Manejo dental de paciente con Síndrome de Hallervorden-Spatz: reporte de caso / Manejo odontológico de paciente com síndrome de Hallervorden-Spatz: relato de caso / Dental management of a patient with Hallervorden-Spatz syndrome: case report

El Síndrome de Hallervorden-Spatz es una afección neurodegenerativa rara, autosómica recesiva, caracterizada por depósitos en gran cantidad de hierro en los ganglios de base, lo que ocasiona gran pérdida motora y mental. Presenta dos formas de manifestación: la clásica, que aparece en la infancia a lo largo de la primera década de vida, y la atípica, cuyas manifestaciones clínicas aparecen de forma tardía, entre la segunda y tercera décadas de vida. El objetivo del presente estudio es describir un caso clínico de tratamiento endodóntico, en ambulatorio, de una paciente del sexo femenino, de 28 años, con manifestaciones clásicas de dicho síndrome, con cambio de color en el elemento 11 y lesión periapical, que justifica la indicación de endodoncia. La atención odontológica de una enfermedad neurodegenerativa rara, realizada en forma ambulatoria, requiere el conocimiento del dentista para que se conduzca de forma eficiente y segura

A síndrome de Hallervorden-Spatz é uma afecção neurodegenerativa rara, autossômica recessiva, caracterizada por depósitos em grande quantidade de ferro nos gânglios de base, o que ocasiona grande perda motora e mental. Apresenta duas formas de manifestações: a clássica, que surge na infância na primeira década de vida; e a atípica, cujas manifestações clínicas surgem mais tardiamente, entre a segunda e terceira décadas de vida. O objetivo desse estudo foi descrever um caso clínico de tratamento endodôntico, ambulatorial, de uma paciente do sexo feminino, 28 anos, com manifestações clássicas da síndrome, apresentando mudança de cor no elemento 11 com lesão periapical, justificando a indicação para endodontia. O atendimento odontológico de uma doença neurodegenerativa rara; realizado em ambiente ambulatorial, requer o conhecimento do dentista para que seja conduzido de forma eficiente e segura.

Hallervorden-Spatz syndrome is a rare, autosomal recessive neurodegenerative disorder characterized by large deposits of iron in the basal ganglia, which causes great motor and mental loss. It presents two forms of manifestations: the classic, which arises in childhood in the first decade of life; and the atypical, whose clinical manifestations appear later, between the second and third decades of life. The objective of this study was to describe a clinical case of endodontic outpatient treatment of a female patient, 28 years old, with classic manifestations of the syndrome, showing color change in element 11 with periapical lesion, justifying the indication for endodontics. The dental care of a rare neurodegenerative disease in an outpatient setting requires the dental surgeon's knowledge so that it is conducted efficiently and safely.

Leigh syndrome in an infant: autopsy and histopathology findings

Leigh syndrome is an inherited neurodegenerative disorder of infancy that typically manifests between 3 and 12 months of age. The common neurological manifestations are developmental delay or regression, progressive cognitive decline, dystonia, ataxia, brainstem dysfunction, epileptic seizures, and respiratory dysfunction. Although the disorder is clinically and genetically heterogeneous, the histopathological and radiological features characteristically show focal and bilaterally symmetrical, necrotic lesions in the basal ganglia and brainstem. The syndrome has a characteristic histopathological signature that helps in clinching the diagnosis. We discuss these unique findings on autopsy and radiology in a young infant who succumbed to a subacute, progressive neurological illness suggestive of Leigh syndrome. Our case highlights that Leigh syndrome should be considered in the differential diagnosis of infantile-onset, subacute neuroregression with dystonia and seizures, a high anion gap metabolic acidosis, normal ketones, elevated lactates in blood, brain, and urine, and bilateral basal ganglia involvement.

Encefalitis severa por el virus de Epstein-Barr en paciente inmunocompetente: reporte de caso / Severe encephalitis due to Epstein-Barr virus in an immunocompetent patient: case report

RESUMEN El virus de Epstein Barr (VEB) infecta a cerca del 90 % de las personas en la primera década de vida y, como los demás herpes virus, se mantiene en estado de latencia con riesgo de reactivaciones posteriores. La infección por VEB puede tener un curso asintomático o causar mononucleosis infecciosa (MI), especialmente en adolescentes y adultos. Rara vez, es responsable de infecciones en el sistema nervioso central como encefalitis, cerebelitis, mielitis, neuritis, encefalomielitis aguda diseminada y, más comúnmente, en relación con la infección por el virus de inmunodeficiencia humana (VIH), relacionado con el linfoma primario del sistema nervioso central (SNQ. La encefalitis por VEB no difiere en su presentación clínica de las demás encefalitis virales. Su diagnóstico se realiza mediante detección serológica de anticuerpos de respuesta aguda contra el virus o por reacción en cadena de la polimerasa (PCR) en líquido cefalorraquídeo (LCR). Las opciones terapéuticas en el momento son limitadas y con una baja evidencia de efectividad. A continuación, se presenta el caso de una mujer adulta, inmunocompetente, con clínica de encefalitis aguda severa y marcadas anormalidades en las neuroimágenes. El diagnóstico se hizo mediante la detección de ADN viral en LCR, apoyado por la exclusión de otros agentes patógenos y otras posibles etiologías mediante estudios microbiológicos, patológicos y serológicos.

SUMMARY Epstein Barr virus (EBV) infects about 90 % of people in the first decade of life and, like other herpes viruses, remains dormant with risk of subsequent reactivations. EBV infection can have an asymptomatic course or cause infectious mononucleosis (IM), especially in teenagers and adults. Rarely, it can be responsible for infections in the central nervous system such as encephalitis, cerebellitis, myelitis, neuritis, acute disseminated encephalomyelitis and, more commonly, primary lymphoma of the central nervous system (PLCNS) related to infection by human immunodeficiency virus (HIV). VEB encephalitis does not differ from other viral encephalitis in its clinical presentation. Its diagnosis is performed by serological detection of acute antibody response against the virus or by polymerase chain reaction (PCR) in cerebrospinal fluid (CSF). Treatment options are limited and with a low evidence of effectiveness. We report an unusual case of an immunocompetent mid adult woman, who presented clinically severe acute encephalitis and marked abnormalities in neuroimaging. The diagnosis was made by EBV DNA detection in CSF, supported by the exclusion of the presence of other pathogens and etiologies by microbiological and pathological studies.

Amnesia anterógrada debida a lesión bilateral de los hipocampos en un paciente probablemente intoxicado con metanol / Anterograde amnesia due to bilateral lesion of the hippocampus in a patient probably intoxicated with metanol

RESUMEN Se presenta el caso clínico de un hombre de 56 años que ingresó con un cuadro agudo de alteración de la conciencia luego de intoxicación alcohólica. Durante su hospitalización se evidenció alteración de la memoria anterógrada y neuropatía óptica derecha. Las imágenes diagnósticas demostraron lesiones bilaterales de los hipocampos, globos pálidos y corteza cerebral frontal paramediana. La evaluación neuropsicológica confirmó una grave amnesia anterógrada que mejoró muy poco al cabo de 2 años de seguimiento. A pesar de la falta de confirmación por el laboratorio, las manifestaciones clínicas y las imágenes están a favor de una intoxicación por metanol. Se llama la atención sobre esta rara presentación clínica.

SUMMARY We report the case of a 56-year-old man who was hospitalized because acute altered consciousness after alcoholic intoxication. During his hospitalization, there was observed an alteration of anterograde memory and right optic neuropathy. Diagnostic images showed bilateral lesions of the hippocampus, globus pallidus and paramedian cerebral frontal cortex. The neuropsychological evaluation confirmed a severe anterograde amnesia that barely improved after 2 years of follow up. Despite the lack of confirmation by the laboratory, the clinical manifestations and imaging results are in favor of methanol intoxication. Attention is drawn to this rare clinical presentation.

Interregional correlations of glucose metabolism between the basal ganglia and different cortical areas: an ultra-high resolution PET/MRI fusion study using 18F-FDG

Basal ganglia have complex functional connections with the cerebral cortex and are involved in motor control, executive functions of the forebrain, such as the planning of movement, and cognitive behaviors based on their connections. The aim of this study was to provide detailed functional correlation patterns between the basal ganglia and cerebral cortex by conducting an interregional correlation analysis of the 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) data based on precise structural information. Fifteen participants were scanned with 7-Tesla magnetic resonance imaging (MRI) and high resolution research tomography (HRRT)-PET fusion system using 18F-FDG. For detailed interregional correlation analysis, 24 subregions of the basal ganglia including pre-commissural dorsal caudate, post-commissural caudate, pre-commissural dorsal putamen, post-commissural putamen, internal globus pallidus, and external globus pallidus and 80 cerebral regions were selected as regions of interest on the MRI image and their glucose metabolism were calculated from the PET images. Pearson's product-moment correlation analysis was conducted for the interregional correlation analysis of the basal ganglia. Functional correlation patterns between the basal ganglia and cerebral cortex were not only consistent with the findings of previous studies, but also showed new functional correlation between the dorsal striatum (i.e., caudate nucleus and putamen) and insula. In this study, we established the detailed basal ganglia subregional functional correlation patterns using 18F-FDG PET/MRI fusion imaging. Our methods and results could potentially be an important resource for investigating basal ganglia dysfunction as well as for conducting functional studies in the context of movement and psychiatric disorders.

Isquemia talámica bilateral secundaria a trombosis venosa cerebral: tratamiento endovascular, presentación de un caso / Bilateral thalamic stroke due to cerebral vein thrombosis: endovascular treatment, case report

RESUMEN La trombosis venosa cerebral (TVC) es una entidad poco frecuente y potencialmente devastadora que se presenta con mayor frecuencia en adultos jóvenes, especialmente en mujeres. La TVC representa el 0,5 % a 1 % de todos los ACV, su amplio espectro clínico la convierte en un desafío diagnóstico, especialmente en aquellos casos de mujeres jóvenes sin factores protrombóticos conocidos que debutan con cefalea como único síntoma. La TVC puede ocasionar isquemia o infartos talámicos y gangliobasales bilaterales con delirio y deterioro del estado de conciencia, en especial cuando hay compromiso del sistema venoso profundo. El diagnóstico se confirma con resonancia magnética cerebral (RM) y venografía por resonancia. La anticoagulación sigue siendo la piedra angular del tratamiento; sin embargo, los pacientes que no responden a terapia médica intensiva pueden beneficiarse de la trombectomía endovascular mecánica (TEM). A continuación presentamos el caso clínico de una paciente de 29 años con una isquemia talámica y gangliobasal bilateral secundaria a trombosis venosa cerebral que fue llevada a trombectomía endovascular mecánica.

SUMMARY Thrombosis of the dural sinus and/or cerebral veins (CVT) is a rare but potentially devastating type of stroke that tends to occur in young adults, especially women. CVT represents about 0.5% of all strokes and can be challenging to diagnose because headache, rather than focal neurologic symptoms, is the prominent feature. However, some patients (especially those with deep cerebral venous occlusion) may present with bilateral thalamic or basal infarction and often will have decreased level of consciousness and rapid neurologic deterioration. The diagnosis of CVT is confirmed with MRI and magnetic resonance venogram (MRV). The mainstay of acute management is anticoagulation. However, some patients do not respond to medical therapy and these might benefit from endovascular mechanical thrombectomy. We present the case of a 29 year old female patient with bilateral thalamic and gangliobasal ischemia secondary to CVT, who was treated with anticoagulation and endovascular mechanical thombectomy.

Distonías secundarias / Secondary dystonia

RESUMEN Las distonías que se generan por una causa específica (secundaria), son menos frecuentes que las distonías primarias (genéticas). Las causas son multifactoriales, incluyendo lesiones puntuales en alguna estructura cerebral, así como toxinas, medicamentos o infecciones.

SUMMARY Dystonia emerging from a specific cause (secondary dystonia) are by far less frequent that genetic counterpart (primary dystonia). Some of these causes include focal brain lesions, toxins, drugs and infections among many others.

Cognitive dysfunction in corticobasal degeneration / Disfunção cognitiva na degeneração corticobasal

ABSTRACT Corticobasal degeneration (CBD) was originally described as a distinct clinicopathological entity in 1967. Since then, different phenotypic presentations have emerged as possible manifestations of CBD histopathological findings. In addition, pathophysiological findings and the molecular basis have been delineated and several aspects of its cognitive manifestations have been clarified. Thus, not only the spectrum of what is currently designated as CBD has expanded, but overlap with other degenerative and even secondary disorders has made clinical diagnostic certainty even more challenging in the absence of specific and readily-available markers. Cognitive deficits in CBD are now recognized as a frequent initial presentation and may appear up to eight years before the motor symptoms, depending on the phenotypic variant. Characteristic cognitive features of CBD involve language deficits, visuospatial and executive dysfunctions, apraxia, and behavioral disorders. Semantic and episodic memories are usually preserved, while language is often impaired in the early stages.

RESUMO A degeneração corticobasal (DCB) foi originalmente descrita como uma entidade clínico-patológica distinta em 1967. Desde então, nossa compreensão sobre DCB evoluiu substancialmente. Diferentes apresentações fenotípicas emergiram refletindo possíveis manifestações das anormalidades histopatológicos da DCB. Adicionalmente, dados fisiopatológicos e moleculares foram delineados e aspectos das manifestações cognitivas foram explorados. Assim, não só o espectro do que é atualmente designado DCB foi expandido, mas a sobreposição com outras doenças degenerativas e até mesmo secundárias tornaram o diagnóstico clínico ainda mais desafiador na ausência de marcadores específicos e prontamente disponíveis. Déficits cognitivos na DCB são agora reconhecidos frequentemente como apresentações iniciais e podem surgir até 8 anos antes dos sintomas motores, dependendo da variante fenotípica. O quadro cognitivo envolve característicamente déficits de linguagem, disfunção visuoespacial e executiva, apraxia, e distúrbios comportamentais. Anormalidades da linguagem são frequentemente descritas nos estágios iniciais da DCB.

Caso 24 / Case 24

Trata-se de apresentação da Análise da Imagem de lesões cutâneas e intracranianas reveladas pelo exame clínico direto e radiológico, em que características clínico-epidemiológicas e de exames complementares permitem a identificação de agente etiológico mais provável, e revela gravidade extrema de nosologia eminentemente brasileira, que requer alerta para seu diagnóstico precoce e tratamento adequado para impedir sequelas e morte precoce. (AU)

This is a presentation of the Image Analysis of cutaneous and intracranial lesions revealed by direct clinical and radiological examination, in which clinical-epidemiological and complementary features allow the identification of the most probable etiological agent, and reveals extreme severity of nosology in Brazil, which requires alertness for its early diagnosis and appropriate treatment to prevent sequelae and early death. (Au)

Abnormal eye movements in three types of chorea / Anormalidades dos movimentos oculares em três tipos de coreia

ABSTRACT Chorea is an abnormal movement characterized by a continuous flow of random muscle contractions. This phenomenon has several causes, such as infectious and degenerative processes. Chorea results from basal ganglia dysfunction. As the control of the eye movements is related to the basal ganglia, it is expected, therefore, that is altered in diseases related to chorea. Sydenham’s chorea, Huntington’s disease and neuroacanthocytosis are described in this review as basal ganglia illnesses that can present with abnormal eye movements. Ocular changes resulting from dysfunction of the basal ganglia are apparent in saccade tasks, slow pursuit, setting a target and anti-saccade tasks. The purpose of this article is to review the main characteristics of eye motion in these three forms of chorea.

RESUMO Coreia é um movimento anormal caracterizado pelo fluxo contínuo de contrações musculares ao acaso. Este fenômeno possui variadas causas, como processos infecciosos e degenerativos. A coreia resulta de disfunção dos núcleos da base, os quais estão envolvidos no controle da motricidade ocular. É esperado, então, que esta esteja alterada em doenças com coreia. A coreia de Sydenham, a doença de Huntington e a neuroacantocitose são apresentadas como modelos que têm por característica este distúrbio do movimento, por ocorrência de processos que acometem os núcleos da base. As alterações oculares decorrentes de disfunção dos núcleos da base se manifestam em tarefas de sacadas, perseguição lenta, fixação de um alvo e em tarefas de antissacadas. O objetivo deste artigo é revisar as principais características dos movimentos oculares nestas três formas de coreias.

On the centenary of the birth of Francis H. C. Crick – from physics to genetics and neuroscience / O centenário de nascimento de Francis H.C. Crick – da física para a genética e a neurociência

ABSTRACT The year 2016 marks the centenary of the birth of Francis Crick (1916–2004), who made outstanding contributions to genetics and neuroscience. In 1953, in a collaborative study, Francis Crick and James Watson discovered the DNA double helix, and in 1962 they and Maurice Wilkins were awarded the Noble Prize in Physiology or Medicine. Crick subsequently became very interested in neuroscience, particularly consciousness and its relationship to the claustrum, a small gray matter structure between the insula and putamen.

RESUMO O ano de 2016 é o centenário de nascimento de Francis Crick (1916–2004), físico, biólogo e neurocientista, cujas contribuições para a genética e a neurociência foram magníficas. Crick, em um estudo colaborativo com Watson, descobriu a estrutura molecular do DNA (dupla hélice) em 1953, e em 1962 ambos receberam o prêmio Nobel de Fisiologia ou Medicina, junto com Wilkins. Após Crick tornou-se muito interessado na área de neurociência, particularmente no estudo da consciência, e a sua relação com o claustrum, uma pequena estrutura de substância cinzenta localizada entre a ínsula e o putame.

Magnetic ressonance imaging in the diagnosis of Creutzfeldt-Jakob disease: report of two cases / Ressonância magnética no diagnóstico da doença de Creutzfeldt-Jakob: relato de dois casos

ABSTRACT Creutzfeldt-Jacob disease (CJD) is a rare condition caused by a pathogenic prion protein that evolves with rapidly progressive dementia and death. The clinical presentation may sometimes be misleading. Magnetic Resonance Imaging (MRI) aids diagnosis with patterns that can guide or confirm clinical hypotheses. Two cases of rapidly progressive dementia with ataxia, myoclonus and restricted diffusion on MRI in cortical/basal ganglia are presented to draw attention to CJD.

RESUMO Doença de Creutzfeldt-Jacob (CJD) é uma rara doença relacionada a uma proteína priônica patogênica que evolui com demência rapidamente progressiva e morte. Por vezes, a apresentação clínica é inespecífica e desafiadora. A ressonância magnética contribui para o diagnóstico com padrões de imagem que podem orientar ou confirmar as hipóteses diagnósticas baseadas na clínica. Serão apresentados dois casos de pacientes com a forma esporádica da doença.

Optical coherence tomography imaging of the basal ganglia: feasibility and brief review

Optical coherence tomography (OCT) is a promising medical imaging technique that uses light to capture real-time cross-sectional images from biological tissues in micrometer resolution. Commercially available optical coherence tomography systems are employed in diverse applications, including art conservation and diagnostic medicine, notably in cardiology and ophthalmology. Application of this technology in the brain may enable distinction between white matter and gray matter, and obtainment of detailed images from within the encephalon. We present, herein, the in vivo implementation of OCT imaging in the rat brain striatum. For this, two male 60-day-old rats (Rattus norvegicus, Albinus variation, Wistar) were stereotactically implanted with guide cannulas into the striatum to guide a 2.7-French diameter high-definition OCT imaging catheter (Dragonfly™, St. Jude Medical, USA). Obtained images were compared with corresponding histologically stained sections to collect imaging samples. A brief analysis of OCT technology and its current applications is also reported, as well as intra-cerebral OCT feasibility on brain mapping during neurosurgical procedures.

Nuestra experiencia en el manejo de los cavernomas de tronco, de tálamo y de ganglios basales / Our experience in the management of trunk, thalamus and basal ganglia cavernomas

OBJETIVO: Presentar nuestra experiencia en el manejo de los cavernomas de tronco cerebral, de tálamo y de ganglios basales. MATERIAL Y MÉTODO: Analizamos una serie de 16 pacientes asistidos en nuestra Institución, entre enero de 1990 y diciembre del año 2013. De ellos, 9 fueron varones y 7 mujeres. El rango de edad osciló entre 3 y 61 años. RESULTADOS: Siete debutaron con hemorragia cerebral, de ellos 4 se localizaban en protuberancia y 3 en el bulbo raquídeo. Siete pacientes tuvieron cavernomas múltiples, de ellos 3 tenían familiares con la misma enfermedad. El procedimiento diagnóstico de elección fue la resonancia nuclear magnética de cerebro en todos, y en los pacientes con cavernomas múltiples se completó el estudio con resonancia nuclear magnética de médula espinal. El tratamiento fue conservador en 9 enfermos, quirúrgico en 6 y radiocirugía estereotáctica en 1 enfermo; a éste paciente hubo necesidad de operarlo 6 meses después del tratamiento radiante por un resangrado voluminoso en la protuberancia. DISCUSIÓN: La cirugía es exitosa cuando el cavernoma se ubica a 2 mm de la piamadre, o del epéndimo. La radiocirugía puede ser causante de resangrado y de mayor volumen que las hemorragias previas. Por último, el tratamiento conservador sigue teniendo vigencia en los pacientes que se recuperaron neurológicamente y cuando se ubican en la profundidad del tronco cerebral, tálamo óptico o ganglios basales. CONCLUSIÓN: Cada paciente debe evaluarse individualmente para decidir el tipo de tratamiento, teniendo en cuenta la edad, la recuperación de los signos neurológicos, el volumen y la localización precisa del cavernoma

Objective: To present our experience in the management of brainstem, thalamus and basal ganglia cavernous malformations. Material and Method: We analyzed a series of 16 patients admitted to our Institution between January 1990 and December 2013. Nine of them were male and 7 female. Age ranged between 3 and 61. Results: Seven patients presented brainstem hemorrhage, 4 being pontine and the remaining 3 were medullary. Seven patients had multiple cavernomas, and 3 of them had a family background with the disease. The chosen diagnostic procedure was brain MRI in all patients; in patients with multiple cavernomas spine MRI was also requested. Nine patients received conservative treatment, 6 patients underwent surgery and one was treated with stereotactic radiosurgery but had to be operated on six months after radiation treatment due to voluminous re-bleeding at protuberance. Discussion: Surgery is successful when the cavernous malformation is placed 2 mm away from pia mater or ependyma. Radiosurgery can cause re-bleeding and of a greater volume than previous hemorrhages. Finally, conservative treatment is useful in patients who get neurologically recovered and when malformations are placed deep in brainstem, optic thalamus or basal ganglia. Conclusion: Each patient has to be individually assessed to individually assessed to make a decision regarding the type of treatment, taking into account age, recovery of neurological signs, volume, and precise location of cavernous malformation

Encefalopatía e infarto del globus pallidum bilateral, secundarios aintoxicación con disulfiram. Reporte de caso pediátrico / Encephalopathy and bilateral globus pallidum infarction secondary to disulfiram poisoning. Case reports in pediatrics

El disulfiram (Antabuse®) es un fármaco que se usa ampliamente en pacientes con consumo crónico de alcohol,como parte del tratamiento del alcoholismo, con el fin de producir síntomas desagradables al mezclarsecon etanol, por incremento de acetaldehído. Aunque se han descrito casos de intoxicación aun sin ingesta dealcohol con dosis terapéuticas y más comúnmente por sobredosis en adultos, son pocos los casos que se handescrito en niños.Se presenta a continuación el caso de un niño con intoxicación aguda por disulfiram, quien presentó en la faseaguda somnolencia que evolucionó a estupor; concomitantemente presentó crisis convulsivas, vómito, deshidratación,rigidez, temblor distal y diaforesis; al egreso, con secuelas neurológicas severas, caracterizadas pordistonía bilateral y signos piramidales como resultado de lesión bilateral de los ganglios basales documentadapor imagenología.

Disulfiram (Antabuse ®) is a drug that is widely used in patients with chronic alcohol consumption as part of treatment for alcoholism, in order to produce unpleasant symptoms when mixed with ethanol, acetaldehyde increased.Although there have been cases of poisoning even without alcohol intake at therapeutic doses and overdose most commonly in adults, few cases have been reported in children. We present a Child with disulfiram intoxication, who presented in acute sleepiness, progressing to stupor concomitantly presented seizures, vomiting, dehydration, rigidity, tremor and sweating; in output with severe neurological sequelae characterized by bilateral dystonia and pyramidal signs as a result of bilateral basal ganglia lesion documented by imaging.

Estructuras cerebrales implicadas en el procesamiento temporal / Brain structures involved in temporal processing

El tiempo siempre ha sido una constante regulatoria del comportamiento humano. Durante las últimas dos décadas, la neurociencia ha buscado las bases biológicas al igual que la localización específica de estructuras o sistemas que el cerebro humano utilice en la ejecución, interpretación o retención de procesamientos temporales. La presente revisión reúne gran parte de los estudios actuales enfocados en la ubicación de las estructuras que involucran lapsos de retención, cronometraje o procesamiento temporal, reportando evidencia respecto a diversas estructuras involucradas, como lo son los ganglios basales; en la activación del putamen respecto a conteos dentro de respuestas motoras; Al cerebelo, en relación a intervalos de duración relativamente breves de tiempo, que van desde 300, 400, 600 y 800 ms, hasta 1-2 o 12-24 segundos; En la relación entre amígdala y corteza insular, en la ejecución de cronometraje controlado; En corteza frontal/pre-frontal, asociado a períodos breves (menores a 1 s) o al menor rendimiento de procesos atencionales o de memoria, propios del funcionamiento ejecutivo; y corteza parietal, asociada a la comparación consiente de las duraciones en rango de segundos (intervalos de 1 s) en relación a señales auditivas y visuales, así como en la codificación espacial y el procesamiento de cantidades numéricas. La localización de estas estructuras es la base para el desarrollo de modelos de estudio e integración de sistemas cada vez más precisos respecto a cómo el cerebro humano interpreta el tiempo...

Time has always been a constant regulatory human behavior. During the past two decades, neuroscience has sought the biological basis as the specific location of structures or systems that use the human brain in the execution, interpretation or retention of time processing. The present revision gathers much of the current research focused on the location of structures that involve retention periods, timing or temporal processing, reporting evidence regarding various structures involved, such as the basal ganglia, in the putamen regarding activation counts within motor responses; The cerebellum, in relation to a relatively short duration intervals of time, ranging from 300, 400, 600 and 800 ms, 1-2 or 12-24 seconds; In the relationship between amygdale and insular cortex, in performing timing control; In frontal/prefrontal cortex, associated with brief periods (less than 1-s) or lower yield of attention or memory processing, typical of executive functioning; And parietal cortex associated consents comparison of durations in seconds range (1-s intervals) in relation to auditory and visual cues, as well as spatial encoding and processing numerical quantities. The location of these structures is the basis for the development of study models and integration systems increasingly accurate as to how the human brain interprets the time...

Encefalopatía por Kernicterus. serie clínica / Kernicterus (bilirubin encephalopathy): case reports

Introducción: La ictericia es común en los recién nacidos (RN). Niveles de bilirrubina a partir de 20 mg/dL (en RN de término) pueden causar parálisis cerebral coreoatetósica, hipoacusia sensorioneural, trastornos de la mirada y displasia del esmalte dental, cuadro clínico conocido como kernicterus. Objetivo: Describir 5 casos de kernicterus controlados en una Unidad de Neurología, entre los años 2002-2012. Casos clínicos: Se presentan 5 niños con edades gestacionales entre 35 y 39 semanas, con peso de nacimiento rango 2.580 y 4.250 g y niveles de bilirrubina entre 24 y 47 mg/dL. Dos RN estaban en su domicilio cuando iniciaron la encefalopatía aguda. Todos se trataron con fototerapia y en 3 casos se realizó además exanguineotransfusión. La edad del diagnóstico de kernicterus fluctuó entre los 12 días y 10 años (3 pacientes se diagnosticaron en etapa neonatal) con una resonancia magnética que demostró impregnación de ganglios basales. Todos evolucionaron con trastornos del movimiento de severidad variable. En 3 pacientes se diagnosticó hipoacusia sensorioneural y en dos hubo trastornos de la mirada. Los test psicométricos evaluaron retraso cognitivo en 3 pacientes y desarrollo normal en los restantes. Conclusión: El kernicterus en una enfermedad devastadora que aún está presente en la realidad nacional. Es una causa de parálisis cerebral prevenible, por lo cual es necesario educar a los padres, población y equipo de salud para la detección precoz y tratamiento oportuno de la hiperbilirrubinemia neonatal.

Introduction: Jaundice is common in newborn babies (NB). Bilirubin levels of 20 mg/dL or higher may cause choreoathetoid cerebral palsy, sensorineural hearing loss, eye disorders and enamel dysplasia in term infants; clinical picture compatible with kernicterus. Objective: To describe five cases of kernicterus treated at a Neurology Unit between 2002 and 2012. Case reports: Five cases of babies with gestational ages between 35 and 39 weeks, birth-weight ranging from 2580 to 4250 grams and bilirubin levels between 24 and 47 mg/dL are presented. Two infants were at home when acute encephalopathy developed, all were treated with phototherapy and 3 of them underwent exchange transfusion. The age of diagnosis of kernicterus was between 12 days to 10 years; three patients were diagnosed in neonatal period through MRI that revealed basal ganglia impregnation. All patients evolved presenting movement disorders of varying severity. Three of them were diagnosed with sensorineural hearing impairments and two presented eye disorders. Psychometric tests showed cognitive delay in three patients and normal development in the remaining children. Conclusion: Kernicterus in a devastating disease present in the national reality. It is a preventable cause of cerebral palsy; therefore, it is necessary to educate parents, population and health care professionals about neonatal hyperbilirubinemia early detection and treatment.