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Spinal dysraphisms (SDs) are congenital malformations of the spinal cord, determined by derangement in the complex cascade of embryologic events involved in spinal development. They represent a heterogeneous group ranging from mild clinical manifestations-going unnoticed or being discovered at clinical examination-to a causal factor of life quality impairment, especially when associated with musculoskeletal, gastrointestinal, genitourinary, or respiratory system malformations. Knowledge of the normal embryologic development of the spinal cord-which encompasses three main steps (gastrulation, primary neurulation, and secondary neurulation)-is crucial for understanding the pathogenesis, neuroradiologic scenarios, and clinical-radiologic classification of congenital malformations of the spinal cord. SDs can be divided with clinical examination or neuroradiologic study into two major groups: open SDs and closed SDs. Congenital malformations of the spinal cord include a wide range of abnormalities that vary considerably in imaging and clinical characteristics and complexity and therefore may represent a diagnostic challenge, even for the experienced radiologist. Online supplemental material is available for this article. ©RSNA, 2021.
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Imagen por Resonancia Magnética , Disrafia Espinal , Desarrollo Embrionario , Humanos , Médula Espinal , Disrafia Espinal/diagnóstico por imagen , Columna VertebralRESUMEN
The presence of dysmorphic neurons with strong cytoplasmatic accumulation of heavy non-phosphorylated neurofilament is crucial for the diagnostics of focal cortical dysplasia type II (FCDII). While ILAE's classification describes neocortical dysplasias, some groups have reported patients with mesial t abnormal neurons in the hippocampus of mesial temporal lobe epilepsy. Here we report a patient with such abnormal neurons in the hippocampus and compared it with previous reports of hippocampal dysplasia. Finally, we discuss the need for diagnostic criteria of hippocampal dysplasia.
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Epilepsia del Lóbulo Temporal , Hipocampo , Adulto , Humanos , Epilepsia , Epilepsia del Lóbulo Temporal/diagnóstico , Epilepsia del Lóbulo Temporal/patología , Hipocampo/patología , Imagen por Resonancia Magnética , Malformaciones del Desarrollo Cortical/diagnóstico , Malformaciones del Desarrollo Cortical/patología , Malformaciones del Desarrollo Cortical de Grupo I/diagnóstico , Malformaciones del Desarrollo Cortical de Grupo I/patologíaRESUMEN
Juvenile myoclonic epilepsy (JME) is a well-defined idiopathic generalized epilepsy (IGE) syndrome, being the most common IGE in adults and accounting for 5-11% of patients with epilepsy. While neuropsychological and neuroimaging studies have discussed the thalamofrontal dysfunction as the major pathophysiologic mechanism of JME, investigation on memory is scarce in patients with JME, with lack of objective assessments addressing common complaints and daily difficulties such as recalling telephone numbers, messages to pass on, and taking antiepileptic drugs regularly. The aim of this study was to objectively assess memory deficits in a group of patients with JME using neuropsychological examination combined with structural MRI of the hippocampi. After informed consent, a cohort of 56 consecutive patients with JME (29 males; mean age ± SD = 26.5 ± 9.01 years; range = 14.0-55.0 years) was included. The control group consisted of 42 healthy volunteers (18 males; mean age ± SD = 31.0 ± 8.54 years; range=20.0-56.0 years) without a family history of neuropsychiatric disorders. Patients and controls were submitted to a MRI and to a neuropsychological assessment, and comparisons between groups were performed, as well as a correlation study between hippocampal atrophy and neuropsychological performance in a group of patients with JME. The level of statistical significance was set at p<0.05. Significant hippocampal atrophy among patients with JME was observed, which was correlated with memory dysfunctions. The present findings reinforce the existence of functional-anatomic ictogenic networks that are not limited to frontal lobes, providing further support towards the concept of 'system epilepsies' in JME.
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Hipocampo/patología , Trastornos de la Memoria/etiología , Epilepsia Mioclónica Juvenil/complicaciones , Epilepsia Mioclónica Juvenil/patología , Adolescente , Adulto , Atrofia/etiología , Electroencefalografía , Femenino , Humanos , Procesamiento de Imagen Asistido por Computador , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Análisis Multivariante , Pruebas Neuropsicológicas , Grabación en Video , Adulto JovenRESUMEN
The Sotos syndrome is an autosomal dominant disorder characterized by haploinsufficiency of NSD1 gene, with some individuals affected by epilepsy and, rarely, drug-resistant seizures. A 47-years-old female patient with Sotos syndrome was diagnosed with focal-onset seizures in left temporal lobe, left-side hippocampal atrophy, and neuropsychological testing with decreased performance in several cognitive domains. Patient was treated with left-side temporal lobe resection and developed complete awake seizure control in 3-years of follow-up, with marked improvement in quality-of-life. In selected, clinically concordant patients, resective surgeries may play a significant role in improving patient's quality of life and seizure control.
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A five-and-a-half-year-old girl started experiencing progressive left hemiparesis at age two and a half years. At age five years and four months she started presenting clusters of asymmetric periodic epileptic spasms with no hypsarrhythmia. The ictal EEG showed periodic, constant and stereotyped complexes. Serial brain imaging revealed progressive atrophy of the right hemisphere with increased T2 signal on MRI. She underwent a right hemispherotomy, and histological examination showed signs of inflammation and features of focal cortical dysplasia (FCD). She has been seizure-free for 16 months. This case is unique in the following aspects: the presence of typical Rasmussen encephalitis features of progressive unilateral brain involvement without seizures, a delay of almost three years prior to seizure onset; an atypical seizure type presentation with periodic epileptic spasms and the presence of FCD associated with inflammatory changes. [Published with video sequences].
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Encefalitis/diagnóstico , Epilepsia/etiología , Espasmo/etiología , Encéfalo/patología , Preescolar , Electroencefalografía , Encefalitis/cirugía , Encefalitis/terapia , Epilepsia/diagnóstico , Femenino , Hemisferectomía , Humanos , Lactante , Imagen por Resonancia Magnética , Procedimientos Neuroquirúrgicos , Paresia/etiología , Espasmo/diagnóstico , Espasmos Infantiles/diagnóstico , Tomografía Computarizada por Rayos XRESUMEN
Here we describe a successful mechanical thrombectomy (MT) for acute large vessel occlusion in stroke treatment with one passage (thrombolysis in cerebral infarction, TICI 3). Immediately after the withdrawing of the stent retriever, a narrowing of the middle cerebral artery was diagnosed. The rate of vasospasms during this procedure can be as higher as 41% (range from 6-41%). Here we describe our protocol when a narrowing of the artery is visualized after a stent retriever is withdrawn. A patient presented in our emergency room with National Institute of Health Stroke Scale (NIHSS) of 21, Alberta Stroke Program Early CT Score (ASPECTS) 8, computed tomography angiography revealed occlusion of the M1 segment and MT was indicated. One passage TICI â ¢ was achieved. After that, the image showed a narrowing of the artery. We present one case of a spasm after stent retriever technique for MT, we injected vasodilator and the artery became normal in a few minutes differentiating between atheromatous stenosis and vasospasm. We present a technical note that can help to make the differentiation of vasospasm or atheromatous disease after MT with the stent retriever technique.
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INTRODUCTION: Parkinson's disease (PD) is the second most common neurodegenerative disease. However, the rate of misdiagnosis remains high, even at specialized movement disorder centers. PURPOSE: To assess the accuracy of nigrosome 1 and midbrain neuromelanin evaluation in the diagnosis of PD, and to identify possible differences in diagnostic accuracy between neuroradiologists of different experience levels in analyzing these structures. METHODS: A case-control study was conducted between April 2017 and January 2019. We prospectively evaluated 41 PD patients and 21 control individuals. Participants underwent axial, T2*-weighted, multi-echo gradient echo (GRE), and susceptibility-weighted imaging phase (SWIp) magnetic resonance imaging (MRI) sequences to evaluate nigrosome 1 and axial, T1-weighted, turbo spin-echo magnetization transfer contrast (MTC) MRI sequences to evaluate midbrain neuromelanin. All MRI sequences were acquired at 3.0 T. The images were analyzed by two experienced neuroradiologists, one with and one without expertise in nigrosome 1 and neuromelanin imaging. The sensitivity, specificity, and accuracy of the diagnoses were calculated between PD patients vs. healthy participants and between the two neuroradiologists. RESULTS: The sensitivity and specificity of each imaging sequence for PD diagnosis were as follows: multi-echo, 100% and 86%; SWIp, 91% and 88%; and T1 MTC, 90% and 93%, respectively. The accuracy of clinical PD diagnosis was higher for the expert neuroradiologist compared to the neuroradiologist lacking expertise. CONCLUSION: Nigrosome 1 and midbrain neuromelanin represent useful tools for PD diagnosis. However, these structures should be evaluated by experienced neuroradiologists in order to ensure high accuracy and reproducibility.
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Imagen por Resonancia Magnética/normas , Mesencéfalo/diagnóstico por imagen , Neuroimagen/normas , Enfermedad de Parkinson/diagnóstico por imagen , Anciano , Estudios de Casos y Controles , Competencia Clínica/normas , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Masculino , Melaninas/metabolismo , Mesencéfalo/metabolismo , Persona de Mediana Edad , Neuroimagen/métodos , Enfermedad de Parkinson/metabolismo , Estudios Prospectivos , Sensibilidad y Especificidad , Sustancia Negra/diagnóstico por imagen , Sustancia Negra/metabolismoRESUMEN
Formerly called dysembryoplastic neuroepithelial tumour (DNET) of the septum pellucidum, myxoid glioneuronal tumour (MGT) was recently recognized as a distinct entity. We report three cases of presumed MGT with typical location and image features.
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Isquemia Encefálica , Accidente Cerebrovascular , Humanos , Stents , Trombectomía , Resultado del TratamientoAsunto(s)
Humanos , Isquemia Encefálica , Accidente Cerebrovascular , Stents , Resultado del Tratamiento , TrombectomíaRESUMEN
We investigated a relationship between the FLAIR signal found in mesial temporal sclerosis (MTS) and inflammation. Twenty nine patients were selected through clinical and MRI analysis and submitted to cortico-amygdalo-hippocampectomy to seizure control. Glutamate, TNFα, IL1, nitric oxide (NO) levels and immunostaining against IL1β and CD45 was performed. Control tissues (n=10) were obtained after autopsy of patients without neurological disorders. The glutamate was decreased in the temporal lobe epilepsy (TLE) -MTS group (p<0.001), suggesting increased release of this neurotransmitter. The IL1β and TNFα were increased in the hippocampus (p<0.05) demonstrating an active inflammatory process. A positive linear correlation between FLAIR signal and NO and IL1β levels and a negative linear correlation between FLAIR signal and glutamate concentration was found. Lymphocytes infiltrates were present in hippocampi of TLE patients. These data showed an association between hippocampal signal alteration and increased inflammatory markers in TLE-MTS.
Este estudo foi delineado para investigar a presença de relação entre a intensidade de sinal em FLAIR e níveis de citocinas, óxido nítrico (NO) e glutamato no hipocampo de pacientes com epilepsia do lobo temporal refratária, associada com esclerose mesial (TLE-MTS). Vinte e nove pacientes foram selecionados através de análise clínica e de ressonância magnética (RM) que foram submetidos a cortico-amigdalo-hipocampectomia para o controle das crises. Os níveis de glutamato foram avaliados por HPLC, as citocinas TNFα e IL1β por ELISA e os níveis de NO via NO system. Avaliamos também por imuno-histoquímica a expressão de IL1β e CD45 em tecidos controles e com esclerose. Tecido controle foi obtido após autópsia de indivíduos mortos sem disfunções inflamatórias e neurológicas (n=10). A concentração de glutamato se mostrou reduzida no tecido TLE-MTS (p<0,001) sugerindo aumento na liberação desse neurotransmissor. TNFα e IL1β também apresentaram níveis elevados no hipocampo dos pacientes (p<0,05), demonstrando um processo inflamatório crônico. Houve uma correlação linear positiva entre a intensidade do sinal em FLAIR e os níveis de NO e IL1β. Em contraste, uma correlação linear negativa foi encontrada entre a intensidade do sinal em FLAIR e níveis de glutamato no hipocampo com esclerose. Infiltrado linfocitário hipocampal também foi visualizado pela imuno-marcação com CD45 em pacientes com TLE-MTS. Esses dados mostraram uma associação entre alteração de sinal na RM e marcadores inflamatórios em pacientes com TLE-MTS.
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Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Epilepsia del Lóbulo Temporal/metabolismo , Epilepsia del Lóbulo Temporal/patología , Hipocampo/patología , Mediadores de Inflamación/análisis , Imagen por Resonancia Magnética/métodos , Lóbulo Temporal/patología , Amígdala del Cerebelo/patología , /análisis , Epilepsia del Lóbulo Temporal/cirugía , Ácido Glutámico/análisis , Hipocampo/química , Hipocampo/cirugía , Interleucina-1/análisis , Interleucina-1beta/análisis , Óxido Nítrico/análisis , Esclerosis , Lóbulo Temporal/química , Factor de Necrosis Tumoral alfa/análisisRESUMEN
INTRODUÇÃO: A estereoeletroencefalografia (E-EEG), conforme introduzida na década de 50 por Talairach, é um método invasivo de análise tridimensional da zona epilpeptogênica, baseado na técnica de implantação intracraniana de eletrodos de profundidade (EP). O advento das modernas técnicas de cirurgia guiadas por imagem revolucionaram a técnica de implantação dos EP. OBJETIVO: O objetivo deste artigo é discutir os princípios da E-EEG e sua evolução, desde a era Talairach até a era atual, da cirurgia guiada por imagem, e suas perspectivas futuras. CONCLUSÕES: Embora os princípios gerais da E-EEG tenham permanecidos intactos ao longo dos anos, a implantação de EP, que é a técnica cirúrgica que viabiliza este método, sofreu uma tremenda evolução ao longo das últimas três décadas devido ao advento das modernas técnicas de imagem, de sistemas de computação e das novas técnicas estereotáxicas. O uso de sistemas robotizados, a evolução constante das técnicas de imagem e computação e a utilização de EP com sondas para micro diálise associados a si, abre no futuro uma enorme perspectiva para a aplicação dos EP e da E-EEG, tanto para uso investigativo como terapêutico. A descoberta de novos alvos, em localizações profundas e a fabricação de eletrodos "inteligentes", poderá incrementar, num futuro próximo, a necessidade do uso deste método.
INTRODUCTION: The stereoelectroencephalography (SEEG), introduced by Talairach in 50', is an invasive method of tridimensional analysis of epileptogenic zone based on the intracranial placement of depth electrodes (DE). The advent of modern imaging guided surgery had a tremendous impact in DE implantation techniques. OBJETIVE: The aim of this article is to discuss the main principles of SEEG and its evolution along the years since Talairach era until the imaging guide surgery era, with its new perspectives. CONCLUSIONS: Although the main principles of SEEG have remained intact, the placement of depth electrodes (DE) which is the surgical technique that supports this method has suffered a tremendous evolution along the last three decades due the advent of the modern imaging, the computer systems and the new stereotactic techniques. The use of robotic, the new imaging and computed systems and the use of probes of micro dialise adaptated to EP opened a tremendous perspective to DE and SEEG application as an investigative and therapeutical method. The discovery of new targets in deep brain localization and the manufacturing of "smart" DE, can increment, in a near future, the number of indications to this method.
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Humanos , Cirugía Asistida por Computador , Electrodos , Epilepsia/cirugíaRESUMEN
Introducción. La encefalitis de Rasmussen (ER) es una afección cerebral progresiva que provoca una atrofia hemisférica unilateral, disfunción neurológica y epilepsia refractaria. La hemisferotomía se considera actualmente el tratamiento más efectivo, pero algunos casos presentan particularidades que dificultan la decisión de realizarla. Objetivo. Evaluar la evolución posquirúrgica de niños con ER operados por hemisferotomía, que en la evaluación prequirúrgica presentaron algunas características que dificultaron la decisión quirúrgica. Pacientes y métodos. Se seleccionaron los casos con ER, atendidos en el Hospital São Paulo entre 2003 y 2012, que en la evaluación prequirúrgica presentaran evidencias clínicas, electroencefalográficas o en la neuroimagen de participación de ambos hemisferios cerebrales, afectación del hemisferio cerebral dominante, ausencia de déficit neurológico grave y ausencia de criterios de epilepsia refractaria y crisis atípicas. Para la evaluación posquirúrgica de las crisis epilépticas se utilizó la escala de Engel; para analizar la función motora, las escalas Gross Motor Function Classification System y Manual Ability Classification System, y el lenguaje se evaluó clínicamente. Resultados. Se seleccionaron seis casos (cuatro niñas), con edad media de inicio clínico de 3,3 ± 1,2 años (rango: 2-7 años) y edad media de hemisferotomía de 6,7 años (rango: 2,3-16,5 años). El tiempo medio de seguimiento posquirúrgico fue de tres años (rango: 0,5-7,2 años). En la evaluación posquirúrgica de las crisis epilépticas, cuatro casos se clasificaron como Engel clase I (66%); hubo mejoría en la función motora en cinco, y mejoría en el lenguaje, en todos. Conclusión. La hemisferotomía debe considerarse una opción eficiente de tratamiento en niños con ER (AU)
Introduction. Rasmussens encephalitis (RE) is a progressive pathology affecting the brain that causes unilateral hemispheric atrophy, neurological dysfunction and refractory epilepsy. Hemispherotomy is considered the most effective treatment today, but some cases present certain peculiarities that can seriously affect the decision to go ahead with this procedure. Aims. To evaluate the post-operative progress made by children with RE who have undergone hemispherotomy surgery, and who, in the pre-operative assessment, presented certain characteristics that complicated the decision to perform surgery. Patients and methods. The sample selected for study consisted of the cases of RE attended in the Hospital São Paulo between 2003 and 2012 who, in the pre-surgery evaluation, presented clinical, electroencephalographic or neuroimaging evidence of involvement of both brain hemispheres, compromise of the dominant brain hemisphere, absence of severe neurological deficit and absence of criteria for refractory epilepsy and atypical crises. The post-operative assessment of the epileptic seizures was evaluated using the Engel scale; motor function was analysed with the Gross Motor Function Classification System and Manual Ability Classification System scales, and language was evaluated clinically. Results. Six cases were selected (four girls), with a mean age at clinical onset of 3.3 ± 1.2 years (range: 2-7 years) and a mean age at hemispherotomy of 6.7 years (range: 2.3-16.5 years). The mean post-surgery follow-up time was three years (range: 0.5-7.2 years). In the post-surgery evaluation of the epileptic seizures, four cases were classified as Engel class I (66%); there was some improvement in motor functioning in five of them, and language improved in all cases. Conclusions. Hemispherotomy must be considered an efficient option for treatment in children with RE (AU)
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Humanos , Femenino , Preescolar , Niño , Encefalitis/rehabilitación , Encefalitis/cirugía , Calidad de Vida , Epilepsia/complicaciones , Epilepsia/diagnóstico , Neuroimagen/métodos , Neuroimagen , Lenguaje , Imagen por Resonancia Magnética/métodos , Imagen por Resonancia Magnética , Electroencefalografía/normas , Electroencefalografía , Neuroimagen/instrumentación , Neuroimagen/tendenciasRESUMEN
OBJECTIVE: To evaluate the prevalence of leukoaraiosis (LA) and its correlation with vascular risk factors and the cognitive performance of elderly patients. METHOD: 78 patients were randomly selected and submitted to clinic-laboratorial evaluation for vascular risk factors. Two cognitive tests were performed. All patients were submitted to cranial computerized tomography (CT), which was analyzed in two situations: the spontaneous and the directed way. RESULTS: There was no statistically significant difference between the prevalence of spontaneous and the directed diagnoses of LA (20.5 and 18 percent, respectively). The presence of LA was not significantly correlated with cognitive impairment. Only age was positively associated with LA. CONCLUSION: Cranial CT is a trustworthy method for the diagnosis of LA. Only age showed a positive association. No clinical implications were found, concerning the cognitive performance of the patients.
OBJETIVO: Avaliar a prevalência da leucoaraiose (LA) em idosos e correlacioná-los com fatores de risco vasculares e seu desempenho cognitivo. MÉTODO: 78 pacientes foram selecionados aleatoriamente e submetidos a avaliação clinico-laboratorial para fatores de risco vascular. Dois testes cognitivos foram realizados. Todos os pacientes foram submetidos a tomografia computadorizada (TC) de crânio, que foi analisada em duas situações: de forma espontânea e dirigida. RESULTADOS: Não houve diferença estatisticamente significativa entre as formas de diagnóstico espontâneo e dirigido da LA (20,5 and 18 por cento, respectivamente). Com relação ao desempenho cognitivo, a presença da LA não determinou prejuízo significativo. Somente a idade mostrou associação positiva com a LA. CONCLUSÃO: A TC de crânio é um método confiável para o diagnóstico de LA. A idade foi o único fator de risco que mostrou associação positiva. Não houve prejuízo quanto ao desempenho cognitivo.
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Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Trastornos del Conocimiento , Leucoaraiosis , Brasil/epidemiología , Enfermedad Crónica , Escolaridad , Leucoaraiosis/epidemiología , Prevalencia , Factores de Riesgo , Índice de Severidad de la Enfermedad , Tomografía Computarizada por Rayos XRESUMEN
OBJECTIVE: Early brain insults can cause cavitary lesions including porencephaly (POR) and multicystic encephalopathy (MCE). The objective of this study was to investigate clinical and electrographic correlates associated to these types of destructive brain lesions. METHOD: Patients with POR and MCE were selected and submitted to clinical and Video-EEG monitoring. The following variables were analyzed: demographic data, type of lesion, presence of gliosis, perinatal complications, epilepsy, brain atrophy, and presence and frequency of epileptiform discharges. RESULTS: Twenty patients were included, 65 percent males, 35 percent females, ages ranging from 1 to 40 years, 14 with MCE and 6 with POR. Eighteen patients had hemiparesis, 19 had epilepsy (current or in the past), seven of them had refractory seizures, and 16 had epileptiform discharges. All patients with MCE had gliosis while only 2 with POR had it. CONCLUSIONS: No correlation was observed between type of lesion and clinical and electrographical outcome. However, a positive correlation was observed between frequency of discharges and presence of brain atrophy, and between MCE and gliosis.
OBJETIVO: Insultos cerebrais precoces podem causar lesões cavitárias incluindo porencefalias (POR) e encefalomalacias multicisticas (EMC). O objetivo deste estudo foi investigar correlatos clínicos e eletrográficos associados a estes dois tipos de lesões destrutivas. MÉTODO: Pacientes com POR e EMC foram selecionados e submetidos à avaliação neurológica e monitorização vídeo-eletrencefalográfica, analisando-se as seguintes variáveis: dados demográficos, tipo de lesão, presença de gliose, complicações perinatais, epilepsia, atrofia cerebral, presença e freqüência de descargas epilépticas. RESULTADO: Vinte pacientes foram incluídos, sendo 65 por cento do sexo masculino, 35 por cento do feminino, idades entre 1 e 40 anos, sendo 14 com EMC e 6 com POR. Dezoito pacientes tinham hemiparesia, 19 tinham ou tiveram epilepsia (7 deles refratários ao tratamento medicamentoso) e 16 deles tinham paroxismos epileptiformes. Todos com MCE tinham gliose associada, contra apenas 2 dos pacientes com POR. CONCLUSÃO: Não houve correlação entre tipo de lesão e evolução clínica e eletrográfica. Houve, entretando, correlação positiva entre freqüência de descargas epilépticas e presença de atrofia cerebral, e entre lesão do tipo EMC e presença de gliose.
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Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Quistes del Sistema Nervioso Central/patología , Corteza Cerebral/patología , Encefalomalacia/patología , Atrofia/patología , Quistes del Sistema Nervioso Central/complicaciones , Quistes del Sistema Nervioso Central/fisiopatología , Corteza Cerebral/fisiopatología , Electroencefalografía , Encefalomalacia/complicaciones , Encefalomalacia/fisiopatología , Epilepsia/etiología , Epilepsia/patología , Gliosis/patología , Imagen por Resonancia MagnéticaRESUMEN
A esclerose lateral amiotrófica (ELA) é doença neurodegenerativa que afeta o trato córtico-espinhal. A escala funcional de avaliação em ELA (ALSFRS) é um questionário que quantifica clinicamente as perdas motoras, enquanto a imagem por tensor de difusão (DTI) avalia a integridade das fibras através da fração de anisiotropia (FA). No presente estudo, sete pacientes com ELA definida foram avaliados pela ALSFRS e imediatamente submetidos à DTI, obtendo valores de FA nas regiões: pedúnculo cerebral (PC), cápsula interna (CI) e substância barnca subjacente às áreas motora primária (M1), motora secundária (M2) e somestésica (SI). Um grupo controle foi constituído de doze indivíduos saudáveis. Os pacientes apresentaram valores de FA significativamente menores que os controles, com tendência à maior redução à direita e nas regiões mais caudais. Curiosamente, os valores de FA estavam reduzidos na área somestésica. Não foi observada correlação entre a duração dos sintomas e os valores de FA. Apesar da correlação entre os valores da ALSFRS e a degeneração em PC e CI, nossos resultados sugerem que essa escala subjetiva não é um bom parâmetro para a avaliação do dano estrutural nas porções encefálicas do trato corticoespinhal.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that affects the corticospinal tract. ALS functional rating scale (ALSFRS) is a questionnaire that quantifies motor deficits, while diffusion tensor imaging (DTI) evaluates the integrity of fibers through the fractional anisotropy (FA). In the present study, seven ALS patients were evaluated by ALSFRS and immediately submitted to DTI, getting FA values in the following regions: cerebral peduncle (PC), internal capsule (CI) and the white matter under the primary motor cortex (M1), secondary motor cortex (M2) and somestetic cortex (SI). A control group was constituted by twelve healthy individuals. FA values in patients were significantly lower when compared with controls, with a tendency to higher reductions in the right hemisphere and more inferior regions. Interestingly, FA values were reduced in somestetic area. No correlation was observed between symptoms duration and FA values. Despite the correlation observed between ALSFRS scores and degeneration in PC and CI, our results suggest that this subjective scale is not a good parameter for the evaluation of the structural damage in encephalic portions of the corticospinal tract.
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Femenino , Humanos , Masculino , Persona de Mediana Edad , Esclerosis Amiotrófica Lateral/patología , Imagen de Difusión por Resonancia Magnética , Tractos Piramidales/patología , Encuestas y Cuestionarios , Estudios de Casos y Controles , Reproducibilidad de los ResultadosRESUMEN
OBJECTIVE: To determine the frequency and regional involvement of temporal pole signal abnormality (TPA) in patients with hippocampal sclerosis (HS) using fluid-attenuated inversion-recovery (FLAIR) MR imaging, and to correlate this feature with history. METHOD: Coronal FLAIR images of the temporal pole were assessed in 120 patients with HS and in 30 normal subjects, to evaluate gray-white matter demarcation. RESULTS: Ninety (75 percent) of 120 patients had associated TPA. The HS side made difference regarding the presence of TPA, with a left side prevalence (p=0.04, chi2 test). The anteromedial zone of temporal pole was affected in 27 (30 percent) out of 90 patients. In 63 (70 percent) patients the lateral zone were also affected. Patients with TPA were younger at seizure onset (p=0.018), but without association with duration of epilepsy. CONCLUSION: Our FLAIR study show temporal pole signal abnormality in 3/4 of patients with HS, mainly seen on the anteromedial region, with a larger prevalence when the left hippocampus was involved.
OBJETIVO: Determinar a freqüência e o envolvimento regional da anormalidade de sinal do pólo temporal (APT) em pacientes com esclerose hipocampal (EH) utilizando seqüência inversão recuperação com supressão da água (FLAIR) por RM, e correlacioná-la com a história. MÉTODO: Foram analisadas as imagens coronais FLAIR dos pólos temporais de 120 pacientes com EH e de 30 indivíduos normais, para avaliar a demarcação entre substâncias branca e cinzenta. RESULTADOS: Noventa (75 por cento) dos 120 pacientes tinham APT associada. Houve prevalência do lado esquerdo (p=0.04, chi2 teste) na relação entre APT e o lado da EH. A zona ântero-medial estava acometida em 27 (30 por cento) destes pacientes. Em 63 (70 por cento) pacientes também a zona lateral estava acometida. Pacientes com APT apresentaram início da epilepsia quando mais jovens (p=0.018), porém sem associação com a sua duração. CONCLUSÃO: A seqüência FLAIR mostra haver ATP em 3/4 dos pacientes com EH, principalmente na região ântero-medial, com maior prevalência quando o hipocampo esquerdo estava envolvido.
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Adolescente , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Encefalopatías/patología , Epilepsia del Lóbulo Temporal/patología , Hipocampo/patología , Lóbulo Temporal/anomalías , Lóbulo Temporal/patología , Edad de Inicio , Encefalopatías/epidemiología , Brasil/epidemiología , Distribución de Chi-Cuadrado , Epilepsia del Lóbulo Temporal/epidemiología , Lateralidad Funcional , Imagen por Resonancia Magnética/métodos , Esclerosis , Procesamiento de Señales Asistido por Computador , Factores de TiempoRESUMEN
OBJETIVO: Estabelecer valores de referência para medidas de velocidade sanguínea e índices de impedância em indivíduos saudáveis por meio do Doppler transcraniano convencional (DTC) e observar suas variações em relação à idade e sexo. MÉTODO: Foram examinados 88 voluntários assintomáticos, sem antecedentes de doença cerebrovascular, cardíaca ou vascular periférica. Os exames de DTC foram realizados pelo mesmo examinador, sendo as artérias cerebrais médias, anteriores e posteriores estudadas por meio da janela temporal e as artérias vertebrais e basilar por meio da janela suboccipital. Foram arquivados os valores de velocidade média (VM), velocidade de pico sistólico (VPS), velocidade diastólica final (VDF) e índices de resistência (IR) e pulsatilidade (IP) de cada segmento arterial examinado. RESULTADOS: Os limites de referência encontrados por nós foram semelhantes aos descritos por outros autores para todos os parâmetros estudados. Houve tendência das velocidades de fluxo diminuírem e dos índices de impedância aumentarem com o avançar da idade. Os valores de velocidade nas mulheres foram superiores aos encontrados em homens. CONCLUSÃO: O DTC se mostrou útil na avaliação hemodinâmica cerebral de nossa população. Os nossos resultados são semelhantes aos de outros estudos, tanto nos valores de referência quanto na sua relação com a idade e o sexo.
OBJECTIVE: To establish reference values for measures of blood flow velocities and impedance indexes in healthy individuals through conventional transcranial Doppler (TCD) and to observe their variations in relation to the age and sex. METHOD: 88 asymptomatic volunteers were examined without antecedents of cardiac, peripheral or cerebrovascular diseases. The TCD were accomplished by the same examiner. The middle, anterior and posterior cerebral arteries were studied through the temporal acoustic window and the vertebral and basilar arteries through the suboccipital acoustic window. The values of mean velocity (MV), peak-systolic velocity (PSV), final diastolic velocity (FDV), resistance index (RI) and pulsatility index (PI) of each examined arterial segment were filed. RESULTS: The reference limits found by us were similar to the described by other authors for all of the studied parameters. There was tendency of the flow velocities decrease and of the impedance indexes increase with moving forward of the age. The values of velocities in the women were superior to the found in men. CONCLUSION: TCD was shown useful in the evaluation of hemodynamic cerebral for our population. Our results are similar to the other studies, so much in the reference values as in relationship with the age and the sex.
Asunto(s)
Adolescente , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Velocidad del Flujo Sanguíneo/fisiología , Encéfalo/irrigación sanguínea , Arterias Cerebrales , Ultrasonografía Doppler Transcraneal , Circulación Cerebrovascular , Arterias Cerebrales/fisiología , Valores de ReferenciaRESUMEN
INTRODUCTION: Somatosensory auras (SSAs) are more usually described in patients with parietal lobe epilepsy (PLE), producing more commonly a localized cutaneous tingling sensation, involving hands and fingers followed by tonic or clonic focal seizures. These usually originate in the contralateral hemisphere. Etiology includes dysplasias, tumours, ischemic or postencephalitic gliosis. However, other focal epilepsies, such as frontal and temporal, may also originate SSAs. Although this type of aura is reported as rare in patients with mesial temporal lobe epilepsy (MTLE), this association has not been systematically studied.OBJECTIVES: The aim of this article was to describe the cases of four patients with refractory MTLE and SSAs, reporting their clinical characteristics and MRI findings. We discuss the localizing and lateralizing value of SSAs, particularly in the context of MTLE. METHODS AND RESULTS: Four patients with refractory MTLE and SSAs followed-up in the outpatient's clinic at the Epilepsy Section, Universidade Federal de São Paulo, were submitted to presurgical evaluation and corticoamygdalohippocampectomy. MRI in all cases showed unilateral mesial temporal sclerosis (MTS). Regarding seizure semiology, tingling sensation involving the upper extremity was the most prevalent symptom. Three of the four patients had SSAs contralateral to the MTS. Following the SSAs all patients most of the time presented other symptoms such as autonomic or psychic auras evolving to psychomotor seizures. After surgical treatment, two of the patients presented infrequent auras, and two were rendered seizure-free. CONCLUSION: Although rare, SSAs can be present in MTLE. The characteristics of autonomic or psychic auras, psychomotor seizures, neuropsychological deficits, and typical neurophysiologic and MRI findings may help differentiate patients with MTLE from those with PLE
INTRODUÇÃO: Auras somatossensoriais (AS) são mais frequentemente descritas em pacientes com epilepsia de lobo parietal (ELP), produzindo sensação de formigamento cutâneo envolvendo mão e dedos seguida de sinais motores focais tônicos ou clônicos. Estas crises habitualmente originam-se no hemisfério cerebral contralateral. Displasias, tumores, patologias vasculares ou gliose pós encefalite constituem as etiologias mais prováveis. Outras epilepsias focais, como as de lobo frontal e temporal podem também originar (AS). Embora este tipo de aura seja considerada rara em pacientes com epilepsia mesial do lobo temporal (EMLT), esta associação ainda não foi sistematicamente estudada. OBJETIVOS: A proposta deste artigo foi descrever quatro casos de EMLT refratária ao tratamento clínico, apresentando AS, reportar as características clínicas comuns, achados de neuroimagem e estudar o valor localizatório e lateralizatório das mesmas. MÉTODOS E RESULTADOS: Quatro pacientes com EMLT refratária e AS acompanhados no ambulatório de Epilepsia, Universidade Federal de São Paulo, foram submetidos a avaliação pré-cirúrgica. Sensação de formigamento envolvendo a extremidade superior foi o sintoma predominante em todos. Em três (75%) as auras foram contralaterais à esclerose mesial temporal (EMT). Em todos as AS eram, com maior freqüência, seguidas por outras auras autonômicas e psiquícas e evoluiam para crises parciais complexas (crises psicomotoras). CONCLUSÃO: Embora raras, AS podem estar presentes em EMLT. A associação de auras autonômicas e psíquicas, presença de crises parciais complexas (psicomotoras), déficits neuropsicológicos distintos, além de achados neurofisiológicos e de neuroimagem podem diferenciar pacientes com EMLT daqueles com ELP.