Successful Unilateral Surgical Approach to Internal Globus Pallidus and Ventral Intermediate Nucleus of the Thalamus in 3 Cases of Myoclonus-Dystonia Syndrome.

Surgical approaches of internal globus pallidus (GPi) and ventral intermediate thalamic nucleus (Vim) have been used to treat different movement disorders. Three subjects with myoclonus-dystonia syndrome were surgically treated, one of them with GPi and Vim stimulation, while radiofrequency ablation of these structures was performed in the other 2 subjects. Surgical approach of both targets was performed simultaneously on each subject. Mean follow-up was of 33.3 months (22-48 months), the Unified Myoclonus Rating Scale action myoclonus (AM), functional tests (FT), patient questionnaire (PQ) sub-scores, and the Unified Dystonia Rating Scale (UDRS) were used during assessments. Improvement in all scales were seen 6 months after surgery (AM: 74%, FT: 60%, PQ: 63%, UDRS: 65%), and this benefit persisted throughout follow-up (AM: 61%, FT:62%, PQ: 65%, UDRS: 86%). No adverse events were noticed. Simultaneous unilateral procedures of GPi and Vim by either stimulation or ablation techniques improve both motor and functional scores in myoclonus-dystonia syndrome.

Response to SARS-CoV-2 Pandemic in a Non-COVID-19 Designated Latin-American Neurosurgery Department.

Background: Mexico declared the first case of novel coronavirus disease (COVID-19) in February 2020. At the time we write this article, our country is facing a community spread phase, expecting a rapid increase in the number of cases and fatalities. The Fray Antonio Alcalde Civil Hospital of Guadalajara has been declared a non-COVID-19 hospital with the mission of providing care to patients already registered and also those transferred from neurosurgical departments of neighboring centers, which have been converted into COVID-19 only hospitals. Methods: An organized response regarding personnel, surgical case selection, operating room behavior, and facility reorganization were designed to prevent an internal coronavirus outbreak in the neurosurgery department at the Fray Antonio Alcalde Civil Hospital of Guadalajara. Results: Distancing actions by the staff and residents, including ward case discussions, neurosurgery rounds, and classes, will be carried out virtually. We classified neurosurgical patients into 4 groups depending on whether their condition demands care in 0-6 hours, 6-48 hours, 48 hours to 14 days, and >14 days. Subsequently, a questionnaire with epidemiologic, radiologic, clinical, and serologic criteria will be applied to determine the risk of COVID-19 infection to define to which area they are going to be transferred according to the different risk zones in our facility. Conclusions: Despite not being a COVID-19 center, we consider all patients at the neurosurgical ward and staff members as asymptomatic carriers or infected in the preclinical period. Specific measures must be taken to ensure the safety and care of neurosurgical patients and medical staff during the community spread phase.

Generalized Dystonia and Paroxysmal Dystonic Attacks due to a Novel ATP1A3 Variant.

Background: Paroxysmal movement disorders are a heterogeneous group of neurological diseases, better understood in recent years thanks to widely available genetic testing. Case report: A pair of monozygotic twins with dystonia and paroxysmal attacks, resembling paroxysmal non-kinesigenic dyskinesias, due to a novel ATP1A3 variant are reported. The complete resolution of their paroxysms was achieved using levodopa and deep brain stimulation of the internal globus pallidus. Improvement of interictal dystonia was also achieved with this therapy. Discussion: Paroxysmal worsening of movement disorders should be suspected as part of the ATP1A3 spectrum. Treatment outcome might be predicted based on the phenotype.

Posterior fossa tuberculoma in a Huichol native Mexican child: a case report.

BACKGROUND: Tuberculosis is a major health concern in Mexico, especially among the native population. Tuberculomas are a frequent and severe complication of pediatric tuberculosis, these are observed as tumors in neuroimaging studies but are often not diagnosed adequately. CASE PRESENTATION: We present a case of a 12-year-old native Mexican girl Huichol ethnicity diagnosed with a large posterior fossa tuberculoma found by imaging. This tuberculoma was surgically removed. Histopathologic examination and staining with hematoxylin and eosin, and Ziehl-Neelsen techniques of the surgical specimen were performed. Cerebrospinal fluid was analyzed by using the newly available Xpert® MTB/RIF assay (Cepheid, Sunnyvale CA, USA). Granulomatous inflammation with central caseous necrosis surrounded by edematous brain with reactive gliosis and acid-fast bacilli were revealed on histopathologic analysis. Mycobacterium tuberculosis DNA susceptible to rifampicin was detected in the patient's cerebrospinal fluid and the patient was started on anti-tuberculosis treatment. The girl continued to show severe neurologic damage despite surgery and anti-tuberculosis treatment, and she eventually died of respiratory complications. CONCLUSION: Our case highlights the need for early confirmation of tuberculoma diagnosis by molecular assay so that timely treatment can be initiated to prevent severe brain damage. Furthermore, it emphasizes the need to consider tuberculomas in the differential diagnosis of children with neurologic symptoms living in areas of high tuberculosis incidence and those belonging to native populations in developing countries.

Microscopia de força atômica no estudo da superfície endotelial de bifurcações arteriais cerebrais humanas / Atomic force microscopy in the study of the endothelial surface of the human brain artery bifurcations

Objetivo: Apresentar o estudo morfológico do endotélio vascular de artérias cerebrais humanas e discutir aplicações e limitações da miceoscopia de força atômica (AFM) no estudo de patologia vascular. Material e método: Foram utilizadas amostras do segmento M1 e da bifurcação da artéria cerebral média, com e sem doença aterosclerótica. Imagens topográficas, obtidas por microscopia eletrônica de varredura (MEV) das amostras fixadas quimicamente e desidratadas mediante ponto crítico foram confrontadas com imagens de amostras não fixadas, obtidas por AFM. Resultados: As células endoteliais nos segmentos retos de M1 e da bifurcação da ACM, sem doença aterosclerótica, são alongadas e alinhadas com eixo axial do vaso, porém poligonais e sem orientação preferencial na bifurcação aterosclerótica.A bifurcação com aterosclerose apresenta uma monocamada endotelial altamente irregular e corrugada que invagina na luz do vaso e mostra características heterogêneas na superfície da membrana. A resolução das imagens de MEV foi superior àquela obtida nas imagens de AFM a baixa magnificação. Nas imagens de AFM, a parte lateral e as uniões celulares são pouco definidas e a varredura em altas magnificações diminui a resolução. Contudo, é possível determinar a topografia tridimensional da superfície celular, podendo-se, ao mesmo tempo, realizar estudos funcionais. Conclusão: Apesar da combinação única de alta resolução de imagem e operação sob condições próximas às fisiológicas, a AFM em amostras flexíveis apresenta resolução que depende do tipo de cantilever e do grau de hidratação da amostra. Por isso, em amostras vasculares as condições fisiológicas terão de ser estritamente reproduzidas...

Hemangioblastomas aparentemente esporádicos e doença de von Hippel-Lindau não suspeita / Apparently sporadic hemangioblastomas and not suspected von Hippel-Lindau disease

Objetivo: Apresentar os achados associados à doença de von Hippel-Lindau em pacientes portadores de hemangioblastoma do sistema nervoso central, aparentemente esporádico, submetidos a rastreamento (screening)em um centro neurocirúrgico de referência.Métodos:Na base de dados do Instituto Nacional de Neurologia e Neurocirurgia da Cidade do México foram identificados todos os pacientes com diagnóstico histológico confirmado de hemangioblastomado sistema nervoso central entre janeiro de 1980 e agosto de 2000.Os arquivos médicos, radiológicos e patológicos desses pacientes foram revisados retrospectivamente. Destes, 25 pacientes assintomáticos concordaram em submeter-se ao rastreamento, por meio de exame físico, ultra-sonografia e tomografia axial computadorizada de abdômen,ressonância magnética cranioespinhal e exame oftalmológico.Resultados: 32 por cento dos pacientes apresentaram policistose, 16 por cento, hemangioblastomas múltiplos e 12 por cento, carcinomas viscerais. Segundo os critérios de Neumann, logrou-se estabelecer o diagnóstico clínico da doença de von Hippel-Lindau em 60 por cento dos casos. Conclusão:A presença de lesões assintomáticas nos portadores da doença de von Hippel-Lindau assinala a falta de sensibilidade do exame clínico, fazendo-se necessário o rastreamento de todo paciente portador de hemangioblastomas do sistema nervoso central.