Alucinosis peduncular en esclerosis múltiple, síntoma poco común en la expresión de enfermedad desmielinizante / Peduncular hallucinosis in multiple sclerosis, a rare symptom in demyelinating disease

Introducción: La alucinosis peduncular (AP) hace referencia a alucinaciones autodiscriminadas, cuyo origen son lesiones en el mesencéfalo y en el puente. Presentación del caso: Paciente 27 años, femenina, con alucinaciones visuales, auditivas autodiscriminadas por ella misma, sin antecedentes previos de importancia y con lesiones en resonancia magnética cerebral y cervical en el pedúnculo cerebeloso superior, tegmento pontino, y en columna cervical con bandas oligoclonales patrón 2, que cumplían criterios de Mc Donalds para esclerosis múltiple. Discusión: La alucinosis peduncular hace referencia a la presencia de alucinaciones visuales, criticadas por el paciente, con la consecuencia de lesiones de las vías inhibitorias por deaferentación y desinhibición mesencéfalotalámicas, y retinogenículo calcarina, descritas como manifestación de múltiples patologías neurológicas como trauma, afectación vascular, tumores y pocos casos de enfermedad desmielinizante, entre otras. Conclusión: La alucinosis peduncular es una forma atípica de presentación de lesiones pontomesencefálicas descritas en varias patologías; se debe tener en cuenta en la localización de la lesión neurológica; se han reportado pocos casos como síntoma de la enfermedad desmielinizante.

Introduction: Peduncular hallucinosis (PA) refers to self-discriminating hallucinations, these are caused by lesions in the midbrain and pons. Presentation of the case: 27-year-old right handed female patient with visual and auditory hallucinations self-discriminated by the patient, with no prior history of importance and with lesions in cerebral and cervical Magnetic Resonance in the superior cerebellar peduncle, pontine tegmentum, and in the cervical spine with pattern 2 oligo clonal bands, which met Mc Donald's criteria for multiple sclerosis. Discussion: Peduncular hallucinosis refers to the presence of visual hallucinations criticized by the patient, consequence of lesions in the inhibitory pathways with deafferentation and disinhibition of the midbrain-thalamic and retinogeniculus-calcarine pathways. Described as a manifestation of multiple neurological pathologies such as trauma, vascular, tumor and few cases of demyelinating among others. Conclusion: Peduncular hallucinosis is an atypical form of presentation of pontomesencephalic lesions described in several pathologies, it must be taken into account when locating the neurological lesion, few cases have been reported as symptom of the demyelinating disease.

S100 proteins and their implication in the thalamus: an integrative review / Las proteínas S100 y su implicación en el tálamo: una revisión integradora

SUMMARY: S100 proteins belong group of calcium-binding proteins and are present in physiological intracellular and extracellular regulatory activities, such as cell differentiation, and act in inflammatory and neoplastic pathological processes. Recently, its expressions in the nervous system have been extensively studied, seeking to elucidate its action at the level of the thalamus: A structure of the central nervous system that is part of important circuits, such as somatosensory, behavioral, memory and cognitive, as well as being responsible for the transmission and regulation of information to the cerebral cortex. This article is an integrative review of scientific literature, which analyzed 12 studies present in Pubmed. The analysis showed that the relationship of S100 proteins and the thalamus has been described in neoplastic processes, mental disorders, hypoxia, trauma, stress, infection, Parkinson's disease and epilepsy. In summary, it is possible to conclude that this protein family is relevant as a marker in processes of thalamic injury, requiring further studies to better understand its clinical, preclinical meanings and its prognostic value.

Las proteínas S100 pertenecen al grupo de proteínas fijadoras de calcio y están presentes en actividades reguladoras fisiológicas intracelulares y extracelulares, como la diferenciación celular, y actúan en procesos patológicos inflamatorios y neoplásicos. Recientemente, sus expresiones en el sistema nervioso han sido ampliamente estudiadas, buscando dilucidar su acción a nivel del tálamo: una estructura del sistema nervioso central que forma parte de importantes circuitos, como el somatosensorial, conductual, de memoria y cognitivo, así como además de ser responsable de la transmisión y regulación de la información a la corteza cerebral. Este artículo es una revisión integradora de la literatura científica, que analizó 12 estudios presentes en Pubmed. El análisis mostró que la relación de las proteínas S100 y el tálamo ha sido descrita en procesos neoplásicos, trastornos mentales, hipoxia, trauma, estrés, infección, enfermedad de Parkinson y epilepsia. En resumen, es posible concluir que esta familia de proteínas es relevante como marcador en procesos de lesión talámica, requiriendo más estudios para comprender mejor su significado clínico, preclínico y su valor pronóstico.

Prenatal diagnosis of fetal diencephalic-mesencephalic junction dysplasia: a case report / 中华围产医学杂志

We report a case of fetal diencephalic-mesencephalic junction dysplasia (DMJD) diagnosed prenatally. Prenatal ultrasound at 24 gestational weeks showed that the fetus was small, about the size at 22 weeks' gestation, with short biparietal diameter and enhanced echo at the anterior border of thalamus. Fetal MRI showed short T2 signal shadow in the left choroid plexus, and hemorrhage and midbrain dysplasia were suspected. A pathogenic homozygous mutation variant in protocadherins 12 gene (c.1558C>T) was found in this fetus by whole exome sequencing and both parents carried the same heterozygous variation revealed by Sanger sequencing. All of the above information lead to the diagnosis of fetal DMJD, and the pregnancy was terminated after genetic counseling.

Cerebral localization of higher functions: anatomic structures before the identification of their memory function / Localização cerebral de funções superiores: estruturas anatômicas antes da identificação de suas funções de memória

The nature of memory and the search for its localization have been a subject of interest since Antiquity. After millennia of hypothetical concepts the core memory-related structures finally began to be identified through modern scientifically-based methods at the diencephalic, hippocampal, and neocortical levels. However, there was a clear temporal delay between the finding of these anatomic structures ignoring their function, and their identification related to memory function. Thus, the core structures begun to be identified with a pure anatomical view in the late Middle Ages on, while the memory function related to them was discovered much later, in the late Modern Period.

A natureza da memória e a busca de sua localização tem sido objeto de interesse desde a Antiguidade. Após milênios de conceitos hipotéticos as estruturas centrais relacionadas com a memória finalmente começaram a ser identificadas através de métodos modernos com base científica, nos níveis diencefálico, hipocampal e neocortical. Entretanto, houve um claro retardo temporal entre o achado dessas estruturas anatômicas ignorando sua função e sua identificação relacionada à função da memória. Assim, as estruturas centrais começaram a ser identificadas com uma visão puramente anatômica da Idade Média tardia em diante, enquanto a função da memória relacionada com as mesmas foi descoberta muito mais tarde, no Período Moderno tardio.

Cerebral localization of higher functions: Memory-related anatomic structures / Localização cerebral de funções superiores: estruturas anatômicas relacionadas com a memória. achados iniciais

ABSTRACT The nature of memory and the search for its localization have been a subject of interest since Antiquity. After millennia of theoretical concepts, shifting from the heart to the brain, then from the ventricles to solid parts, the core memory-related structures finally began to be identified through modern scientifically-based methods at the diencephalic and cortical (hippocampal and neocortical) levels, mostly in the late Modern period, culminating in the current state of knowledge on the subject.

RESUMO A natureza da memória e a busca de sua localização tem sido objeto de interesse desde a Antiguidade. Após milênios de conceitos teóricos, mudando do coração para o cérebro e daí dos ventrículos para as partes sólidas, as estruturas centrais relacionadas com a memória finalmente começaram a ser identificadas através de métodos modernos com base científica, nos níveis diencefálico e cortical (hipocampal e neocortical), principalmente no período Moderno tardio, aproximando-se do estado atual do conhecimento sobre o tema.

Effect of light intensity on expression of gonadotropin-releasing hormone in mesencephalon and diencephalon of Jinghong-1 hens / 解剖学报

Objective To explore the nervous regulating mechanism in the effect of light intensity on reproductive performance. Methods Sixty healthy Jinghong-1 hens aged 100 days were randomly divided into four groups of 15 hens and applied a light of 30 lx, 20 lx, 10 lx, and 1 lx, respectively, for 7 days. The distribution and expression of gonadotropin-releasing hormone (GnKH) in mesencephalon and diencephalon were detected using immunohistochemical and image analysis methods. Results GnKH immunoreactive positive neurons were mainly observed in stratum griseum centrale (SCC) of tectum, nucleus isthmi pars magnocellularis (Imc) , nucleus isthmi pars parvocellularis (Ipc) , nucleus subpre-tectalis/interstito-pretectalis-subpretectalis (SP/IPS), nucleus of basal optic root (nbor), and nucleus semilunaris (Slu) of mesencephalon. The mean gray values of positive neurons in 30 lx and 20 lx groups were significantly higher than those of 10 lx and 1 lx groups (P0. 05) between 30 lx and 20 lx groups, but they were significant higher than those of 10 lx and 1 lx (P<0. 05) in PHN, PVN, and GLv. The percent cell area in Hot was the largest in 30 lx group, having no significant difference comparing 20 Ix, being significant higher than those of 10 lx and I Ix (P<0. 05). Those in 30 lx and 20 lx groups were significant higher than 10 lx and 1 lx (P<0.05) in PHN and PVN. Conclusion Light intensity affect the expression of GnRH in hen mesencephalon and diencephalon obviously in Jinghong-1 hens aged 100 days, regulating their reproductive function. The tectofugal pathway of the optical pathways, including collateral circulation of nucleus isthmi, plays an important role in the course of regulation.

Intermediate pilomyxoid astrocytoma and diencephalic syndrome: imaging findings / Astrocitoma pilomixoide intermediário e síndrome diencefálica: aspectos de imagem

Pilomyxoid astrocytoma, an entity described as a histological variant of pilocytic astrocytoma, is a rare primary tumor of the central nervous system. It is usually located in the hypothalamic-chiasmatic area, affecting children with a mean age of 10 months. It has a high rate of recurrence and cerebrospinal fluid dissemination, which may be present throughout the neuroaxis. Due to its topography, it may present developmental delay in childhood and diencephalic syndrome, characterized by extreme weight loss, lack of fat accumulation, hyperactivity, euphoria and alertness. Magnetic resonance imaging has an important role in its diagnosis, staging and follow-up of pilomyxoid astrocytoma. However, for a definitive diagnosis, anatomopathology is particularly important to differentiate it from pilocytic astrocytoma. Some cases, as in this present one, have simultaneous histological features of pilocytic and pilomyxoid astrocytomas, constituting a group called intermediate pilomyxoid astrocytoma. Surgery is the best treatment option and it usually requires adjuvant therapy.

O astrocitoma pilomixoide, entidade descrita como variante histológica do astrocitoma pilocítico, é um raro tumor primário do sistema nervoso central. Geralmente, localiza-se em topografia hipotálamoquiasmática, acomentendo crianças com idade média de 10 meses. Apresenta alta taxa de recorrência e disseminação liquórica, podendo se apresentar ao longo de todo o neuroeixo. Dada sua topografia, pode se apresentar com atraso do desenvolvimento na infância e síndrome diencefálica, caracterizada por emagrecimento extremo, ausência de acúmulo de tecido adiposo, hiperatividade motora, euforia e estado de alerta. A ressonância magnética possui um papel importante para o diagnóstico, estadiamento e seguimento do astrocitoma pilomixoide. No entanto, para o diagnóstico definitivo, o estudo anatomopatológico é fundamental, principalmente na diferenciação com o astrocitoma pilocítico. Além disso, em alguns casos, como o aqui apresentado, evidencia-se a apresentação simultânea de características histológicas do astrocitoma pilomixoide e pilocítico, constituindo um grupo denominado astrocitoma pilomixoide intermediário. A cirurgia é a melhor opção de tratamento e geralmente há necessidade de tratamento adjuvante.

The CK1 gene family: expression patterning in zebrafish development

Protein kinase CK1 is a ser/thr protein kinase family which has been identified in the cytosol cell fraction, associated with membranes as well as in the nucleus. Several isoforms of this gene family have been described in various organisms: CK1 , CK1á, CK1δ, CK1å and CK1γ. Over the last decade, several members of this family have been involved in development processes related to wnt and sonic hedgehog signalling pathways. However, there is no detailed temporal information on the CK1 family in embryonic stages, even though orthologous genes have been described in several different vertebrate species. In this study, we describe for the first time the cloning and detailed expression pattern of five CK1 zebrafish genes. Sequence analysis revealed that zebrafish CK1 proteins are highly homologous to other vertebrate orthologues. Zebrafish CK1 genes are expressed throughout development in common and different territories. All the genes studied in development show maternal and zygotic expression with the exception of CK1å. This last gene presents only a zygotic component of expression. In early stages of development CK1 genes are ubiquitously expressed with the exception of CK1å. In later stages the five CK1 genes are expressed in the brain but not in the same way. This observation probably implicates the CK1 family genes in different and also in redundant functions. This is the first time that a detailed comparison of the expression of CK1 family genes is directly assessed in a vertebrate system throughout development.

Distribution of trkA in cerebral cortex and diencephalon of the mongolian gerbil after birth

TrkA is essential components of the high-affinity NGF receptor necessary to mediate biological effects of the neurotrophins NGF. Here we report on the expression of trkA in the cerebral cortex and diencephalon of mongolian gerbils during postnatal development. The expression of trkA was identified by immunohistochemical method. In parietal cortex and piriform cortex, higher levels of trkA-IR (immunoreactivity) were detected at 3 days postnatal (P3) and at P9. Although trkA was not expressed till P3 in the parietal cortex, it was detectable at birth in the piriform cortex. Several regions, such as Layers I, IV & VI, did not show much expression. Layer I showed especially weak labeling. In the hippocampus, thalamus, and hypothalamus, higher levels of trkA-IR were detected at P6 and P12 than earlier days. But trkA was not expressed at birth in the hippocampus, at P3 in the reticular thalamic nucleus (Rt), or neonatally in the dorsomedial hypothalamic nucleus (DM). This data shows that expression of trkA is developmentally regulated and suggests that high affinity neurotrophin-receptors mediate a transient response to neurotrophines in the cerebral cortex and diencephalon during mongolian gerbil brain ontogeny.

Postnatal Development of Transforming Growth factor-alpha-immunoreactive Neurons in the Diencephalon of the Rat / 체질인류학회지

Transforming growth factor-alpha (TGF-alpha) immunoreactivity during postnatal development was examined in the rat diencephalon using immunohistochemistry. The time of appearance and localization of TGF-alpha immunoreactivity was slightly different in many areas of diencephalon during postnatal development. At birth, TGF-alpha immunoreactivity was mainly evident in thalamic medial, median and parafascicular thalamic nucleus of intralaminar nuclei. In addition, TGF-alpha immunoreactivity was clearly evident at the first postnatal week in most hypothalamic nuclei. Therefore, TGF-alpha immunoreactivity was found at postnatal days 7 in most diencephalic nuclei excepting the vental posterior thalamic nuclei, metathalamus and epithalamus. The quantitative increase of number was first apparent in the midline structures of thalamus in the first postnatal week. And then TGF-alpha-immunoreactive cells progressively increased throughout diendephalon by postnatal days 15. Adult patterns were reached at postnatal days 20. These results indicate that TGF-alpha-immunoreactive cells were first appeared in thalamic midline structures, increased progressively in the first two postnatal weeks, and followed mediolateral gradient. In addition to maturation of TGF-alpha-immunoreactive cells requires a relatively prolonged period of time to achieve an adult configuration. Also, the early appearance of TGF-alpha immunoreactivity in most diencephalic nuclei may be related to the early appearance of EGFR immunorecativity in many other brain regions. Taken together, these findings suggest that TGF-alpha immunoreactivity correlated with the appearance of the related functional activity in the different regions of diencephalon.

A Case of Ophthalmofacial-Diencephalic Arteriovenous Malformation: A Variant of Bonnet-Dechaume-Blanc Syndrome

Authors experienced a rare case of ophthalmofacial-diencephalic arteriovenous malformation, named Bonnet-Dechaume-Blanc syndrome in 12 year-old girl. Clinically patient had an episode of frequent bleeding from the nose and gingival. Also she was left blindness of which the ophthalmologist gave the warning for cerebral hemorrhage because of the tangled, engorged vessels in left fundus. The multiple nidus on angiography revealed on left intraorbital, maxillopalatal, hypothalamic and basal ganglia area. Surgery underwent for huge intracerebral hematoma. The patient passed away due to rebleeding 15 months after initial hemorrhage.

DISTRIBUTION OF CATECHOLAMINE-CONTAINING CELL BODIES IN THE HUMAN DIENCEPHALON / 解剖学报

The distribution and morphological characteristics of CA-containing cell bodies of diencephalon in 7-9monthold fetuses were studied by means of Faglu histofluoresccnce method. Although the general distribution of CA cell bodies in the human diencephalon observed in the present study is similar to that in other mammals, there are some significant differences. A great number of CA neurons occur within the lateroventral hypothalamic zone. There are more numerous larger CA neurons distributed outside the periventricular gray in human diencephalon.

AN IN VITRO STUDY ON THE INTERACTIONS OF CELLS FROM VARIOUS VISUAL STRUCTURES OF NEONATAL RATS / 解剖学报

We have investigated the growth and interactions between dissociated cells from different visual structures obtained from neonatal albino rats, namely, retina (R), superficial gray of the tectum (T), the lateral posterior portion of the dieneephalon (G), and occipital cortex (C). Dissociated cells obtained were either cultured separately or mixed with another cell type in 24-well plates coated with laminin. After 3 days of culture surviving cells were quantified by the MTT colorimetric microassay.The optical densities obtained from mixed cultures with cells from any two of the four types (G, R, T, C) of brain tissues were 1.3 to 3 times higher than the sum of their corresponding individual cultures except for those of G+C and T+G. Among them R+T and R+G were the most active cultures, followed by R+C and T+C. Further, condition medium from tectal cells (Tc) increased the activities of R and G for 3 and 2 times, respectively, and condition medium from retinal cells (Re) increased the activities of G and C for 1.3 and 2 times, respectively. Therefore it is suggested that neuronotrophic factors may be present in Tc and Rc.

AFFERENT PROJECTIONS FROM THE DIENCEPHALON AND SUBCORTICAL TELENCEPHALON TO THE PREFRONTAL CORTEX IN THE RAT——A WGA-HRP STUDY / 解剖学报

WGA-HRP was injected into the dorsal part of anterior cingulate area,prelim- bic area,and dorsal agranular insular area of the prefrontal cortex in 25 rats to observe the afferent connections from the diencephalon and subcortical telencephalon. The afferent projections from the diencephalon mainly originate from the dorsome- dial nucleus of the thalamus,and some topographic localization may exist.Further- more,the afferent projections to the prefrontal cortex arise from the thalamic intralaminar nuclei(centrolateral nucleus,paracentral nucleus,centromedial nucleus and parafascicular nucleus),the ventral thalamic nuclei(ventrolateral nucleus,ven- tromedial nucleus,ventroanterior nucleus and ventroposterior nucleus),the midline nuclei(rhomboid nucleus,reuniens nucleus,paratenial nucleus,and paraventricular nucleus of the thalamus),the anteromedial nucleus,the lateral habenular nucleus, the posterior nucleus of the thalamus,and the lateral nucleus of the thalamus.The numbers projecting to the prefrontal cortex were different in different thalamic nuclei.The afferent projections from the hypothalamus mainly originate from the lateral hypothalamic area,lateral preoptic area,caudal magnocellular nucleus,and supramamillary nucleus.A few of afferent projections from the posterior hypothala- mic area,dorsomedial and ventromedial nucleus of the hypothalamus and zona inserta were also observed.The afferent projections from the subcortical telence- phalon mainly originate from the globus pallidus,the diagonal band nucleus,the septal nuclei(medial septal nucleus,lateral septal nucleus,septofimbrial nucleus and nucleus triangularis septi),the amygdaloid nuclei(lateral amygdaloid nucleus,lateral and medial part of basal amygdaloid nucleus,medial amygdaloid nucleus and cortical amygdaloid nucleus)and the claustrum.