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Objective:To investigate the effect of bilateral globus pallidus internus deep brain stimulation (GPi-DBS) on motor performance, quality of life, sleep quality, neuropsychological status, and mood in patients with Meige syndrome.Methods:The clinical data of 17 patients with Meige syndrome accepted bilateral GPi-DBS in Department of Neurosurgery, People's Hospital of Peking University from May 2019 to March 2021 were retrospectively analyzed. Established and validated rating scales were used to assess the motor performance, quality of life, sleep quality, neuropsychological status, and mood at baseline, and 1 and 2 years after GPi-DBS.Results:Burke-Fahn-Marsden dystonia rating scale (BFMDRS) motor total scores decreased from 14.4±6.2 at baseline to 4.3±2.2 and 3.5±1.9 at 1 and 2 years after GPi-DBS, with significant differences ( P<0.05). BFMDRS disability total scores decreased from 6.2±4.0 at baseline to 2.8±2.0 and 2.2±1.5 at 1 and 2 years after GPi-DBS, with significant differences ( P<0.05). In 36-item Short-Form Health Survey (SF-36), the scores of physical function, role-physical, general health sub-items at 1 and 2 years after GPi-DBS were significantly higher than those at baseline ( P<0.05). No significant differences were noted in scores of sleep quality, neuropsychological function, or mood scales at 1 and 2 years after GPi-DBS compared with those at baseline ( P>0.05). Conclusion:Bilateral GPi-DBS is effective and safe in Meige syndrome, which can improve dystonic symptom and quality of life without adverse effects on sleep quality, neuropsychological function, or emotional status.
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Objective:To summarize genotype-phenotype features and explore the long-term outcome of bilateral globus pallidus interna deep brain stimulation (DBS) in chorea-acanthocytosis (ChAc) patients.Methods:Seven patients who diagnosed with ChAc were included in this study from April 2016 to April 2018 at Peking Union Medical College Hospital. Whole-exome sequencing was used for gene analysis of the patients, and the genotype-phenotype features of these patients were recorded. All patients underwent the DBS surgery, and long term follow-up was conducted before surgery, 3 months, 6 months, 1 year, 3 years, and 5 years after surgery. Patients were scored using the Unified Huntington Disease Rating Scale (UHDRS) to evaluate the long-term efficacy of DBS surgery.Results:The main clinical manifestations in all 7 patients were oro-faciol-ingual dyskinesia, limb chorea, dystonia, and dysarthria. Genetic testing found that all patients had VPS13A gene pathogenic variation, but the type of variation was different. The UHDRS motor score before bilateral pallidal DBS surgery was 37.00±16.68, which significantly improved to 19.67±5.99 at 1 year post-surgery, with average improvement of 46.8% ( t=5.20, P=0.003), to 23.86±8.99 at 3 years post-surgery, with average improvement of 35.5% ( t=3.08, P=0.022), and to 29.00±14.97 at 5 years post-surgery, with average improvement of 21.6% ( t=1.41, P=0.217). The symptoms of patients were most significantly improved in limb chorea and oro-facio-lingual dyskinesia. However, at the 5-year follow-up, severe dystonia and gait difficulties reoccurred in 3/7 and 4/7 of the patients, respectively. The patient′s dysarthria had not been effectively improved. Conclusions:The clinical manifestations of patients with ChAc are relatively consistent, but there is significant genetic heterogeneity. Bilateral pallidal DBS therapy is effective for patients with ChAc, but the long-term efficacy decreases with disease progression.
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RESUMEN El abordaje de pacientes intoxicados que presentan antecedentes de patología psiquiátrica supone un reto en la atención médica. La presencia de una patología dual puede conducir a que algunos síntomas somáticos sean desestimados y no se consideren como manifestación de una posible patología grave subyacente. Se reporta el caso de una paciente que, durante su hospitalización debida a un episodio depresivo, presentó una sobredosis incidental de opioides. Al recuperarse de ella, sin embargo, presentó comportamiento desorganizado e ideas delirantes, inicialmente atribuidas a la depresión. Estudios ulteriores demostraron una causa orgánica grave subyacente: lesión isquémica bilateral del globo pálido. Se analiza el cuadro clínico a la luz de la literatura pertinente, abordando los temas de delirium, espectro clínico debido a las lesiones del globo pálido y finalizando con una breve descripción sobre la sobredosis y neurotoxicidad por opioides. Frente a síntomas no concordantes con la evolución usual de una descompensación psicopatológica o una intoxicación por drogas, debe ahondarse en la exploración de posibles causas orgánicas, objetivo para el cual se sugiere considerar estudios de neuroimágenes en todo paciente que experimente una sobredosis de opioides y que posteriormente presente graves manifestaciones de una alteración cognitiva.
SUMMARY The approach to intoxicated patients with a previous history of psychopathological manifestations represents a challenge to medical care. The presence of a dual pathology can lead to somatic symptoms being dismissed and not considered as possible manifestations of a serious underlying pathology. We report the case of a patient who, during her hospitalization for a depressive episode, presented an incidental opioid overdose. Upon recovery, she presented disorganized behavior and delusions, initially attributed to her depression. Further studies demonstrated, however, a serious underlying organic cause: A bilateral ischaemic injury of the globus pallidus. The clinical picture is analyzed in the light of the relevant literature, addressing the issues of delirium, clinical spectrum of globus pallidus lesions and ending with a brief description of opioid overdoses and neurotoxicity. Faced with symptoms that are not consistent with the usual course of a psychopathological decompensation or drug intoxication, possible organic causes should be delved into. It is suggested that neuroimaging studies be considered in any patient who suffers from an opioid overdose and who later presents severe cognitive alterations.
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RESUMEN INTRODUCCIÓN: La distonía mioclónica es un trastorno del movimiento con poca prevalencia, pero muy discapacitante, en el cual es frecuente la refractariedad al tratamiento médico. Cómo opción terapéutica se ha planteado la estimulación cerebral profunda, buscando con ello mejorar la función motora, la discapacidad y la calidad de vida de estos pacientes. MATERIALES Y MÉTODOS: Se presentan 3 pacientes con diagnóstico clínico de distonía mioclónica sin confirmación genética, que fueron llevados a estimulación cerebral profunda bilateral del globo pálido interno. RESULTADOS: Se evidenció una mejoría significativa en la evaluación de la escala unificada de mioclonías (80-90 %) y en la escala de distonía de Burke-Fahn-Marsden (tanto en movilidad como en discapacidad). La mejoría clínica se evidenció en los tres pacientes, en periodos de seguimiento que estuvieron entre los 6 meses y los 5 años luego de la estimulación cerebral profunda. DISCUSIÓN Y CONCLUSIONES: Los hallazgos en esta serie de 3 pacientes colombianos son consistentes con lo reportado en la literatura. Sin embargo, aportan información sobre el desenlace de pacientes sin genotipificación sometidos a estimulación cerebral profunda, dado que la eficacia de la intervención en pacientes con distonía sin confirmación genética aún no ha sido determinada, y depende de otros factores como la edad, el tiempo de evolución y el tipo de distonía.
ABSTRACT INTRODUCTION: Myoclonic dystonia is a movement disorder with low prevalence, but very disabling, where refractoriness to medical treatment is frequent. Deep brain stimulation has been proposed as a therapeutic option, seeking to improve motor function, disability and quality of life in these patients. MATERIALS AND METHODS: We present 3 patients with a clinical diagnosis of Myoclonic-Dystonia without genetic confirmation, who underwent bilateral deep brain stimulation of the Globus Pallidus Internus. RESULTS: A significant improvement was evidenced in the evaluation of the unified myoclonus scale (80-90 %) and in the Burke-Fahn-Marsden dystonia scale (both in mobility and in disability). The clinical improvement was evidenced in the 3 patients, in follow-up periods that were between 6 months and 5 years after deep brain stimulation. DISCUSSION AND CONCLUSIONS: Findings in this Colombian case series are consistent with that reported in the literature. However, the current description provides information on the outcome of patients without genotyping undergoing deep brain stimulation, considering that the efficacy of the intervention in these types of patients without genetic confirmation has not been determined and depends on other factors.
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Qualidade de Vida , Estimulação Encefálica Profunda , Distonia , Globo PálidoRESUMO
Objective To study the clinical significance of globus pallidus signal intensity and the intensity ratio of globus pallidus and putamen (G/P ratio) on magnetic resonance T1WI for the early recognition of neonatal bilirubin encephalopathy.Method From January to December 2017,full-term neonates with hyperbilirubinemia admitted to the neonatology department of our hospital were enrolled in the case group,and full-term neonates without hyperbilirubinemia in the control group.The clinical data,globus pallidus T1WI signal intensity,G/P ratio and the follow-up data were collected.According to the level of hyperbilirubinemia,the neonates in the case group were further assigned into mild hyperbilirubinemia group (serum bilirubin:222 to <256 μmol/L),moderate hyperbilirubinemia group (serum bilirubin:256 to <342 μmol/L),and severe hyperbilirubinemia group (serum bilirubin:≥ 342 μmol/L).According to the injury score of ABE,the neonates with ABE were assigned into mild ABE group,moderate ABE group and severe ABE group.The correlation of globus pallidus T1WI and T2WI signal values,G/P ratio and the serum bilirubin level and ABE degree were analyzed;receiver operating characteristic (ROC) curve was drawn to explore the predictive value of the T1WI signal value and G/P ratio for the diagnosis of ABE;the changes of globus pallidus T1WI and T2WI signal values during the first 6 months after birth and the results of follow-up to 1 year after discharge were also analyzed.Result A total of 175 neonates were included in the case group (65 in the mild hyperbilirubinemia group,71 in the moderate hyperbilirubinemia group and 39 in the severe hyperbilirubinemia group) and 43 neonates in the control group.39 neonates were diagnosed as ABE (21 mild ABE,12 moderate ABE,and 6 severe ABE).The first T1WI signal value and G/P ratio of neonates in the severe hyperbilirubinemia group was higher than the moderate hyperbilirubinemia group,the mild hyperbilirubinemia group and the control group;the T1WI signal value and G/P ratio in the moderate hyperbilirubinemia group was higher than the mild hyperbilirubinemia group and the control group (P < 0.05).No significant difference existed between the mild group and the control group(P > 0.05).T2WI values showed no differences among neonates with different bilirubin levels (P > 0.05).The first T1WI signal value and G/P ratio in the severe ABE group were significantly higher than the moderate and mild ABE group,and the moderate ABE group higher than the mild ABE group (P < 0.05).The ROC curve indicated the optimal cut-off value of T1WI signal and G/P ratio were 628 and 1.38,respectively.Among all the 175 neonates,9 had a decrease in T1WI signal value and an increase in T2WI signal value at 6 months after birth.After 1 year of follow-up visits,7 children were finally diagnosed as chronic bilirubin encephalopathy.All these children had increased signal intensity on T1WI in the acute phase,plus a decreased T1WI signal and an increased T2WI signal in 1 ~ 6 months after birth.Conclusion The globus pallidus T1WI signal and G/P ratio are closely related to the serum bilirubin level and ABE severity.If T1WI signal value > 628 or G/P value > 1.38,ABE should be considered.The T1WI signal value and G/P ratio play important roles as indicators for the early recognition of neonatal bilirubin encephalopathy.
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BACKGROUND AND PURPOSE: Various features of the cerebral cortex and white matter have been extensively investigated in migraine with aura (MwA), but the morphological characteristics of subcortical structures have been largely neglected. The aim of this study was to identify possible differences in subcortical structures between MwA patients and healthy subjects (HS), and also to determine the correlations between the characteristics of migraine aura and the volumes of subcortical structures. METHODS: Thirty-two MwA patients and 32 HS matched by sex and age were analyzed in this study. Regional subcortical brain volumes were automatically calculated using the FSL/FMRIB Image Registration and Segmentation Tool software (https://fsl.fmrib.ox.ac.uk/fsl/fslwiki/Glossary). A general linear model analysis was used to investigate differences in the volume of subcortical structures between the MwA patients and HS. A partial correlation test was used to assess correlations between the volume of subcortical structures and characteristics of MwA. RESULTS: The volumes of the right globus pallidus, left globus pallidus, and left putamen were significantly smaller in MwA patients than in HS (mean±SD): 1,427±135 mm³ vs. 1,557±136 mm³ (p<0.001), 1,436±126 mm³ vs. 1,550±139 mm³ (p=0.001), and 4,235±437 mm³ vs. 4,522±412 mm³ (p=0.006), respectively. There were no significant relationships between subcortical structures and clinical parameters. CONCLUSIONS: These findings suggest that both the globus pallidi and left putamen play significant roles in the pathophysiology of the MwA. Future studies should determine the cause-and-effect relationships, since these could not be discriminated in this study due to its cross-sectional design.
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Humanos , Gânglios da Base , Encéfalo , Córtex Cerebral , Epilepsia , Globo Pálido , Voluntários Saudáveis , Modelos Lineares , Transtornos de Enxaqueca , Enxaqueca com Aura , Putamen , Substância BrancaRESUMO
BACKGROUND AND PURPOSE: There is accumulating evidence that epilepsy is caused by network dysfunction. We evaluated the hub reorganization of subcortical structures in patients with focal epilepsy using graph theoretical analysis based on diffusion-tensor imaging (DTI). In addition, we investigated differences in the values of diffusion tensors and scalars, fractional anisotropy (FA), and mean diffusivity (MD) of subcortical structures between patients with focal epilepsy and healthy subjects. METHODS: One hundred patients with focal epilepsy and normal magnetic resonance imaging (MRI) findings and 80 age- and sex-matched healthy subjects were recruited prospectively. All subjects underwent DTI to obtain data suitable for graph theoretical analysis. We investigated the differences in the node strength, cluster coefficient, eigenvector centrality, page-rank centrality measures, FA, and MD of subcortical structures between patients with epilepsy and healthy subjects. RESULTS: After performing multiple corrections, the cluster coefficient and the eigenvector centrality of the globus pallidus were higher in patients with epilepsy than in healthy subjects (p=0.006 and p=0.008, respectively). In addition, the strength and the page-rank centrality of the globus pallidus tended to be higher in patients with epilepsy than in healthy subjects (p=0.092 and p=0.032, respectively). The cluster coefficient of the putamen was lower in patients with epilepsy than in healthy subjects (p=0.004). The FA values of the caudate nucleus and thalamus were significantly lower in patients with epilepsy than in healthy subjects (p=0.009 and p=0.007, respectively), whereas the MD value of the thalamus was higher than that in healthy subjects (p=0.005). CONCLUSIONS: We discovered the presence of hub reorganization of subcortical structures in focal epilepsy patients with normal MRI findings, suggesting that subcortical structures play a pivotal role as a hub in the epilepsy network. These findings further reinforce the idea that epilepsy is a network disease.
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Humanos , Anisotropia , Núcleo Caudado , Conectoma , Difusão , Epilepsias Parciais , Epilepsia , Globo Pálido , Voluntários Saudáveis , Imageamento por Ressonância Magnética , Estudos Prospectivos , Putamen , TálamoRESUMO
RESUMEN OBJETIVO: Describir aspectos generales en relación con los eventos cerebrovasculares (ECV) isquémicos, así como los principales factores pronósticos que se han relacionado con el desenlace y la recuperación funcional posterior al evento cerebrovascular. MATERIALES Y MÉTODOS: Se realizó una revisión narrativa utilizando bases de datos (PubMed, Science Direct, MEDLINE), plataformas virtuales (National Institutes of Health) y publicaciones del Acta Neurológica Colombiana. RESULTADOS: Ser mujer, la edad avanzada, la inatención (negligencia), la gravedad del compromiso cognitivo y de la función ejecutiva, la desnutrición, y comorbilidades como la neumonía se asocian con un peor pronóstico en los 90 días posteriores al evento. Las alteraciones en la esfera mental (delirio), alteración de la conciencia, hemiplejia o parálisis de la mirada conjugada y el origen cardioembólico del ECV son algunos de los factores asociados con la mortalidad. La lateralidad hemisférica es una variable importante a tener en cuenta para valorar el pronóstico y la discapacidad funcional residual posevento; sin embargo, la evidencia actual es poco concluyente y algo contradictoria en relación con su rol como factor pronóstico. CONCLUSIONES: Es importante un diagnóstico temprano y una intervención adecuada de los pacientes afectados con ECV isquémico, así como el control precoz de los factores modificables de mal pronóstico. Entre los no modificables, la lateralidad hemisférica podría ser más bien un criterio de selección para un programa de rehabilitación específico y personalizado, pues indudablemente existe un compromiso cognitivo y del lenguaje que difiere sustancialmente en relación con la ubicación topográfica de la lesión.
SUMMARY OBJETIVE: To describe general aspects related to acute ischemic stroke (AIS), as well as to know the main prognostic factors that have been related to the outcome and functional recovery after the AIS. MATERIALS AND METHODS: A narrative review was performed using databases (PubMed, Science-Direct, MEDLINE), virtual platforms (National Institutes of Health) and publications of the Colombian Neurological Record. RESULTS: Being female, advanced age, inattention (neglect), severity of cognitive and executive function impairment, malnutrition, and comorbidities such as pneumonia are associated with a worse prognosis in the 90 days after the event. Alterations in the mental sphere (delirium), altered consciousness, hemiplegia or paralysis of the conjugate gaze and the cardioembolic origin of the AIS are some of the factors that are associated with higher mortality. Hemispheric laterality is an important variable to consider in assessing the prognosis and residual functional disability post-event; however the current evidence is inconclusive and somewhat contradictory. CONCLUSIONS: Early diagnosis and adequate intervention of patients with AIS and early control of modifiable factors of poor prognosis are important. Among the non-modifiable, hemispheric laterality may be more a selection criterion for a specific and personalized rehabilitation program, since there is undoubtedly a cognitive and language compromise that differs substantially in relation to the topographical location of the ischemic lesion.
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Prognóstico , Reabilitação , Isquemia Encefálica , Fatores de Risco , Acidente Vascular Cerebral , Avaliação da DeficiênciaRESUMO
RESUMEN INTRODUCCIÓN: El síndrome de Gilles de la Tourette es un trastorno neuropsiquiátrico caracterizado por tics y comorbilidades que comienza en la infancia. La estimulación cerebral profunda (ECP), aceptada como tratamiento para otros trastornos, se reserva para casos severos y pacientes con farmacorresistencia, aunque sigue permaneciendo en terreno experimental para esta patología. El objetivo de este trabajo es analizar la bibliografía científica actual acerca de la ECP del globo pálido interno en la reducción de tics y comorbilidades asociadas a este Síndrome. MATERIAL Y METODOS: Se ha realizado una revisión sistemática de varios artículos científicos consultando (entre febrero y marzo de 2017) las bases de datos MedLine, PubMed, Scopus, Web of Science y Cochrane. Se ha limitado la búsqueda a todos aquellos artículos publicados entre 2012 y 2017, escritos en inglés y realizados en humanos; se excluyeron revisiones, cartas al editor o aquellos que no se centrasen en el tema de estudio. RESULTADOS: En general, los resultados obtenidos muestran mejoras significativas en casi la totalidad de pacientes, pero, la carencia de estudios controlados aleatorizados con muestras mayores, la falta de resultados fiables, la ausencia de uniformidad en los protocolos y el desconocimiento de la fisiopatología y del área ideal a estimular, hacen que la aplicación de esta técnica no goce de evidencia científica suficiente para ser aceptada como parte del tratamiento de este síndrome hipercinético.
SUMMARY INTRODUCTION: Gilles de la Tourette syndrome is a neuropsyquiatric disorder characterized by tics and comorbidities which starts during the infancy. Deep brain Stimulation, accepted as treatment for other diseases, it is reserved for severe cases and pharmacoresistant patients, even though it still remains on the experimental field for this pathology. The main aim of this review is to analyse the current scientific bibliography about Deep brain Stimulation of the Globus Pallidus Internus on the reduction of tics and associated comorbidities from Gilles de la Tourette Syndrome. MATERIAL AND METHODS: A systematic review of several scientific articles was done checking (February-March, 2017) MedLine, PubMed, Scopus, Web of Science and Cochrane databases. We have restricted the search to all those articles published from 2012 to 2017, written in English and done with humans, excluding those which were reviews, letters to the editor or not focussed on the subject of the study. RESULTS: In general, the outcomes shows significant improvements in almost the totality of patients but the lack of randomised controlled trials with higher samples, the shortage of reliable results and the lack of awareness of the physiopathology and the ideal target to stimulate, don't allow this technique to enjoy scientific evidence enough to be accepted as part of the treatment for this hyperkinetic disorder.
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Mobilidade UrbanaRESUMO
The striatum and globus pallidus are principal nuclei of the basal ganglia. Nissl- and acetylcholinesterase-stained sections of the tree shrew brain showed the neuroanatomical features of the caudate nucleus (Cd), internal capsule (ic), putamen (Pu), accumbens, internal globus pallidus, and external globus pallidus. The ic separated the dorsal striatum into the Cd and Pu in the tree shrew, but not in rats and mice. In addition, computer-based 3D images allowed a better understanding of the position and orientation of these structures. These data provided a large-scale atlas of the striatum and globus pallidus in the coronal, sagittal, and horizontal planes, the first detailed distribution of parvalbumin-immunoreactive cells in the tree shrew, and the differences in morphological characteristics and density of parvalbumin-immunoreactive neurons between tree shrew and rat. Our findings support the tree shrew as a potential model for human striatal disorders.
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Animais , Masculino , Camundongos , Ratos , Acetilcolinesterase , Metabolismo , Mapeamento Encefálico , Corpo Estriado , Biologia Celular , Metabolismo , Globo Pálido , Biologia Celular , Metabolismo , Imageamento Tridimensional , Camundongos Endogâmicos C57BL , Modelos Neurológicos , Neurônios , Metabolismo , Parvalbuminas , Metabolismo , Ratos Sprague-Dawley , Estatísticas não Paramétricas , TupaiidaeRESUMO
Gadolinium-based contrast agents (GBCAs) are widely used in magnetic resonance imaging. Recently, a large amount of evidence indicates that change of magnetic resonance signal in deep brain nuclei is related to repeated injection of GBCAs. Especially in the dentatum and pallidum the gadolinium deposition is most obvious. This paper reviews the research status and policy progress of brain deposition of GBCAs, and introduces the relationship between gadolinium deposition in the brain and the type of GBCAs.
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Acquired hepatocerebral degeneration also termed acquired hepatolenticular degeneration, cirrhosis-related Parkinsonism o pseudo-Wilson is a rare, progressive and chronic neurological syndrome that occurs in patients with chronic liver disease, particularly in those with surgically or spontaneously induced portosystemic shunts. The clinical features of this pathological entity include extrapyramidal signs, ataxia, cognitive decline and neuropsychiatric changes, such as delirium, apathy, lethargy and emotional instability. Brain Magnetic Resonance Imaging classically shows symmetrical T1-weighted hyperintensities in the globus pallidus, substantia nigra and periaqueductal gray matter. Although its pathogenesis is not completely elucidated, it is postulated that the excess manganese accumulation and deposition in the basal ganglia, leading to dysfunctional dopaminergic system in this anatomical location, would have a key role in triggering the disease. Orthotopic liver transplantation is the mainstay of treatment and is considered effective by reducing motor and cognitive alterations. Other therapeutic alternatives that have reported symptomatic improvement are the use of bromocriptine or levodopa and portosystemic shunts occlusion. In this article, we report a case of a 63-year-old woman with clinical manifestations over the course of one year, characterized by cognitive decline, chorea, gait and language disturbances. She was examined with plasma levels of copper and ceruloplasmin, which excluded the possibility of Wilson´s Disease, its main differential diagnosis. Neuroimaging revealed T1-weighted hyperintensity in the pallidum, confirming suspected diagnosis of acquired hepatocerebral degeneration.
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Humanos , Feminino , Pessoa de Meia-Idade , Degeneração Hepatolenticular/fisiopatologia , Degeneração Hepatolenticular/diagnóstico por imagem , Telangiectasia , Encéfalo/diagnóstico por imagem , Técnicas de Laboratório Clínico , Degeneração Hepatolenticular/terapia , Degeneração Hepatolenticular/epidemiologiaRESUMO
RESUMO Objetivos: Pouco se sabe sobre a atuação dos esteroides androgênicos anabolizantes (EAA) no cérebro humano e, por isso, resolvemos estudar a perda neuronal causada pelo uso e abuso de EAA em camundongos. Métodos: Utilizamos 60 camundongos da linhagem Swiss, sendo 30 machos e 30 fêmeas, divididos em três grupos: 20 animais foram tratados com Deposteron® (cipionato de testosterona); outros 20 animais foram tratados com Winstrol Depot® (stanozolol); os últimos 20 animais foram tratados com solução salina. Todos foram submetidos à natação por 15 minutos. Finalizado o tratamento, os animais foram sacrificados pelo método de inalação de Halotano. Os encéfalos foram retirados e armazenados em solução de formaldeído a 4% por 24 horas. De cada encéfalo foram retiradas amostras homotípicas da região média do cérebro em cortes frontais para que pudéssemos avaliar as áreas estabelecidas para este estudo. Resultados: As análises da estimativa dos perfis celulares mostraram que houve uma diminuição do número de perfis no núcleo pálido dos animais machos tratados com Winstrol Depot®. Conclusão: Esses resultados nos permitem inferir que o uso inadequado e sem orientação médica de EAA pode levar a degenerações celulares.
ABSTRACT Objectives: Little is known about the action of anabolic-androgenic steroids (AAS) on the human brain and, therefore, we decided to study the neuronal loss caused by use and abuse of AAS in mice. Methods: We used 60 Swiss mice, 30 males and 30 females, divided into three groups: 20 animals treated with Deposteron® (testosterone cypionate); another 20 animals were treated with Winstrol Depot® (stanozolol); the last 20 animals were treated with saline solution. All the animals were submitted to swimming for 15 minutes. After the treatment, the animals were euthanized by halothane inhalation (Halotano) method. The brains were removed and stored in 4% formal-dehyde solution for 24 hours. From each brain, homotypic samples were taken from the middle region of the brain in frontal cuts so that we could evaluate the areas established for this study. Results: Analyzes of the estimated cell profiles showed that there was a decrease in the number of profiles in the pallidal nucleus of the male animals treated with Winstrol Depot®. Conclusion: These results allow us to infer that inadequate and non-medical use of AAS can lead to cellular degeneration.
RESUMEN Objetivos: Poco se sabe acerca del efecto de la acción de los esteroides anabólicos androgénicos (EAA) en el cerebro humano y, por este motivo, decidimos estudiar la pérdida neuronal causada por el uso y abuso de EAA en ratones. Métodos: Utilizamos 60 ratones de linaje Swiss, siendo 30 machos y 30 hembras, divididos en tres grupos: 20 animales fueron tratados con Deposteron® (cipionato de testosterona); otros 20 animales fueron tratados con Winstrol Depot® (stanozolol); los últimos 20 animales fueron tratados con solución salina. Todos fueron sometidos a natación durante 15 minutos. Terminado el tratamiento, los animales fue-ron sacrificados por el método de inhalación de Halotano. Los cerebros fueron retirados y almacenados en solución de formaldehído al 4% durante 24 horas. De cada cerebro fueron retiradas muestras homotípicas de la región media del cerebro en cortes frontales, así que pudimos evaluar las áreas establecidas para este estudio. Resultados: El análisis de la estimación de los perfiles celulares mostró que hubo una disminución en el número de perfiles en el globo pálido de los animales machos tratados con Winstrol Depot®. Conclusión: Estos resultados permiten inferir que el uso inadecuado y sin orientación médica de EAA puede conducir a la degeneración celular.
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A 21-year-old male was admitted with severe right arm and hand tremors after a thalamic hemorrhage caused by a traffic accident. He was also suffering from agonizing pain in his right shoulder that manifested after the tremor. Neurologic examination revealed a disabling, severe, and irregular kinetic and postural tremor in the right arm during target-directed movements. There was also an irregular ipsilateral rest tremor and dystonic movements in the distal part of the right arm. The amplitude was moderate at rest and extremely high during kinetic and intentional movements. The patient underwent left globus pallidum internus and ventral intermediate thalamic nucleus deep brain stimulation. The patient improved by more than 80% as rated by the Fahn-Tolosa-Marin Tremor Rating Scale and Visual Analog Scale six months after surgery.
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Humanos , Masculino , Adulto Jovem , Acidentes de Trânsito , Braço , Estimulação Encefálica Profunda , Felodipino , Globo Pálido , Mãos , Hemorragia , Exame Neurológico , Dor de Ombro , Ombro , Tremor , Escala Visual AnalógicaRESUMO
OBJECTIVE: To compare the therapeutic and adverse effects of globus pallidus interna (GPi) and subthalamic nucleus (STN) deep brain stimulation (DBS) for the treatment of advanced Parkinson's disease (PD). METHODS: We retrospectively analyzed the clinical data of patients with PD who underwent GPi (n = 14) or STN (n = 28) DBS surgery between April 2002 and May 2014. The subjects were matched for age at surgery and disease duration. The Unified Parkinson's Disease Rating Scale (UPDRS) scores and levodopa equivalent dose (LED) at baseline and 12 months after surgery were used to assess the therapeutic effects of DBS. Adverse effects were also compared between the two groups. RESULTS: At 12 months, the mean changes in the UPDRS total and part I–IV scores did not differ significantly between the two groups. However, the subscores for gait disturbance/postural instability and dyskinesia were significantly more improved after GPi DBS than those after STN DBS (p = 0.024 and 0.016, respectively). The LED was significantly more reduced in patients after STN DBS than that after GPi DBS (p = 0.004). Serious adverse effects did not differ between the two groups (p = 0.697). CONCLUSION: The patients with PD showed greater improvement in gait disturbance/postural instability and dyskinesia after GPi DBS compared with those after STN DBS, although the patients had a greater reduction in LED after STN DBS. These results may provide useful information for optimal target selection for DBS in PD.
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Humanos , Estimulação Encefálica Profunda , Discinesias , Marcha , Globo Pálido , Levodopa , Doença de Parkinson , Estudos Retrospectivos , Núcleo Subtalâmico , Usos TerapêuticosRESUMO
Internal globus pallidus (GPi) deep brain stimulation (DBS) has been widely accepted as an effective treatment modality of medically refractory dystonia. However, there have been few studies regarding the safety issue of pregnancy and childbirth related with DBS. This report describes a female patient who was pregnant and delivered a baby after GPi DBS surgery. A 33-year-old female patient with acquired generalized dystonia underwent bilateral GPi DBS implantation. She obtained considerable improvement in both movement and disability after DBS implantation. Four years later, she was pregnant and the obstetricians consulted us about the safety of the delivery. At 38-weeks into pregnancy, a scheduled caesarian section was carried out under general anesthesia. After induction using thiopental and succinylcholine, intubation was done quickly, followed by DBS turn off. For hemostasis, only bipolar electrocautery was used. Before awakening from the anesthesia, DBS was turned on as the same parameters previously adjusted. After delivery, she could feed her baby by herself, because the dystonia of left upper extremity and hand was improved. Until now, she has been showing continual improvement and being good at housework, carrying for children, with no trouble in daily life. This observation indicates that the patients who underwent DBS could safely be pregnant and deliver a baby.
Assuntos
Adulto , Criança , Feminino , Humanos , Gravidez , Anestesia , Anestesia Geral , Estimulação Encefálica Profunda , Distonia , Eletrocoagulação , Globo Pálido , Mãos , Hemostasia , Zeladoria , Intubação , Parto , Succinilcolina , Tiopental , Extremidade SuperiorRESUMO
ABSTRACT Background: Patients with Wilson's disease (WD) present cognitive impairment, especially in executive functions. Which other factors might be associated with global cognitive decline in these patients remains unclear. Objective: To assess which factors are associated with worse performance on a global cognitive test in patients with WD. Methods: Twenty patients with WD underwent cognitive assessment with the following tests: the Mini-Mental State Examination (MMSE), Dementia Rating Scale (DRS), verbal fluency test, brief cognitive battery, clock drawing test, Frontal Assessment Battery, Stroop test, Wisconsin card sorting test, Hopper test, cubes (WAIS) and the Pfeffer questionnaire. MRI changes were quantified. Patients with poor performance on the DRS were compared to patients with normal performance. Results: Nine patients had a poor performance on the DRS. This group had lower educational level (9.11±3.58 × 12.82±3.06) and a greater number of changes on MRI (9.44±2.74 × 6.27±2.45). The presence of hyperintensity in the globus pallidus on MRI was more frequent in this group (66.6% vs 9.0%), with OR=5.38 (95% CI 0.85-33.86). Conclusion: Global cognitive impairment was prevalent in this sample of patients with WD and was associated with low educational level, number of changes on MRI and MRI hyperintensity in the globus pallidus.
RESUMO Embasamento: Pacientes com doença de Wilson (DW) apresentam comprometimento cognitivo, principalmente de funções executivas. Existem dúvidas sobre quais outros fatores poderiam estar associados ao declínio cognitivo global nesses pacientes. Objetivo: Avaliar quais fatores estão associados ao pior desempenho em teste cognitivo global em pacientes com DW. Métodos: Vinte pacientes com DW em tratamento regular foram submetidos à avaliação cognitiva com os seguintes testes: Mini-Exame do Estado Mental, escala de demência de Mattis (DRS), fluência verbal, bateria cognitiva breve, desenho do relógio, bacteria de avaliação frontal, Stroop, teste de seleção de cartões de Wisconsin, Hopper, Cubos e ao questionário de Pfeffer. As alterações em RM foram quantificadas. Pacientes com desempenho alterado na DRS foram comparados aos pacientes com desempenho normal. Resultados: Nove pacientes apresentavam desempenho alterado na DRS. Eles apresentavam menor nivel educacional (9,11±3,58 × 12,82±3,06 anos, respectivamente) e maior quantidade de alterações na RM (9,44±2,74 × 6,27±2,45). A presença de hipersinal no globo pálido na RM foi mais frequente nesse grupo (66,6% × 9,0%), com OR=5,38 (IC 95% 0,85-33,86). Conclusão: Alterações cognitivas globais foram frequentes nesta amostra de pacientes com DW e se associaram à baixa escolaridade, quantidade de alterações em RM e a hipersinal no globo pálido à RM.
Assuntos
Humanos , Espectroscopia de Ressonância Magnética , Disfunção Cognitiva , Globo Pálido , Degeneração Hepatolenticular , Testes NeuropsicológicosRESUMO
Objective To investigate the effectiveness of deep brain stimulation (DBS) treating Parkinson's disease.Methods Forty cases of Parkinson's disease were selected from March 2014 to August 2015.The clinical symptoms of these patients were described and quantitatively analyzed with Unified Parkinson's Disease Rating Scale (UPDRS) before and after the procedure of DBS surgery.Results After deep brain stimulation surgery,the symptoms including muscle stiffness,static tremor,bradykinesia were improved,UPDRS scores were significantly lower and the demanding dosage of Parkinson disease drugs such as L-dopa/benserazide and L-dopa/carbidopa were also reduced.Conclusion Deep brain stimulation for Parkinson's disease is safe and effective.It can obviously control the symptoms,reduce the dosage of oral drugs,and improve the quality of life.
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Objective To evaluate functional activity of the subcortical nuclei in Wilson's disease (WD) using resting state functional MRI (rs-fMRI),and to evaluate damage to the functional conjunction in the extracorticospinal tract in WD patients.Methods Twenty-two patients with WD (between January 2015 and January 2016),including 18 with cerebral type and 4 with hepatic type,and 20 age-matched healthy controls were enrolled.Neurological symptoms were scored using the modified Young Scale.Patients with cerebral type WD were divided into 4 subgroups.All study subjects underwent rs-fMRI of the brain.The values of amplitude of low-frequency fluctuation (ALFF) and regional homogeneity (REHO) in the thalamus,caudate nucleus,putamen and globus pallidus were determined.The relationships between rsfMRI metrics and clinical status were evaluated.Results ALFF values were lower in the caudate nucleus,putamen and right thalamus of WD patients than in controls (t =-3.07,-3.00,-3.12,-2.46,-2.20;P =0.005,0.006,0.004,0.020,0.036),while REHO values were lower in the left caudate nucleus and left thalamus of WD patients (t =-2.38,-2.16;P =0.025,0.040).In the caudate nucleus (P =0.032,0.029,0.023),thalamus (P =0.022,0.041,0.035) ALFF values were lower in group 4 than in other groups.REHO values of the putamen (P =0.040,0.017,0.040) and thalamus (P =0.024,0.029 7,0.041) were higher in group 4 than in other groups.ALFF values in the caudate nucleus (t =-0.29,P=0.037),and thalamus (t =-1.77,P =0.042) were lower,and REHO values in the caudate nncleus (t =-1.46,P =0.040) were lower,in patients of cerebral type than in hepatic type patients.Conclusions The damage to the functional activity of the subcortical nuclei may occur in the WD patients.The functional activity of nuclei may be different between hepatic and cerebral type patients.Damage to the activity of neurons in the putamen and thalamus may correlate with psychiatric symptoms in WD patients.
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Chorea-acanthocytosis (ChAc) is a rare hereditary disorder characterized by involuntary choreiform movements and erythrocytic acanthocytosis. Pharmacotherapy for control of involuntary movements has generally been of limited benefit. Deep brain stimulation (DBS) has recently been used for treatment of some refractory cases of ChAc. We report here on the effect of bilateral high-frequency DBS of globus pallidus interna in a patient with ChAc.