Persistencia del cavum septum pellucidum y cavum vergae asociado a retraso psicomotor. Informe de caso

Fundamento: Dos de las tres formas en que se presentan los quistes intracraneales de la línea media anterior son: cavum septum pellucidum y cavum vergae; estos normalmente desaparecen después del nacimiento, de persistir suelen ser asintomáticos, pero también pueden estar asociados a manifestaciones obstructivas, trastornos psicóticos o alteraciones del neurodesarrollo que demandan de un seguimiento clínico. Objetivo: Reportar el caso de un paciente de 6 meses con persistencia de estructuras del periodo embrionario en posible asociación con retraso del desarrollo psicomotor. Presentación de caso: Por lo infrecuente que resulta en la práctica, se informa el caso de un paciente de 6 meses con una persistencia del cavum septum pellucidum y cavum vergae en el que se destaca la posible asociación del retraso del neurodesarrollo a la persistencia de estas estructuras. El diagnóstico se realizó de forma precoz y se intervino oportunamente. Conclusiones: La presentación del caso aportó evidencias epidemiológicas que favorecen la posible asociación entre la persistencia de estas estructuras embrionarias y el retraso del desarrollo psicomotor.

Background: Two out of the three forms in which intracranial anterior midline cysts present are: These usually disappear after birth; if they persist, they are often asymptomatic, but may also be associated with obstructive manifestations, psychotic disorders or neurodevelopmental disorders that require clinical follow up. Objective: To report a case of a 6-month-old patient with persistence of embryonic period structures in possible association with psychomotor developmental retardation. Case presentation: Because of how infrequent it is in practice, a case of a 6-month-old patient with a persistent cavum septum pellucidum and cavum vergae is reported in which the possible association of neurodevelopmental delay with the persistence of these structures is pointed out. The diagnosis was made in an early manner and it was timely intervened. Conclusions: The case presentation provided epidemiological evidences that encourage the possible association among the persistence of these embryonic structures and psychomotor developmental retardation.

Resultado a largo plazo de una serie de fetos con agenesia aislada del septum pellucidum / Long-term postnatal outcome of fetuses with isolated agenesis of the septum pellucidum

Objetivo: Reportar el resultado a largo plazo de una serie de fetos con agenesia del septum pellucidum aislada (ASP), con medición de su quiasma óptico mediante neurosonografía fetal (NSG). Método: Se incluyeron todas las pacientes con ASP y NSG evaluadas desde el año 2008 a la fecha y con seguimiento hasta su edad escolar. En todos los casos se consignaron los datos clínicos de NSG y de resonancia magnética (RM), cuando esta se realizó. Se entrevistó telefónicamente a los padres. Resultados: Nueve pacientes cumplieron los criterios: cuatro con displasia septo-óptica (DSO) (rango de seguimiento: 5-14 años) y cinco sin DSO (rango de seguimiento: 7-10 años). Un décimo caso se excluyó por tener solo 6 meses de seguimiento. Ninguna de las ASP tuvo otra anomalía detectada en su seguimiento. Ninguno de los casos con DSO tuvo alteración del tamaño de su quiasma óptico en la NSG ni anormalidad en la vía óptica en la RM. Conclusiones: En nuestra población, el riesgo residual de DSO frente a ASP es del 44,4%. En el seguimiento, nuestra definición de ASP por NSG no tuvo falsos negativos con relación a otras anomalías de aparición posnatal, a excepción de la DSO.

Objective: To report the long-term outcome of a series of fetuses with isolated septum pellucidum agenesis (ASP) with measurement of their optic chiasm by fetal neurosonography (NSG). Method: All patients with ASP and NSG evaluated from 2008 to date and with follow-up until their school age were included. In all cases, clinical, NSG and magnetic resonance imaging (MRI) data were recorded. Parents were interviewed by telephone. Results: Nine patients met the criteria: four with septo-optic dysplasia (SOD) (follow-up range: 5-14 years) and five without SOD (follow-up range: 7-10 years). A tenth case was excluded because only 6 months of follow-up. None of the ASP cases had another anomaly detected in their follow-up. None of the cases with DSO had anomaly of the size of their optic chiasm on NSG or abnormality in the optical pathway in the MRI. Conclusions: In our population, the residual risk of DSO versus ASP is 44.4%. At follow-up, our NSG definition of ASP had no false negatives in relation to other postnatal-onset anomalies, except for SOD.

Comparison of Cavum Septum Pellucidum Size in Euploid and Aneuploid Fetuses / Comparação do tamanho do cavum septum pellicidium em fetos euploides e aneuploides

Abstract Objective The aim of the present study is to compare the cavum septum pellucidi (CSP) z-score in euploid and aneuploid fetuses and to investigate the performance of the CSP width/length and CSP width/biparietal diameter (BPD) ratios as a diagnostic marker in aneuploidy. Methods A total of 54 patients, 20 aneuploid and 35 euploid fetuses, between 18 and 37 weeks of gestation, were included in this retrospective study. The CSP width z-score was compared between the two groups. Receiver operating characteristic (ROC) curves were calculated for the CSP width/length and CSP width/BPD ratios to predict aneuploidy. Results The median CSP width was 4.8 mm (range, 1.8 to 8.5 mm) in the euploid group, and 5.4 mm (range 3.1 to 8.4 mm) in the aneuploid group. Cavum septum pellucidi width z-score, CSP width/length ratio, and CSP width/BPD ratio were significantly higher in fetuses with aneuploidy than in fetuses with normal karyotype (p= 0.001; p= 0.013; p= 0.028). In the ROC analysis, the CSP width/length ratio had the optimal cutoff value of 0.59, with 72.0% sensitivity and 58.0% specificity, and for the CSP width/BPD ratio, the cutoff value was 0.081 with 83.0% sensitivity and 61.0% specificity for detection of aneuploidy. Conclusion CSP width z-score was found to be increased in aneuploid fetuses. The CSP width /BPD ratio can be used as a new marker for predicting aneuploidy.

Resumo Objetivo: O objetivo do presente estudo é comparar o escore z do cavum septum pellucidi (CSP) em fetos euploides e aneuploides e investigar o desempenho das relações largura/comprimento do CSP e largura do CSP/diâmetro biparietal (BPD) como marcador diagnóstico de aneuploidia. como marcador de diagnóstico de aneuploidia. Métodos: Um total de 54 pacientes, 20 fetos aneuploides e 35 fetos euploides, entre 18 e 37 semanas de gestação, foram incluídos neste estudo retrospectivo. O escore z da largura da CSP foi comparado entre os dois grupos. As curvas ROC (Receiver Operating Characteristic) foram calculadas para as relações largura/comprimento da PEC e largura da PEC/BPD para prever a aneuploidia. Resultados: A largura mediana da CSP foi de 4,8 mm (variação de 1,8 a 8,5 mm) no grupo euploide e de 5,4 mm (variação de 3,1 a 8,4 mm) no grupo aneuploide. O escore z da largura do cavum septum pellucidi, a relação largura/comprimento do CSP e a relação largura do CSP/BPD foram significativamente maiores em fetos com aneuploidia do que em fetos com cariótipo normal (p < 0,001; p < 0,013; p < 0,028). Na análise ROC, a relação largura/comprimento da CSP teve o valor de corte ideal de 0,59, com 72,0% de sensibilidade e 58,0% de especificidade, e para a relação largura da CSP/BPD, o valor de corte foi de 0,081, com 83,0% de sensibilidade e 61,0% de especificidade para a detecção de aneuploidia. Conclusão: Verificou-se que o escore z da largura da CSP estava aumentado em fetos aneuploides. A relação A relação largura da CSP /BPD pode ser usada como um novo marcador para prever a aneuploidia.

Evaluation of relationships between presence of adhesio interthalamica and cannabis use in first-episode psychosis: a magnetic resonance imaging investigation

ABSTRACT Recent studies suggested that cannabis use influences on the emergence of psychosis by disrupting neurodevelopmental processes that occur during adolescence and early adulthood and which are reflected on brain anatomical changes detectable with MRI. However, no MRI studies have investigated whether intrauterine neurodevelopmental abnormalities also interact with later cannabis use to influence on psychosis risk. We investigated differences between first-episode psychosis (FEP) patients with history of cannabis use (FEPC+, n=28), FEP subjects without cannabis use (FEPC-, n=78) and healthy controls (n=80) in regard to the frequency of absent or short Adhesio Interthalamica (AI), a well-established marker of intrauterine neurodevelopment. The FEPC+ subgroup had a significantly lower prevalence of absent AI than FEPC- subjects, as well as a lack of a significantly shorter AI length compared to controls (as found in FEPC- subjects). These preliminary results show that psychosis subjects with cannabis use present a low rather than high frequency of absent AI, suggesting that fixed intrauterine neurodevelopmental abnormalities may not be associated with cannabis use later in life to influence on the emergence of psychosis. This is consistent with a view that multiple different etiological processes may lead to similar clinical presentations in patients with FEP.

Midline Cava And Limbic System Dysfunction In Community-Dwelling Individuals Aged ≥20 Years Living In Rural Ecuador. A Case-Control Study Nested To A Population-Based Cohort / Cavum De Línea Media Y Disfunción Del Sistema Límbico En Individuos De 20 Años O Más Que Viven En Zonas Rurales Del Litoral Ecuatoriano. Estudio De Caso-Control Anidado En Una Cohorte Poblacional

ABSTRACT Background: Persistence of cavum septum pellucidum (CSP) and cavum vergae (CV) into adulthood and their association with mood disorders is unknown. Objective: We aimed to assess persistence of these cava in Atahualpa cohort individuals, and their association with clinical depression (as a surrogate of limbic system dysfunction). Methods: Cases were defined as Atahualpa residents aged ≥20 years with CSP and/or CV and paired 1:1 to age- and sex-matched randomly-selected controls. A board-certified psychiatrist (blinded to case-control status) interviewed individuals with the aid of the Patient Health Questionnaire depression module (PHQ-9) to establish a diagnosis of clinical depression. The McNemar's test and conditional logistic regression models were fitted to assess the independent association between persistence of CSP and/or CV and clinical depression (as the dependent variable). Results: Of 1,298 individuals undergoing a head CT, 51 (3.9%) had a CSP and/or CV. The selection process for the nested case-control study on the Atahualpa cohort (after excluding eight missing individuals with midline cava) generated 43 pairs. Nine of 43 case-patients (20.9%) and only two control subjects (4.7%) had moderate-to-severe scores on the PHQ-9 (cutoff ≥10 points). Clinical depression was significantly more frequent among case-patients than controls by the McNemar's test (OR: 8; 95% C.I.: 1.1 - 354.9) and the conditional logistic regression model (OR: 8; 95% C.I.: 1.00 - 63.96). Conclusions: This study provides epidemiological evidence favoring the association between midline cava and clinical depression, supporting their relationship with limbic system dysfunction.

RESUMEN Antecedentes: La persistencia de cavum septum pellucidum (CSP) y cavum Vergae (CV) en adultos y su asociación con trastornos del estado de ánimo es desconocida. Objetivo: Evaluar la persistencia de estas cavidades y su asociación con depresión clínica (como marcador de disfunción del sistema límbico). Métodos: Los casos se definieron como residentes de Atahualpa con edades ≥20 años con CSP y/o CV y se emparejaron 1:1 con controles aleatoriamente seleccionados de acuerdo con edad y sexo. Un psiquiatra (ciego al estado caso/control) entrevistó a los individuos con el módulo de depresión del cuestionario de salud del paciente (PHQ-9) para establecer diagnóstico de depresión clínica. La prueba de McNemar y modelos de regresión logística condicional se utilizaron para evaluar la asociación independiente entre persistencia de CSP y/o CV y depresión clínica (como variable dependiente). Resultados: De 1.298 individuos sometidos a TC de cerebro, 51 (3.9%) tenían un CSP y/o CV. El proceso de selección para el estudio de casos y controles generó 43 pares. Nueve de 43 casos (20.9%) y solo dos controles (4.7%) tuvieron puntajes de moderados a severos en el PHQ-9 (punto de corte ≥10 puntos). La depresión clínica fue significativamente más frecuente entre los pacientes que en los controles mediante la prueba de McNemar (OR: 8; 95% C.I.: 1.1 - 354.9) y la regresión logística condicional (OR: 8; 95% C.I.: 1.00 - 63.96). Conclusiones: Este estudio proporciona evidencia epidemiológica que favorece la asociación entre persistencia de CSP y/o CV y depresión clínica, lo que favorece su relación con disfunción del sistema límbico.

Cavum Septum Pellucidum, da embriologia à clínica: uma revisão da literatura / Cavum Septum pellucidum from embryology to clinics: review of literature

Introdução: O septo pelúcido (SP) é formado por duas lâminas neurais separadas, que se fundem após o nascimento. Quando não há fusão completa, tem-se a persistência do Cavum embriológico, que pode ser chamado Cavum Septum pellucidum (CSP), Cavum Vergae ou Cavum Septum pellucidum et Vergae (CSP et Vergae), dependendo da extensão anteroposterior. Embriologicamente, o CSP está relacionado à formação do corpo caloso e de outras estruturas cerebrais. O SP faz parte do sistema límbico, por isso há possibilidade de que Cava persistentes tenham repercussão nas funções neuropsíquicas. Objetivo: revisar a literatura sobre a formação e o significado clínico da persistência do CSP em adultos. Métodos: foi realizada revisão da literatura de artigos publicados na base de dados PUBMED, utilizando os descritores: "Cavum", "Brain Cava" e "Cavum Septum Pellucidum". Os artigos selecionados continham considerações sobre formação e possível significado clínico do CSP. Resultados e Discussão: CSP vestigiais são considerados como variantes da normalidade. Porém, vários estudos sugerem que a persistência de CSP com grandes dimensões pode representar uma disgenesia cerebral na linha média. Estudos tentam relacionar o CSP com múltiplos distúrbios neuropsiquiátricos, especialmente com esquizofrenia. De maneira geral, há relativo consenso de que os CSP alargados têm maior chance de representar significado patológico, embora suas manifestações não sejam bem reconhecidas. Conclusão: existem indícios de que CSP persistentes, quando alargados, podem manifestar-se como distúrbio neuropsíquico, de espectro ainda indefinido. Estudos em população geral ainda são escassos e com prevalências variadas. Trabalhos mais abrangentes são necessários para melhor entendimento de seu significado clínico.

Introduction: The septum pellucidum (SP) is formed by separated neural laminae, that subsequently merge. When there is no complete fusion of the laminae, there is persistence of the embryological Cavum, that can be named Cavum Septum pellucidum (CSP), Cavum Vergae or Cavum Septum pellucidum et Vergae (CSP et Vergae) according to its anterior-posterior length. Embryologically, CSP is related to the development of the corpus callosum and other cerebral structures. The SP belongs to the limbic system, and thus, it is possible that Cava persistence may lead to neuropsychiatry dysfunction. Objective: To review the literature about development and clinical significance of CSP. Methods: A review of literature of articles published on the database PUBMED. The following descriptors were used: "Cavum", "Brain Cava" and "Cavum Septum Pellucidum". The select articles had much information about development and clinical significance of CSP. Results and Discussion: Vestigial CSP are considered normal variants. However, several studies suggest that their persistence in adults may represent a form of midline cerebral dysgenesis. Studies attempted to link CSP to multiple neuropsychiatric disturbances, especially schizophrenia. There is relative agreement about the idea that large CSP are more likely to have pathological meanings, but their symptoms are not well recognized. Conclusion: There is evidence that persistent CSP, if large, may exhibit neuropsychic disorder, of unknown magnitude. Studies in the general population are scarce and with variable prevalence. Further comprehensive studies are necessary to better understand their clinical meaning.

Analysis of outcome of fetuses with dilated fetal cavity of septum pellucidum detected with prenatal ultrasonography / 中国介入影像与治疗学

Objective: To analyze the outcome of fetuses with dilated fetal cavity of septum pellucidum (CSP), and to explore the clinical significances of dilated CSP detected with prenatal ultrasonography. Methods: Fetuses with dilated CSP detected with prenatal ultrasonography were followed up, and the results of prenatal imaging examination and perinatal outcomes were recorded. Results: Totally 48 fetuses were found with dilated CSP using prenatal ultrasound, among them 9 were lost during follow-up and 39 were enrolled in the study, including 13 with isolated dilated CSP and 26 with accompanied other abnormalities. Chromosome analysis was performed in 11 fetuses, and 2 were found abnormal. Induced labor was performed in 6 fetuses with various deformities. One fetus died intrauterine at 38 weeks of pregnant for unknown cause, 2 newborns died several days after delivery, while postnatal motor and cognitive developmental delay were observed in 1 case. During 3-46 months' follow-up, 29 cases (including 13 with isolated dilated CSP and 16 dilated CSP accompanied with other mild abnormalities) showed no significant abnormalities compared with their peers after birth. Conclusion: The outcome of fetuses with isolated dilated CSP detected with prenatal ultrasonography is good, and the prognosis of those with other malformations depends on the severity of the accompanying malformations.

Outcome of Neonates with Agenesis of Septum Pellucidum: A Retrospective Single Center Study

PURPOSE: Agenesis of the septum pellucidum (ASP) is a very rare disease that can be isolated or associated with other brain abnormalities. The neurological prognosis of isolated ASP remains controversial. The aim of this study was to evaluate the clinical outcome of neonates with ASP. METHODS: We retrospectively analyzed the medical records of 12 neonates with isolated ASP or ASP combined with other brain abnormalities who were born at Cheil General Hospital & Women's Healthcare Center between January 2007 and December 2017. RESULTS: Of the 12 neonates, six were identified prenatally and six were identified postnatally. Isolated ASP was found in eight neonates; of these, four were detected antenatally. ASP associated with other brain abnormalities was found in four neonates. ASP was complete in nine neonates, including six with isolated ASP, and partial in three, including two with isolated ASP. Six of the eight neonates with isolated ASP had normal neurological development, except two who were lost to follow-up. Among the four neonates with other associated brain abnormalities, two had delayed motor development and a seizure, one had normal development, and one was lost to follow-up. In all neonates, ophthalmological examination revealed no optic nerve abnormalities. CONCLUSION: Isolated ASP seems to have a good neurological prognosis without ocular problems. This result needs to be confirmed by larger prospective studies over a longer developmental timeline.

The value of the Oblique of three-dimensional ultrasonography in diagnosis of fetal cavum septum pellucidum / 中华超声影像学杂志

Objective To evalute the clinical significance of the Oblique view of three-dimensional ultrasonography(3DUS) in diagnosis of the fetal cavum septum pellucidum(CSP) malformation.Methods Thirty-eight cases of fetuses with the CSP malformation,found originally by trans-abdominal two dimensional ultrasonography(2DUS),were scanned using Oblique of trans-abdominal 3DUS.The results were compared with MRI inspection and post-natal follow-up.Results Thirty-eight fetuses suspected with 3DUS Oblique were fully confirmed by autopsy and MRI,the distribution of CSP malformation included:①Twenty-one cases with absence of septum pellucidum(ASP),among them 10 cases with isolates of septum pellucidum,1 case with septooptic dysplasia(SOD),10 cases with agenesis of corpus callosum(9 complete agenesis of corpus callosum and 1 partial agenesis of corpus callosum.②Twelve cases with shrinkage of them 9 simple shrinkage and 3 partial agenesis of corpus callosum respectively.③ Five cases with enlargement,all of which also with increasing sizes of cavum vergae.Thirty-five cases are consistent with MRI observations.The diagnosis consistency differences between both methods were not significant.Conclusions 3DUS Oblique view can reveal the structure of fetal cavum septum pellucidum and corpus callosum clearly,which enable more accurate prenatal counseling and boild firm base for further diagnosis.

Biometría del cráneo y sistema ventricular cerebral fetal: tablas de normalidad a través de pruebas no paramétricas

Objetivos: Realizar tablas de referencia a través de estadística no paramétrica para definir en percentiles los rangos de normalidad del diámetro biparietal, la circunferencia cefálica, el atrio ventricular cerebral, la cisterna magna, el cavum septum pellucidum y los ventrículos laterales, según recomendaciones de la International Society of Ultrasound in Obstetrics and Gynecology de evaluación y medida. Métodos: Estudio transversal realizado desde enero 2014 a enero 2016. Se evaluaron 1004 embarazadas normales, en diferentes edades gestacionales, y de manera previamente estandarizada se midieron las estructuras mencionadas. El análisis estadístico se realizó con el software libre PAST 3.04 para la organización de los datos de cada edad gestacional en percentiles. Se presentaron en gráficos tipo nomogramas y en modelo de regresión polinómica de primer orden. Cada gráfico fue evaluado con significancia estadística con P<0,05. Resultados: Las estructuras intracraneales pudieron medirse en su totalidad en 864 casos (86 % de los exámenes). Los diámetros biparietal y las circunferencias cefálica pudieron obtenerse en todos los casos, se observó un crecimiento directamente proporcional a la edad gestacional (P< 0,05). La medida del atrio ventricular resultó estable lo largo del embarazo. Se presentan las medidas de la cisterna magna, del cavum septum pellucidum y de los ventrículos laterales. Conclusiones: Los rangos de normalidad se representaron en tablas para correcto uso clínico y de investigación, no difieren de investigaciones previas realizadas en otros países. Se presentan valores de referencia utilizables en la consulta prenatal, a través de estadística no gaussiana.

Objectives: To carry out reference tables through non-parametric statistics to define in percentile ranges of normality of the biparietal diameter, head circumference, the cerebral ventricular atrium, the cisterna magna, cavum septum pellucidum, and the lateral ventricles, according to the recommendations of the International Society of Ultrasound in Obstetrics and Gynecology of evaluation and measurement. Methods: A cross-sectional study was carried out from January 2014 to January 2016; 1004 normal pregnant women, in different gestational ages, were evaluated, and the mentioned structures, previously standardized, were measured. The statistical analysis was performed with the FOSS PAST 3.04 for the organization of the data at each gestational age, in percentiles. They arose in graphic type nomograms and first-order polynomial regression model. Each graphic was evaluated with statistics significance with P < 0.05. Results: The intracranial structures could be measured entirely in 864 cases (86% of the tests). Biparietal diameter and head circumferences were obtained in all cases; it was observed a directly proportional growth to gestational age (P < 0.05). The measurement of the ventricular Atrium was stable throughout the pregnancy. Measures of the cisterna magna, cavum septum pellucidum and of the lateral ventricles are represented. Conclusions: Normal ranges are represented in tables for correct clinical use and research, the results are not different from previous research conducted in other countries. Usable reference values, in the prenatal consultation, through non-Gaussian statistics are presented.

Variantes anatómicas del septum pellucidum / Anatomical variations of septum pellucidum

El septum pellucidum es un fino tabique formado por dos láminas gliales dispuestas entre el cuerpo calloso en dirección cefálica y el trígono ubicado caudalmente. Durante la vida embrionaria existen variantes anatómicas del septum pellucidum que se disponen en sentido rostro-dorsal. Estas son el cavum del septum pellucidum, el cavum vergae yel cavum velum interpositum. Su presencia o ausencia puede estar relacionada con alteraciones del desarrollo del sistema nervioso y trastornos cognitivo-psiquiátricos, por lo que deben conocerse bien para evitar diagnósticos erróneos

Septum pellucidum consists of a two thin laminae situated caudal to the corpus callosum and cephalic to the fornix (trigonum). Anatomical variations of septum pellucidum appear during fetal life in the ventro-dorsal position. These variations are: cavum septi pellucidi, cavum vergae and cavum veli interpositi. The presence or absence of these cavities can be related to the presence of nervous system or neuropsychiatric dysfunction, therefore they have to be well known to avoid a wrong diagnoses

Cavum Septum Pellucidum: Hallazgo Mediante Disección Neuroanatómica / Cavum Septum Pellucidum: Finding from Neuroanatomical Dissection

Se reporta una marcada cavitación a nivel del septum pellucidum, un gran cavum septum pellucidum de una persona adulta, en el espacio reconocido generalmente como cerrado del sistema nervioso central, que se hace evidente como tal, en ciertas fases de la vida intrauterina. Esta cavitación está ausente en el adulto sano, pero en algunas personas esquizofrénicas y con demencia pugilística, dicha cavidad, puede considerarse como un hallazgo de relativa frecuencia. Este hallazgo se encontró aleatoriamente en uno de cuarenta y dos encéfalos de adultos, valorados superficialmente desde la anatomía macroscópica como normales. Dicho hallazgo posiblemente se asocie con desórdenes conductuales, en vista que dicho encéfalo correspondía a un individuo proveniente de un hospital psiquiátrico.

A marked cavitation in the septum pellucidum level is reported, a great cavum septum pellucidum of an adult in the closed space generally recognized as the central nervous system, which is evident in itself, at certain stages of intrauterine life. This cavitation is usually absent in the healthy adult, but in some people with schizophrenia and pugilistic dementia, the cavity can be regarded and found relatively frequently. This finding was found randomly in one of forty-two adult brains, superficially valued from the gross anatomy as normal. This finding may be associated with behavioral disorders, taking in to account that the brain corresponded to a person from a psychiatric hospital.

Transseptumpellucidumrostrostomy: anatomical considerations and neuroendoscopic approach / Transseptopelucidorostrostomia: considerações anatômicas e abordagem neuroendoscópica

PURPOSE: Verify the presence of the rostral lamina of the corpus callosum, and set parameters for neuroendoscopy. METHODS: Relationship of the floor of the frontal horn of lateral ventricle and the hypothalamic-septal region were studied after sagittal and axial sections of the brains. Measurements were compared using F and Student t tests. The correlations between anterior-posterior diameter of the interventricular foramen / anterior-posterior diameter of the fornix column, and between anterior-posterior diameter of the interventricular foramen / length of the rostral lamina were performed by Pearson index test. RESULTS: There was no statistically significant difference in measurements performed in both hemispheres (p<0.05). Positive correlations were observed between the anterior-posterior diameter of the interventricular foramen / anterior-posterior diameter of the fornix column (R = 0.35), and between the anterior-posterior diameter of the interventricular foramen / length of the rostral lamina (R = 0.23). CONCLUSION: Rostral lamina was observed in all brains. It was possible to perform an endoscopic fenestration in the rostral lamina, communicating safely the lateral ventricle with a polygonal subcallosal cistern.

OBJETIVO: Verificar a presença da lâmina rostral do corpo caloso e padronizar parâmetros para a realização de neuroendoscopia. MÉTODOS: A relação do assoalho do corno frontal do ventrículo lateral e a região hipotálamo-septal lateral foi estudada através de secções sagitais e axiais dos cérebros. As medidas foram comparadas utilizando os testes F e t-Student. As correlações entre diâmetro ântero-posterior do forame interventricular / diâmetro ântero-posterior da coluna do fornix, e entre o diâmetro ântero-posterior do forame interventricular / comprimento da lâmina rostral foram estudadas pelo teste de Pearson. RESULTADOS: Não houve diferença estatisticamente significante nas medidas realizadas em ambos hemisférios (p <0.05). Correlações positivas foram observadas entre diâmetros ântero-posteriores do forame interventricular / coluna do fornix (R = 0.35), os diâmetros ântero-posteriores do forame interventricular / comprimento da lâmina rostral (R = 0.23). CONCLUSÃO: A lâmina rostral foi observada em todos espécimes. Foi possível realizar uma fenestração endoscópica na lâmina rostral, comunicando com segurança o ventrículo lateral a uma cisterna poligonal subcalosa.

Absence of the Septum Pellucidum Associated with a Midline Fornical Nodule and Ventriculomegaly: A Report of Two Cases

We report two autopsy cases that revealed the partial absence of the septum pellucidum with ventriculomegaly. In each case, the brain showed mild dilatation of both frontal horns of the lateral ventricles, normal third and fourth ventricles and no aqueductal stenosis. The posterior portion of the septum pellucidum was absent and the fornices were fused in a single midline nodule, abnormally displaced to a caudal position and lodged in the foramina of Monro. The brain base showed no apparent abnormalities; the optic nerves were well developed. We conclude that the caudally displaced fornix in the absence of the septum pellucidum may have intermittently obstructed the foramina of Monro and induced mild ventriculomegaly.

Brain Imaging Studies in Leber's Congenital Amaurosis: New Radiologic Findings Associated with the Complex Trait

PURPOSE: To report the incidence and new findings of abnormal brain imaging studies associated with patients initially diagnosed with Leber's congenital amaurosis (LCA) without definite systemic abnormalities and to determine the need for brain imaging studies in these patients. METHODS: A retrospective review of medical records was performed in 83 patients initially diagnosed as LCA and without definite systemic abnormalities before the age of 6 months in 2 tertiary referral centers. Brain magnetic resonance imaging was performed in 31 of 83 patients (37.3%). RESULTS: Six of 31 patients (19%) had radiologically documented brain abnormalities. Two patients had cerebellar vermis hypoplasia, 1 patient showed an absence of septum pellucidum, 2 subjects showed mild external hydrocephalus, and 1 patient was found to have a small cerebellum. CONCLUSIONS: Approximately one fifth of the LCA patients in whom brain imaging was performed were associated with brain abnormalities, including the absence of septum pellucidum, which has not been documented in the literature. Brain imaging is mandatory in patients primarily diagnosed with LCA, even without definite neurologic or systemic abnormalities.

Treatment of septum pellucidum cysts by CT-guided puncture and cystoperitoneal shunt: Report of 12 cases / 中国微创外科杂志

Objective To explore the feasibility of CT-guided puncture and cystoperitoneal shunt in the treatment of septum pellucidum cysts.Methods Twelve patients with symptomatic septum pellucidum cysts were treated by CT-guided puncture and cystoperitoneal shunt.There were 8 patients with accompanying hydrocephalus.Before operation,routine coronal CT scans were carried out to determine the relationship between the center of the cyst and the baseline.Results Symptoms of headache and dizziness disappeared in all the 12 patients.No recurrence of epileptic attack was found in 3 patients with epileptic history.Clear consciousnesss was observed in 2 patients with loss of consciousnesss.All the 12 patients were followed for 2~36 months(mean,11 months).Postoperative CT and MRI examinations showed that the cyst disappeared in 10 patients and decreased in size in 2 patients(the maximum diameter decreased by 3~18 mm,with a mean of 12.6 mm).The enlarged ventricle in 8 patients with hydrocephalus was 30% or more smaller than that before operation.All the patients were discharged from hospital without any complications.Conclusions CT-guided puncture and cystoperitoneal shunt in the treatment of symptomatic sepeum pellucidum cysts is feasible and it has advantages of minimally invasion,simple to perform,less complications.

Endoscopic neurosurgery for the treatment of septum pellucidum cysts / 中国微创外科杂志

Objective To discuss the techniques and significance of neuroendoscopic treatment for symptomatic septum pellucidum cysts. Methods A total of 12 patients with symptomatic septum pellucidum cysts were given a cyst-lateral ventricle ventriculostomy via a transcallosal and transventricular approach by using a neuroendoscope 4.0 mm in external diameter(Storz,Germany).Headache,epilepsy,and mental symptoms were prominent clinical manifestations.Two patients were complicated with hydrocephalus. Results Improvement of symptoms was achieved postoperatively in all the 12 patients.Out of 6 patients with a major sign of intracranial hypertension,symptoms completely disappeared in 4 patients and improved in 2 patients.Four patients with epilepsy attacks had no recurrence.A follow-up was made in 12 patients for 6~24 months(mean,18 months).Re-examinations of CT or MRI showed the cysts had decreased in size by 90% in 2 patients.The lesions completely disappeared in 2 patients with hydrocephalus. Conclusions Neuroendoscopic cyst-lateral ventricle ventriculostomy for septum pellucidum cysts is a safe,effective,and minimally invasive operation.

Relationship between Cavum Septum Pellucidum and Epilepsy

OBJECTIVE: The authors study a relationship between the presence of cavum septum pellucidum(CSP) and the development of epilepsy by comparing the presence of CSP, which has been known to be a normal variation, in normal control group and epilepsy patients. METHODS: This study included 377 patients with epilepsy and 252 controls without epilepsy. Of epilepsy patients, 168 patients underwent surgery due to intractability and 209 patients was on medication of antiepileptic drugs. Control group had only headache and no visible lesion in MRI. Of 168 surgical patients, 102 patients had temporal lobe epilepsy and 66 patients had extratemporal lobe epilepsy. Ninty five patients showed a neuronal migration disorder in histopathologic findings. Definition of "CSP" and "partial CSP" was followed by Pauling's classification. RESULTS: CSP was present 8.2% of epilepsy patients and 1.6% of control group(p<0.01). CSP was detected in 11.3% of patients with surgical treatment and in 5.7% of patients with medical treatment. CSP was noticed in 8.9% of temporal lobe epilepsy, in 15.2% of extratemporal lobe epilepsy, in 13.7% of patients with neuronal migration disorder, and in 8.2% of patients with no neuronal migration disorder. CONCLUSION: Presence of CSP is statistically higher in epilepsy patients than in control group. This results indicates that the presence of CSP may not be a simple normal variation, and it can be considered a developmental anomaly that may contribute to epileptogenesis.

Transabdominal sonography of the cavum septum pellucidum in normal fetuses in the second and third trimesters of pregnancy / 대한산부인과학회잡지

OBJECTIVE: To assess the visualization rate and size of the cavum septum pellucidum (CSP) by transabdominal sonography in normal fetuses in the second and third trimesters of pregnancy. METHODS: The CSP was prospectively researched and measured using an axial transventricular plane in 308 consecutive uncomplicated singleton pregnancies between 15 and 41 weeks of gestation. RESULTS: The CSP was seen in 42.5% of cases at 15 weeks, 84.78% at 16-17 weeks, 100% at 18-37 weeks and 81.25% at 38-41 weeks. Compared to biparietal diameter (BPD), the visualization rate was 35.29% between 31 and 32 mm, 47.8% between 33 and 34 mm, 84% between 35 and 43 mm, 100% between 44 and 88 mm and 87.5% between 89 and 101 mm. Mean CSP width was 5.5+/-1.7 mm (range 2-9 mm). The CSP width was increased with gestational age and BPD, but with a slight decrease around term. CONCLUSION: In normal fetuses the CSP should always be visualized between 18 and 37 weeks, or with a BPD of 44-88 mm. Failure to observe the CSP in this interval, or possibly the presence of a large CSP, may indicate abnormal cerebral development and warrant further investigation. Conversely, absence of the CSP prior to 18 weeks, or later than 37 weeks, is a normal finding.

A Case of DeMorsier Syndrome Misdiagnosed as Amblyopia

PURPOSE: We report a 32-year-old man with DeMorsier syndrome, who was referred for seizures, esotropia and visual disturbance of left eye. METHODS: Fundus examination, visual field examination and the visual evoked potential were performed, and other diagnostic studies included endocrine determinations, the electroencephalogram and MR imaging. RESULTS: Ophthalmic examination revealed left optic nerve hypoplasia with the "double ring" sign. Axial MR T1 weighted image showed tiny left optic nerve and absence of the septum pellucidum. Subsequent evaluation revealed temporal visual field defect, abnormal VEP and deuteranomaly of left eye.