in the neurobiology of movement disorders, surgical technical advancements, therapeutic device developments and innovative approaches. The growth in our understanding of the neural circuitry of the disease has determined and refined our surgical targets and will guide the discovery of additional targets for surgical exploration and clinical research. The evolution of stereotactic surgical tools and techniques is facilitating safe and minimally invasive approaches that enable neurosurgeons to target various brain structures with reliable accuracy. This coupled with rapid advances in imaging technology and capabilities, will play an important role in improving our capability to visualize brain structures and function with unparalleled resolution. The lessons learned from movement-disorder surgical experience are already being applied to surgical treatment of psychiatric and other chronic neurological disorders(EU)

Age-related mobility loss is joint-specific: an analysis from 6,000 Flexitest results.

Although aging is commonly linked to a reduction in joint range of motion, it is unclear if all body joints behave similarly. To address this issue, the main purpose of this study was to compare age-related loss of mobility of seven body joints. A total of 6,000 participants (3,835 men and 2,165 women) aged 5 to 92 years took part in this study. The maximal passive range of motion of 20 movements was evaluated by Flexitest, and each movement was scored from 0 to 4. Composite scores were obtained for each of seven joints and for overall flexibility (Flexindex (FLX)) by adding individual movement scores. Confirming previous findings, FLX systematically decreased with aging (p < .001), with female participants being more flexible for all ages (p < 0.001) and having a more gradual, 0.6% vs. 0.8%/year, age reduction (p < .001). Starting at 30 and 40 years, respectively, for male and female participants, the relative contribution of each composite joint score to FLX dramatically changed. Shoulder contribution to FLX male's score went from 13.9% at 28 years of age to only 5.2% at 85 years of age. In general, proportionally, shoulder and trunk became less flexible, while elbow and knee mobility was preserved to a greater extent. Our findings indicated that age-related loss of mobility is rather joint-specific, which could be related to distinct routine usage patterns of the major body joints along life.

Demographic and motor features associated with the occurrence of neuropsychiatric and sleep complications of Parkinson's disease.

OBJECTIVE: To determine whether four key neuropsychiatric and sleep related features associated with Parkinson's disease (PD) are associated with the motor handicap and demographic data. BACKGROUND: The growing number of recognised non-motor features of PD makes routine screening of all these symptoms impractical. Here, we investigated the hypothesis that standard demographic data and the routine assessment of motor signs is associated with the presence of dementia, psychosis, clinically probable rapid eye movement (REM) sleep behavior disorder (cpRBD) and restless legs syndrome (RLS). METHODS: 775 patients with PD underwent standardised assessment of motor features and the presence of dementia, psychosis, cpRBD and RLS. A stepwise feature elimination procedure with fitted logistic regression models was applied to identify which/if any combination of demographic and motor factors is associated with each of the four studied non-motor features. A within-study out-of-sample estimate of the power of the predicted values of the models was calculated using standard evaluation procedures. RESULTS: Age and Hoehn&Yahr (H&Y) stage were strongly associated with the presence of dementia (p value<0.001 for both factors in the final selected model) while a combination of age, disease duration, H&Y stage, dopamine agonists and catechol-O-methyltransferase (COMT) inhibitors was associated with the presence of psychosis. Disease duration and H&Y stage were the significant indicators of cpRBD, and the lack of significant motor asymmetry was the only significant feature associated with RLS-type symptoms but the evidence of association was weak. CONCLUSIONS: Demographic and motor features routinely collected in patients with PD can estimate the occurrence of neuropsychiatric and sleep-related features of PD.

[Neuropsychiatric symptoms in older adults with dementia and their relationship to disease severity]. / Síntomas neuropsiquiátricos en adultos mayores con demencia y su relación con la severidad de la enfermedad.

UNLABELLED: Neuropsychiatric symptoms are the psychopathological dimension of dementia and they integrate a set of symptoms among those are: personality, behavioral and basic functions changes. OBJECTIVE: To analyze the prevalence of neuropsychiatric symptoms in elder people diagnosed with dementia, the association of symptoms with each other, their relationship with the severity of the disease and the stress generated by them in caregivers. MATERIAL AND METHODS: Secondary data analysis of 180 cases diagnosed with dementia from a population-based cross-sectional study of 2,003 older adults living in rural and urban areas of low to medium income in Mexico. RESULTS: Twelve symptoms were assessed using the Neuropsychiatric Inventory, with depression (47.8%), sleep disorders (37.2%) and irritability (34.4%) being the most prevalent ones. Statistically significant associations were found between severity of dementia and frequency of delusions, hallucinations, agitation, depression, euphoria, apathy, disinhibition, irritability and aberrant motor behavior. Anxiety, depression and sleep disorders were associated with mild/moderate caregivers stress levels. Three factors were identified by clustering technique Principal Components Analysis: the first conforming by delusions, disinhibition and euphoria; the second factor was integrated for disorders of appetite and apathy and the third factor included anxiety and aberrant motor behavior. CONCLUSIONS: As in samples from other countries neuropsychiatric symptoms are common in Mexican elderly with dementia in non-institutionalized population, they may be auxiliary as indicators of severity of dementia and their grouping is related to 1) behavioral disorders, 2) vegetative symptoms and apathy, and 3) symptoms of anxiety.

Clinical features and treatment with botulinum toxin in blepharospasm: a 17-year experience.

OBJECTIVE: It was to analyze clinical aspects of patients with blepharospasm, including outcomes of botulinum toxin treatment. Additionally, clinical characteristics of isolated blepharospasm were compared to those of blepharospasm plus other movement disorders. METHODS: Clinical data recorded during 17 years were reviewed. The variables included age, gender, age of onset, past medical history, head trauma, smoking history, family history of dystonia, severity, duration of botulinum toxin relief and adverse effects. RESULTS: A total of 125 patients were included and 75.2% were female. The mean age of onset was 54.3 years; 89.6% of the individuals started with contractions in eye region, and 39.2% of them spread to lower face or neck. Isolated blepharospasm group was compared with blepharospasm-plus group for demographic and clinical features, and therapeutic outcomes, without significant differences. Botulinum toxin treatment improved the severity of contractions (p=0.01) with low rate of side effects (14%). CONCLUSIONS: Both groups - isolated blepharospasm and blepharospasm-plus - shared similar results concerning epidemiology, clinical features and therapeutic response to botulinum toxin.

Clinical features and treatment with botulinum toxin in blepharospasm: a 17-year experience / Característica clínicas e tratamento com toxina botulínica em blefaroespasmo: experiência de 17 anos

OBJECTIVE: It was to analyze clinical aspects of patients with blepharospasm, including outcomes of botulinum toxin treatment. Additionally, clinical characteristics of isolated blepharospasm were compared to those of blepharospasm plus other movement disorders. METHODS: Clinical data recorded during 17 years were reviewed. The variables included age, gender, age of onset, past medical history, head trauma, smoking history, family history of dystonia, severity, duration of botulinum toxin relief and adverse effects. RESULTS: A total of 125 patients were included and 75.2% were female. The mean age of onset was 54.3 years; 89.6% of the individuals started with contractions in eye region, and 39.2% of them spread to lower face or neck. Isolated blepharospasm group was compared with blepharospasm-plus group for demographic and clinical features, and therapeutic outcomes, without significant differences. Botulinum toxin treatment improved the severity of contractions (p=0.01) with low rate of side effects (14%). CONCLUSIONS: Both groups - isolated blepharospasm and blepharospasm-plus - shared similar results concerning epidemiology, clinical features and therapeutic response to botulinum toxin.

OBJETIVOS: Analisar as características clínicas de pacientes com blefaroespasmo e os resultados do tratamento com toxina botulínica. Além disso, os pacientes foram divididos em dois grupos, blefaroespasmo isolado e blefaroespasmo associado a outros distúrbios do movimento, os quais foram comparados quanto a características clínicas e terapêuticas. MÉTODOS: Foram revisados prontuários dos últimos 17 anos. As variáveis consideradas foram idade, sexo, idade de início dos sintomas, antecedentes pessoais, história prévia de trauma crânio-encefálico, tabagismo, história familiar de distonia, gravidade do blefaroespasmo, duração do efeito da toxina botulínica e efeitos adversos. RESULTADOS: Foram incluídos 125 pacientes, dos quais 75,2% eram do sexo feminino. A média de idade do início dos sintomas era 54,3 anos; 89,6% dos pacientes tiveram início dos sintomas na região ocular e em 39,2% dos casos houve disseminação para face ou pescoço. Não houve diferenças significativas entre os grupos blefaroespasmo isolado versus blefaroespasmo-plus. O tratamento com toxina botulínica proporcionou melhora dos sintomas (p=0,01) com baixa incidência de efeitos adversos (14%). CONCLUSÕES: Ambos os grupos - blefaroespasmo isolado e blefaroespasmo-plus - apresentaram as mesmas características clínicas, epidemiológicas e resposta terapêutica à toxina botulínica.