Implanted System for Orthostatic Hypotension in Multiple-System Atrophy.

Orthostatic hypotension is a cardinal feature of multiple-system atrophy. The upright posture provokes syncopal episodes that prevent patients from standing and walking for more than brief periods. We implanted a system to restore regulation of blood pressure and enable a patient with multiple-system atrophy to stand and walk after having lost these abilities because of orthostatic hypotension. This system involved epidural electrical stimulation delivered over the thoracic spinal cord with accelerometers that detected changes in body position. (Funded by the Defitech Foundation.).

[Tauopathy, motricity and cognition]. / Tauopathies, motricité et cognition.

The tauopathies are one of the families of proteinopathies causing neurodegenerative diseases. They are characterized by a combination of cognitive and motor disorders. In this article, we summarize the clinical features of progressive supranuclear palsy and cortico-basal degeneration, focusing on their cognitive-behavioral impairment profiles, which in some cases allow them to be differentiated from other neurodegenerative entities. Finally, we propose tools for therapeutic management.

Les tauopathies sont une des familles de protéinopathies engendrant des maladies neurodégénératives. Elles se caractérisent par l'association de troubles cognitifs et moteurs. Dans cet article, nous résumons les caractéristiques cliniques de la paralysie supranucléaire progressive et de la dégénérescence cortico-basale, en nous attardant sur leurs profils d'atteinte cognitivo-comportementale, qui permettent, dans certains cas, de les différencier d'autres entités neurodégénératives. Enfin, nous proposons des outils de prise en charge thérapeutique.

[Neurology: what's new in 2022]. / Neurologie : ce qui a changé en 2022.

The year 2022 was marked by the development of numerous new treatments for refractory myasthenia gravis. The link between epilepsy and cerebrovascular disorder was studied and lamotrigine discovered to be the optimal treatment choice for epilepsy secondary to stroke to prevent mortality on patient of 45 years and older. New randomized study finally demonstrated the utility of thrombectomy in selected patients with basilar artery occlusion. The causal relationship between Epstein-Barr infection and multiple sclerosis has been proved thanks to a large cohort study. A new possibility of subcutaneous continuous levodopa administration gave promising result. Finally, numerous studies confirmed the efficacy and excellent tolerability of anti-CGRP antibodies.

L'année 2022 a été marquée par l'arrivée de nombreux traitements pour la myasthénie réfractaire. Le lien entre l'épilepsie et le risque cérébro-vasculaire a été bien étudié, démontrant que la lamotrigine semble être le meilleur traitement pour prévenir la mortalité chez les patients de 45 ans et plus. De nouvelles études ont enfin pu établir l'utilité de la thrombectomie dans les occlusions basilaires. Le lien entre le virus d'Epstein-Barr et la sclérose en plaques a pu être prouvé à la suite d'une importante étude de cohorte. Une nouvelle technique d'administration sous-cutanée de la lévodopa semble prometteuse. Enfin, de nombreuses études confirment l'efficacité et l'excellente tolérance des anticorps anti-CGRP (Calcitonine Gene Related Protein).

Highlighting the Dystonic Phenotype Related to GNAO1.

BACKGROUND: Most reported patients carrying GNAO1 mutations showed a severe phenotype characterized by early-onset epileptic encephalopathy and/or chorea. OBJECTIVE: The aim was to characterize the clinical and genetic features of patients with mild GNAO1-related phenotype with prominent movement disorders. METHODS: We included patients diagnosed with GNAO1-related movement disorders of delayed onset (>2 years). Patients experiencing either severe or profound intellectual disability or early-onset epileptic encephalopathy were excluded. RESULTS: Twenty-four patients and 1 asymptomatic subject were included. All patients showed dystonia as prominent movement disorder. Dystonia was focal in 1, segmental in 6, multifocal in 4, and generalized in 13. Six patients showed adolescence or adulthood-onset dystonia. Seven patients presented with parkinsonism and 3 with myoclonus. Dysarthria was observed in 19 patients. Mild and moderate ID were present in 10 and 2 patients, respectively. CONCLUSION: We highlighted a mild GNAO1-related phenotype, including adolescent-onset dystonia, broadening the clinical spectrum of this condition. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.

[Neurology : what's new in 2021]. / Neurologie.

In 2021, we assisted to the publication of new diagnostic criteria, classifications, and guidelines (CIDP, brain tumors, auto-immune encephalitis). Several studies helped to define the pharmacological management of focal and generalized epileptic seizures and epilepsy in pregnant women. The availability of biomarkers and the approval of immunotherapies are modifying the landscape of dementia management. Endovascular interventions without previous thrombolysis seems to be effective in anterior circulation acute ischemic stroke (AIS) and severe posterior circulation AIS. Neurologic complications of Sars-CoV-2 infection were further studied, as well as the efficacy of vaccines in immunosuppressed patients. New molecules and techniques show promising results for the treatment of migraine and cluster headache.

L'année 2021 a été marquée par la publication des nouveaux critères diagnostiques, classifications et guidelines (polyradiculonévrite inflammatoire démyélinisante chronique, tumeurs cérébrales, encéphalites autoimmunes). L'attitude thérapeutique dans les épilepsies focales ou généralisées et l'épilepsie chez la femme enceinte a été mieux définie. Les marqueurs biologiques et les immunothérapies modifient le paysage de la prise en charge des démences. Le traitement endovasculaire des AVC de la circulation antérieure semble efficace indépendamment d'une thrombolyse préalable, ainsi qu'en cas d'AVC sévère de la circulation postérieure. Les complications neurologiques du SARS-CoV-2 ont été éclaircies et l'efficacité des vaccins étudiée chez les patients immunosupprimés. Plusieurs nouvelles molécules et techniques montrent des résultats prometteurs pour les migraines et céphalées en grappe.

[Recent Advances in Neurology]. / Neurologie.

Significant developments were published in 2020 in the field of blood biomarkers in Alzheimer's disease. Several studies helped to define more accurately the management of status epilepticus and of epilepsy in women of childbearing age. The new Swiss guidelines for the pre-hospital management of acute stroke were issued, as are new targets for stroke prevention. Numerous advances concerning the management of NMO-SD (NeuroMyelitis Optica Spectrum Disorder) were published. Different neurological presentations linked to the COVID-19 pandemic were described (central and peripheral). Several studies confirmed the effectiveness of new migraine treatments (including anti-CGRP). New pharmacological therapies are available for Parkinson's disease.

L'année 2020 a vu d'importantes avancées dans le domaine des biomarqueurs sanguins pour le diagnostic biologique de la maladie d'Alzheimer. Plusieurs études permettent de mieux définir la prise en charge de l'épilepsie chez la femme en âge de procréer et de l'état de mal épileptique. Les nouvelles recommandations suisses pour la prise en charge préhospitalière de l'AVC aigu sont en cours de publication, tout comme de nouvelles cibles pour leur prévention secondaire. De nombreuses avancées concernant la prise en charge des Neuromyelitis Optica Spectrum Disorder ont été publiées. Divers tableaux neurologiques (centraux et périphériques) liés à la pandémie de Covid-19 ont été décrits. Plusieurs études ont permis de confirmer l'efficacité des nouveaux traitements de la migraine (notamment les anti-Calcitonin Gene-Related Peptide). Enfin, de nouvelles thérapies pharmacologiques sont disponibles pour la maladie de Parkinson.

Principles of gait encoding in the subthalamic nucleus of people with Parkinson's disease.

Disruption of subthalamic nucleus dynamics in Parkinson's disease leads to impairments during walking. Here, we aimed to uncover the principles through which the subthalamic nucleus encodes functional and dysfunctional walking in people with Parkinson's disease. We conceived a neurorobotic platform embedding an isokinetic dynamometric chair that allowed us to deconstruct key components of walking under well-controlled conditions. We exploited this platform in 18 patients with Parkinson's disease to demonstrate that the subthalamic nucleus encodes the initiation, termination, and amplitude of leg muscle activation. We found that the same fundamental principles determine the encoding of leg muscle synergies during standing and walking. We translated this understanding into a machine learning framework that decoded muscle activation, walking states, locomotor vigor, and freezing of gait. These results expose key principles through which subthalamic nucleus dynamics encode walking, opening the possibility to operate neuroprosthetic systems with these signals to improve walking in people with Parkinson's disease.

NGS-Based Diagnosis of Treatable Neurogenetic Disorders in Adults: Opportunities and Challenges.

The identification of neurological disorders by next-generation sequencing (NGS)-based gene panels has helped clinicians understand the underlying physiopathology, resulting in personalized treatment for some rare diseases. While the phenotype of distinct neurogenetic disorders is generally well-known in childhood, in adulthood, the phenotype can be unspecific and make the standard diagnostic approach more complex. Here we present three unrelated adults with various neurological manifestations who were successfully diagnosed using NGS, allowing for the initiation of potentially life-changing treatments. A 63-year-old woman with progressive cognitive decline, pyramidal signs, and bilateral cataract was treated by chenodeoxycholic acid following the diagnosis of cerebrotendinous xanthomatosis due to a homozygous variant in CYP27A1. A 32-year-old man with adult-onset spastic paraplegia, in whom a variant in ABCD1 confirmed an X-linked adrenoleukodystrophy, was treated with corticoids for adrenal insufficiency. The third patient, a 28-year-old woman with early-onset developmental delay, epilepsy, and movement disorders was treated with a ketogenic diet following the identification of a variant in SLC2A1, confirming a glucose transporter type 1 deficiency syndrome. This case study illustrates the challenges in the timely diagnosis of medically actionable neurogenetic conditions, but also the considerable potential for improving patient health through modern sequencing technologies.

Encephalopathies Associated With Severe COVID-19 Present Neurovascular Unit Alterations Without Evidence for Strong Neuroinflammation.

OBJECTIVE: Coronavirus disease (COVID-19) has been associated with a large variety of neurologic disorders. However, the mechanisms underlying these neurologic complications remain elusive. In this study, we aimed at determining whether neurologic symptoms were caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) direct infection or by either systemic or local proinflammatory mediators. METHODS: In this cross-sectional study, we checked for SARS-CoV-2 RNA by quantitative reverse transcription PCR, SARS-CoV-2-specific antibodies, and 49 cytokines/chemokines/growth factors (by Luminex) in the CSF +/- sera of a cohort of 22 COVID-19 patients with neurologic presentation and 55 neurologic control patients (inflammatory neurologic disorder [IND], noninflammatory neurologic disorder, and MS). RESULTS: We detected anti-SARS-CoV-2 immunoglobulin G in patients with severe COVID-19 with signs of intrathecal synthesis for some of them. Of the 4 categories of tested patients, the CSF of IND exhibited the highest level of cytokines, chemokines, and growth factors. By contrast, patients with COVID-19 did not present overall upregulation of inflammatory mediators in the CSF. However, patients with severe COVID-19 (intensive care unit patients) exhibited higher concentrations of CCL2, CXCL8, and vascular endothelium growth factor A (VEGF-A) in the CSF than patients with a milder form of COVID-19. In addition, we could show that intrathecal CXCL8 synthesis was linked to an elevated albumin ratio and correlated with the increase of peripheral inflammation (serum hepatocyte growth factor [HGF] and CXCL10). CONCLUSIONS: Our results do not indicate active replication of SARS-CoV-2 in the CSF or signs of massive inflammation in the CSF compartment but highlight a specific impairment of the neurovascular unit linked to intrathecal production of CXCL8.

Differential contributions of subthalamic beta rhythms and 1/f broadband activity to motor symptoms in Parkinson's disease.

Excessive beta oscillatory activity in the subthalamic nucleus (STN) is linked to Parkinson's Disease (PD) motor symptoms. However, previous works have been inconsistent regarding the functional role of beta activity in untreated Parkinsonian states, questioning such role. We hypothesized that this inconsistency is due to the influence of electrophysiological broadband activity -a neurophysiological indicator of synaptic excitation/inhibition ratio- that could confound measurements of beta activity in STN recordings. Here we propose a data-driven, automatic and individualized mathematical model that disentangles beta activity and 1/f broadband activity in the STN power spectrum, and investigate the link between these individual components and motor symptoms in thirteen Parkinsonian patients. We show, using both modeled and actual data, how beta oscillatory activity significantly correlates with motor symptoms (bradykinesia and rigidity) only when broadband activity is not considered in the biomarker estimations, providing solid evidence that oscillatory beta activity does correlate with motor symptoms in untreated PD states as well as the significant impact of broadband activity. These findings emphasize the importance of data-driven models and the identification of better biomarkers for characterizing symptom severity and closed-loop applications.

Tratamiento endovascular de aneurisma disecante de la arteria basilar con dispositivo redireccionador de flujo FRED: primer reporte de caso en Colombia / Basilar dissecting aneurysm successfully treated by FRED Flow-Diverter device: first case report in Colombia

RESUMEN INTRODUCCIÓN: Los aneurismas disecantes de la arteria basilar tienen un curso clínico impredecible que, eventualmente, evoluciona a un accidente cerebrovascular hemorrágico o isquémico grave. Por otro lado, su abordaje terapéutico es controvertido y no existen líneas claras de tratamiento. CASO CLÍNICO: Presentamos el caso de una mujer de 28 años con un infarto del tallo cerebral secundario a aneurisma disecante de la arteria basilar, tratado exitosamente con dispositivo endoluminal redireccionador de flujo (FRED). CONCLUSIÓN: El tratamiento endovascular con FRED de los aneurismas disecantes parece ofrecer ventajas sobre las técnicas quirúrgicas abiertas y stents tradicionales, en casos seleccionados.

SUMMARY INTRODUCTION. Dissecting basilar artery aneurysms have an unpredictable course, eventually evolving to serious hemorrhagic or ischemic stroke; on the other hand, its therapeutic approach in controversial and there are no clear lines of treatment. CASE REPORT. We report a case of a 28-year-old female with brain stem infarction secondary to basilar dissecting aneurysm successfully treated by Flow-Redirection Endoluminal Device (FRED). CONCLUSION. Endovascular treatment of dissecting basilar artery aneurysms with FRED seems to offer advantages over traditional open surgical techniques and conventional stents in selected cases.

Uso de toxina botulínica tipo A en pacientes con Espasmo Hemifacial en Cuba / Botulinum toxin A in hemifacial spasm in Cuba

Resumen Objetivo: Evaluar los resultados terapéuticos de la aplicación de la toxina botulínica tipo A en pacientes con espasmo hemifacial atendidos en una consulta de Neurología de trastornos de movimiento en Cuba. Métodos: Se determinaron variables temporales: latencia, duración del efecto máximo y desaparición del efecto terapéutico de la toxina botulínica a 35 pacientes con espasmo hemifacial. Fueron evaluados el deterioro funcional, gravedad, frecuencia y efectos adversos. Resultados: Hubo predominio de mujeres (88,6%) con esta enfermedad. El tiempo de latencia más frecuente resultó ser 48 hs, la duración del efecto máximo hasta los 60 días y la desaparición del efecto terapéutico hasta 3 meses. El deterioro funcional mejoró entre la consulta inicial y los 15 meses de evolución, independientemente del tiempo de presentación de los síntomas y causa del espasmo hemifacial. También, la gravedad y frecuencia mejoraron. Las dosis de toxina botulinica aunmentó significativamente durante el seguimiento de los pacientes. Solo se observaron pacientes con debilidad muscular facial y ptosis parpebral. Conclusiones: Debido al favorable comportamiento de las variables temporales, la mejoría del deterioro funcional evolutivo, y los pocos efectos adversos observados se pudiera sugerir que las dosis de toxina botulínica en el tratamiento del espasmo hemifacial parecen ser eficaces y seguras.

Abstract Objective: To evaluate botulinum toxin A therapy in patients with hemifacial spasm attended in a Neurology Service in Cuba. Patients and methods: 35 patients with hemifacial spasm were treated with botulinum toxin A during 15 months. Some parameters of botulinum toxin were collected including doses, latency, maximum duration and total duration of improvement. Clinical data were determined, such as functional status, gravity, frequency and adverse events. Results: There was a high percentage of female with hemifacial spasm. Most patients showed a latency of 48 hs, maximum duration until 60 days and total duration of improvement of 3 months. Functional status decreased through time, independently of hemifacial spasm onset and its origin. Parameters related to functional status, as gravity and frequency also diminished significantly their categories. Botulinum toxin doses were statistically increased over this treatment, independently of any patient conditions. Only eight patients showed adverse events (facial muscle weakness and ptosis). Conclusions: Recovery of functional status, and low rate of adverse events observed during botulinum toxin treatment might suggest that this therapy seems to be effective and low-risk under our conditions.

Predictores clínicos y de imagen funcional con 99tecnecio - sestamibi para diferenciar el grado de malignidad en gliomas cerebrales / Clinical and functional image with 99tecnecio - sestamibi predictors to differentiate the grade of malignancy of brain gliomas

RESUMEN INTRODUCCIÓN: Los gliomas son los tumores primarios más frecuentes del Sistema Nervioso Central. La tomografia de Emisión de Fotón Único (SPECT) es una técnica de imagen funcional que nos permite acceder a información molecular y diferenciar los gliomas entre sí y de otras entidades nosológicas.. De ello depende la estrategia terapéutica a emplear y el pronóstico en estos pacientes. OBJETIVO: determinar la utilidad de las características clínicas de los pacientes al ingreso y de la SPECT cerebral con 99Tc-MIBI para diferenciar el grado de malignidad de los gliomas cerebrales. PACIENTES Y MÉTODOS: estudio observacional, descriptivo y prospectivo realizado en pacientes del Hospital Clínico Quirúrgico "Hermanos Ameijeiras" y del Hospital Universitario "General Calixto García" durante el período mayo 2012 - febrero 2015. La muestra quedó constituida por 102 pacientes con diagnóstico confirmado de glioma cerebral y con estudio de SPECT Cerebral con 99mTc-MIBI. RESULTADOS: Los principales síntomas y signos, así como el índice de Karnofsky al ingreso no mostraron diferencias significativas respecto al grado de malignidad de los gliomas cerebrales estudiados. Los pacientes con gliomas de alto grado se caracterizaron por una elevación de todos los índices tumorales, así como de la cuantificación de la actividad, concentración volumétrica del 99mTc-MIBI para ambas fases, aunque más marcado para la fase tardía e índice de retención. El Indice tumor corteza contralateral en fase tardía mostró una sensibilidad, especificidad, valor predictivo positivo y negativo de 98,9%, 94,0%, 63,0% y 99,0% respectivamente para diferenciar el grado de malignidad de los gliomas cerebrales. La razón fase tardía/fase temprana del índice tumor/corteza contralateral mostró valores de sensibilidad de 94,1%, especificidad de 98,5%, valor predictivo positivo de 98,7% y valor predictivo negativo de 98,8%. El índice de retención del radiofármaco tuvo una sen-sibidad de 99%, especificidad de 89%, y valores predictivos positivo y negativo de 95% y 99% respectivamente. CONCLUSIÓN: La combinación de: Indice tumor corteza contralateral en fase tardía, razón fase tardía/fase temprana del ÍNDICE tumor/corteza contralateral y el índice de retención del radiofármaco son los parámetros más útiles para predecir el grado de malignidad de los gliomas cerebrales utilizando la SPECT con 99mTc-MIBI.

SUMMARY INTRODUCTION: Gliomas are the most common primary tumors of the Central Nervous System. Single Photon Emission Tomography (SPECT) is a functional imaging technique that allows us to access molecular information and differentiate gliomas from each other and from other entities. It depends on the therapeutic strategy to be used and the prognosis in these patients. OBJECTIVE: To determine the utility of the clinical characteristics of patients on admission and of the brain SPECT with 99mTc-MIBI to differentiate the degree of malignancy of brain gliomas. Patients and methods: An observational, descriptive and prospective study carried out in patients from "Hermanos Ameijeiras" Hospital and "Calixto García" University Hospital during the period May 2012 to February 2015. The sample consisted of 102 patients with confirmed diagnosis of brain glioma and with study of Brain SPECT with 99mTc-MIBI. RESULTS: The main symptoms and signs, as well as the Karnofsky index on admission did not show significant differences regarding the degree of malignancy of the brain gliomas studied. Patients with high-grade gliomas were characterized by an elevation of all tumor indices as well as quantification of activity, volumetric concentration of 99mTc-MIBI for both phases, although more marked for the late phase and retention index. Tumor / contralateral cortex index in late phase showed a sensitivity, specificity, positive and negative predictive value of 98.9%, 94.0%, 63.0%, and 99.0% respectively to differentiate the degree of malignancy of brain gliomas. Late phase / early phase ratio of the tumor / contralateral cortex index showed sensitivity values of 94.1%, specificity of 98.5%, positive predictive value of 98.7%, and negative predictive value of 98.8%. Radiopharmaceutical retention index had a sensitivity of 99%, specificity of 89%, and positive and negative predictive values of 95% and 99% respectively. CONCLUSION: The combination of: Tumor / contralateral cortex index in late phase, late phase / early phase ratio of the tumor / contralateral cortex index, and radiopharmaceutical retention index are the most useful parameters for predicting the degree of malignancy of brain gliomas using SPECT with 99mTc-MIBI.

SPECT cerebral con 99Tc- MIBI para discriminar entre infiltración tumoral y absceso cerebral en casos de tomografía computarizada y resonancia magnética no concluyentes. Presentación de un caso / 99mTc-MIBI Brain SPECT to discriminate between tumor infiltration and brain abscess in cases of computed tomography and magnetic resonance unconvincing. Case Presentation

La diferenciación entre abscesos y metástasis cerebrales en pacientes con algún grado de inmunosupresión y con neoplasias hematológicas resulta en ocasiones difícil. Los estudios funcionales como la tomografía de emisión de fotón único (SPECT) y la tomografía por emisión de positrones (PET) son herramientas diagnósticas eficaces para este propósito. Presentamos el caso de una paciente con diagnóstico de leucemia mieloide crónica en crisis blástica a quien se le informó múltiples abscesos cerebrales; diagnóstico basado en que no existió captación de 99mTc - MIBI en SPECT cerebral y en la buena respuesta al tratamiento antimicrobiano en ocasión en que los estudios de tomografía computarizada (TC) cerebral y resonancia magnética (RM) cerebral no fueron concluyentes. Teniendo en cuenta los resultados de la SPECT se decidió postergar el esquema de quimioterapia, ya que esta podría desencadenar complicaciones probablemente fatales en la paciente.

The differentiation between brain abscess and brain metastasis in patients with some grade of immune suppression and hematological neoplasm is difficult in some situations. Functional studies like Single-Photon Emission Tomography (SPECT) and Positron Emission Tomography (PET) are useful for this purpose. We present the case of a patient with diagnosis of chronic myelogenous leukemia in blast crisis that a diagnosis of brain abscess was made. The diagnosis had a base in 99mTc - MIBI brain SPECT did not show MIBI uptake and the good treatment response to antibiotics when brain CT scan and MRI were not conclusive. Attending to the SPECT result, chemotherapy was postponed because of probably fatal complications in this patient with brain abscess.

Cisternografía radioisotópica con 99Tc-DTPA para confirmación diagnóstica de la hidrocefalia oculta normotensa / 99 Tc-DTPA radionuclide cisternography to confirm the diagnosis of normal pressure hydrocephalus

Se presenta el caso clínico de una paciente que ingresó en el Hospital Clinicoquirúrgico Hermanos Ameijeiras con antecedentes de etilismo crónico y cuadro clínico progresivo de deterioro cognitivo-conductual, trastornos de la marcha y esfinterianos, a quien se le realizó una cisternografía radioisotópica con el objetivo de comprobar su eficacia en el estudio de la dinámica del líquido cefalorraquídeo para diagnosticar la hidrocefalia oculta normotensa. La resonancia magnética de cráneo evolutiva evidenció hidrocefalia y la cisternografía radioisotópica confirmó el diagnóstico de hidrocefalia oculta normotensa. A la paciente se le realizó una derivación ventrículo peritoneal con la que se obtuvo una respuesta clínica evolutiva favorable. Se concluye que la cisternografía radioisotópica continúa siendo una herramienta útil para confirmar este diagnóstico y predecir la respuesta al tratamiento derivativo(AU)

A female patient who was admitted to Hermanos Ameijeiras Clinical and Surgical Hospital is presented here. She had a history of chronic alcohol abuse and progressive clinical deterioration of cognitive behavioral, abnormal gait and sphincter disorders. This patient had a radioisotope cisternography in order to test its effectiveness in the study of the dynamics of cerebrospinal fluid, and to diagnose normotensive hidden hydrocephalus. This MRI revealed hydrocephalus and skull evolutionary radioisotope cisternography confirmed the diagnosis of normal pressure hidden hydrocephalus. This patient received a shunt with a favorable evolutionary clinical response. It is concluded that cisternography radioisotope remains a useful tool to confirm this diagnosis and predict response to derivative treatment(AU)

Trombosis de senos venosos cerebrales en la enfermedad de Behçet / Cerebral venous sinus thrombosis in Behcet's disease

La enfermedad de Behçet es una vasculitis sistémica inmunomediada que afecta los pequeños vasos sanguíneos y se presenta frecuentemente con ulceraciones de las membranas mucosas y síntomas oculares. Se presentó un paciente masculino de 25 años de edad, con antecedentes de úlceras bucales y genitales recurrentes, foliculitis, eritema nudoso, lesiones papulo-pustulosas, el cual tenía, desde 3 meses antes de su presentación en nuestro servicio, manifestaciones clínicas compatibles con un síndrome de hipertensión endocraneana benigna que no logra una evolución favorable con el tratamiento. Mediante la angio-TAC de cráneo se identificó una trombosis de senos venosos cerebrales. Los hallazgos clínicos y radiológicos son compatibles con una enfermedad de Behçet(AU)

Behcet's disease is an immune-mediated systemic vasculitis that affects small blood vessels and it frequently presents with ulceration of the mucous membranes and eye symptoms. A 25 year-old male patient is presented with history of recurrent cold sores and genital folliculitis, erythema nodosum, papular-pustular lesions. For three months before submission to our service, this patient had clinical manifestations compatible with benign intracranial hypertension syndrome that fails favorable evolution with treatment. By skull venuos CTA, venous sinus cerebral thrombosis was identified. Clinical and radiological findings are compatible with Behcet's disease(AU)