Mega Cisterna Magna (MCM) in a man with frotteurism symptoms - a case report. / Mega Cisterna Magna (MCM) u mezczyzny z objawami ocieractwa ­ opis przypadku.

Mega cistera magna (MCM) is a brain development abnormality that occurs in 1% of radiographic images. It occurs when the transverse dimension of the cerebellospinal reservoir is greater than 10 mm. It is the most important reservoir in the cranial cavity. From the front it is limited by the extended medulla, from the rear by the dura mater, and from the top by the cerebellum. Isolated anomalies in the structure of this brain region often do not cause any clinical symptoms. However, more and more attention is paid in research on the participation of the cerebellum in the regulation of cognitive and affective processes. These processes, in turn, may also be related to the regulation of sexual life. The aim of the study is to present the psychosexual functioning of a man diagnosed with MCM, who is suspected of having committed five sexual offenses. The existence of such a structure of the CNS in conjunction with the analysis of the interview, data from the case files and the results of sexological, psychological and psychiatric examinations, made it possible to evaluate the limitation of the respondent's ability to direct his conduct during the alleged acts, while maintaining the ability to recognize their significance.

Anatomical Implications of Chiari I and Basilar Invagination (Type B) in the IV Ventricle and Cisterna Magna.

OBJECTIVE: To analyze the anatomical changes of the IV ventricle and cisterna magna in the Chiari malformation I (CMI) and basilar invagination (type B). METHODS: This is a controlled study with 161 exams of magnetic resonance imaging (MRI) of adults grouped into control (n = 37), basilar invagination (BI; n = 31), Chiari malformation I (CMI; n = 37), and CMI+BI (n = 56). The MRIs were analyzed using the visualization software Osirix (Pixmeo, Bernex, Geneva, version 3.8.2). The morphometric variables were: distance from the obex to the McRae line; length of the IV ventricle floor; and the area and volume of the cisterna magna. The univariate ANOVA followed by Tukey's post-hoc test was applied to evaluate the difference between the groups. The difference between sexes was evaluated by the t test for each group. RESULTS: Alterations in the cisterna magna and IV ventricle were more evident only in the CMI and CMI+BI groups. For both sexes, the CMI and CMI+BI groups showed: a reduction in the CSF space (P < 0.001), cisterna magna with volume reduction (P < 0.001), low position of the obex (P < 0.001), and IV ventricle more elongated (male P = 0.007 and female P < 0.001). The BI group had no significant change in the analysis by sex. CONCLUSIONS: The CMI (isolated and associated with BI) showed a low obex position and elongation of the IV ventricle due to traction towards the spinal canal. The reduction of cisterna magna volume added to the occupation of the cerebellar tonsils can impact in the cerebrospinal fluid dynamics. The BI when isolated was not related to alterations in the parameters of cerebrospinal fluid spaces studied.

A valuable subarachnoid space named the occipito-atlantal cistern.

The cisterna magna has been defined as the space between the inferior margin of the cerebellar vermis to the level of the foramen magnum, while an enlarged dorsal subarachnoid space at the occipito-cervical junction extending from the foramen magnum to the upper border of the axis (C2) is still ignored. Recently, the myodural bridge complex is proved to drive the cerebral spinal fluid flowing via this region, we therefore introduce the "occipito-atlantal cistern (OAC)" to better describe the subarachnoid space and provide a detailed rationale. The present study utilized several methods, including MRI, gross anatomical dissection, P45 sheet plastination, and three-dimensional visualization. OAC was observed to be an enlarge subarachnoid space, extending from the foramen magnum to the level of the C2. In the median sagittal plane, OAC was a funnel shape and its anteroposterior dimensions were 15.92 ± 4.20 mm at the level of the C0, 4.49 ± 1.25 mm at the level of the posterior arch of the C1, and 2.88 ± 0.77 mm at the level of the arch of the C2, respectively. In the median sagittal plane, the spino-dural angle of the OAC was calculated to be 35.10 ± 6.91°, and the area of OAC was calculated to be 232.28 ± 71.02 mm2. The present study provides OAC is a subarachnoid space independent from the cisterna magna. Because of its distinctive anatomy, as well as theoretical and clinical significance, OAC deserves its own name.

Reference ranges of fetal cisterna magna volume measurements by three-dimensional ultrasonography in the late second trimester considering sonographic experience.

BACKGROUND: Volume measurements of fetal cisterna magna (CM) by three-dimensional (3D) ultrasonography may have a role in the diagnosis of various posterior fossa abnormalities. PURPOSE: To evaluate reference intervals and reliability of fetal CM volume values by virtual organ computer-aided analysis (VOCAL) in structurally normal fetuses, considering experience of evaluators. MATERIAL AND METHODS: Three operators with different 3D sonography experience levels measured CM volumes of 100 structurally normal fetuses at 18-27 weeks of gestation. Reference intervals for CM volumes were generated. Intraclass correlation coefficients (ICC) were calculated. RESULTS: Mean fetal CM volume measurements by the three operators did not significantly (P = 0.49, P = 0.22, and P = 0.17, respectively) change through 20-23 weeks of gestation. Moderate degrees of inter-observer reliability were found with an ICC of 0.69 between novice and intermediate-level, ICC of 0.74 between experienced and intermediate-level, and ICC of 0.78 between experienced and novice observer, respectively. The novice sonographer generally overestimated CM measurements. Intra-observer reliability was good (ICC=0.85). CONCLUSION: A reference chart for fetal CM volume by VOCAL was formed, revealing uniform mean values of 20-23 weeks of gestation. The inter-observer reliability is moderate, and biases seem relatively common for all experience categories.

Stereotaxic Surgical Approach to Microinject the Caudal Brainstem and Upper Cervical Spinal Cord via the Cisterna Magna in Mice.

Stereotaxic surgery to target brain sites in mice is commonly guided by skull landmarks. Access is then obtained via burr holes drilled through the skull. This standard approach can be challenging for targets in the caudal brainstem and upper cervical cord due to specific anatomical challenges as these sites are remote from skull landmarks, leading to imprecision. Here we outline an alternative stereotaxic approach via the cisterna magna that has been used to target discrete regions of interest in the caudal brainstem and upper cervical cord. The cisterna magna extends from the occipital bone to the atlas (i.e., the second vertebral bone), is filled with cerebrospinal fluid, and is covered by dura mater. This approach provides a reproducible route of access to select central nervous system (CNS) structures that are otherwise hard to reach due to anatomical barriers. Furthermore, it allows for direct visualization of brainstem landmarks in close proximity to the target sites, increasing accuracy when delivering small injection volumes to restricted regions of interest in the caudal brainstem and upper cervical cord. Finally, this approach provides an opportunity to avoid the cerebellum, which can be important for motor and sensorimotor studies.

Are fetal gender and gestational age related to the size of cisterna magna?

OBJECTIVES: The purpose of this article was to explore whether the gestational age(GA)and gender could affect the size of the cisterna magna (CM). METHODS: This study that included pregnant women who were between 20 ∼ 39+6. The recorded included BPD, HC, anteroposterior diameter of CM and gender. The fetuses were divided into normal and isolated enlargement of the CM (IECM)group for statistical analysis. RESULTS: Seven hundred ninety six fetuses with normal CM, 412 cases were boys and 384 cases were girls. 73 fetuses with IECM, 59 cases were boys and 14 cases were girls. The anteroposterior diameter of the CM increased with GA during 20-26+6 weeks. After 27 weeks, the anteroposterior diameter of CM became stable. In the IECM group, the mean anteroposterior of male and female fetuses were 1.31 ± 0.18 cm and 1.24 ± 0.15 cm, respectively. The IECM fetus accounted for 8.4% of the total number of fetuses, male IECM accounted for 14.3% of normal male fetus, and female fetus was 3.6%, which showed that male fetus had a higher rate of IECM than female (χ2 = 21.6, p<.001). CONCLUSIONS: There is a gender difference between normal fetuses and IECM fetuses. Based on our finding, it is reasonable to establish the normal value of CM according to the gender difference.

Fetal intracranial structures: differences in size according to sex.

OBJECTIVES: We aimed to compare gender difference on sizes of some structures in the brain of normal male and female fetuses between 20 and 22 week gestations. METHODS: A total of 300 female and 300 male singleton pregnancies with low risk were included in the study. Biparietal diameter, head circumference, transcerebellar diameter, cisterna magna, nuchal fold thickness, anterior and posterior horn of lateral ventricles, length and width of cavum septum pellucidum were measured transabdominally. Mean±SD values were calculated and comparison of measurements were done between male and female fetuses. Kolmogorov-Smirnov and independent samples t-test were used for statistical analysis. A value of p<0.05 were accepted as statistically significant. RESULTS: We determined statistically significant difference in sizes of some structures of the brain of male and female fetuses. Mean±SD value of cavum septi pellucidi width was 3.38±0.61 and 3.85±0.96 in female and males, respectively (p<0.05). Male fetuses were also found to have larger anterior (1.92±0.30 vs. 1.58±0.26, p<0.0001) and posterior horn of lateral ventricles (6.00±0.87 vs. 5.53±1.17, p<0.05). CONCLUSIONS: Difference in sizes of some structures of the brain starts in fetal life. This finding may be important in evaluating the intracranial structures more precisely. These results may also give a contribution to the understanding physiological and pathologic differences between males and females.

An Uncommon Case of Neuroenteric Cyst Located at the Lateral Cerebellomedullary Cistern.

We present the case of a 42-year-old woman with a 5-week history of headache, progressive dysphagia, dysphonia, and hoarseness. A brain magnetic resonance imaging revealed an extra-axial cyst of the left lateral cerebellomedullary cistern. The subsequent histopathologic examination diagnosed a neuroenteric cyst. This case sheds light on the pivotal role of histologic identification of neuroenteric cyst, which could be crucial for further diagnostic investigations, especially in pediatric patients.

Adult cerebellopontine angle ependymoma presenting as an isolated cisternal mass: A case report.

INTRODUCTION: Ependymomas represent approximately 2%-8% of all primary intracranial brain tumors. The occurrence of extra-axial posterior fossa ependymomas in adults is rare. CASE AND OUTCOMES: We report a case of extra-axial cerebellopontine (CP) angle ependymoma in an adult patient, managed through gross total resection (GTR) and adjuvant radiotherapy. At her one-year postoperative visit, the patient remained clinically stable without any symptoms or focal neurological deficit and a follow up MRI showed no evidence of tumor recurrence. DISCUSSION: Only six cases of adult cerebellopontine angle ependymomas have been reported in the English literature, with the left side affected more commonly. Including this case, the mean age of the reported cases of adult extra-axial CP angle ependymoma is 44.14 years (range 22-66 years). Men accounted for five out of seven cases (71.4%). Maximal surgical resection is the mainstay of treatment in extra-axial CP angle ependymomas. Among seven reported cases, five received GTR and two had subtotal resection (STR). Patients were followed an average of 13.6 months (range 2-30 months) and only two patients with STR died during the follow-up period (6 weeks and 2 months after surgery). Six of the seven reported cases (including this one) received adjuvant radiotherapy. CONCLUSION: Although rare, extra-axial CP angle ependymomas should be considered as a differential diagnosis to other lesions of the CPA. Radical resection, whenever possible, is usually associated with a good outcome. Adjuvant radiotherapy remains an optional treatment with an unknown impact on overall and progression-free survival.

Single median maxillary central incisor syndrome and variant in SMO gene associated with SHH pathway.

Solitary median maxillary central incisor syndrome (SMMCI) is a rare congenital oronasal-dental midline anomaly. The aim of this paper is a presentation of a patient with SMMCI without other visible dentofacial anomalies, with a potentially new molecular etiology consisting of a gene-gene reaction and conservative therapeutic approach to nasal obstruction. Potentially pathogenic variants in the SMO gene (p.Gly422Glu) and in P2RY13 gene (p.Trp205*) inherited from the probant's father, and in the PLD2 gene (p.Gln319fs), inherited from the mother were found. A multidisciplinary approach is necessary for the management of patients with SMMCI, including a genetic consultation with genetic tests.

Three-dimensional volumetric study with VOCAL in normal and abnormal posterior fossa fetuses.

Purpose: The aim of this study was to compare volumetric parameters in the abnormal and normal posterior fossa using the Virtual Organ Computer-aided AnaLysis (VOCAL™) technique to determine whether fetuses with an abnormal posterior fossa have different volumes.Methods: A prospective study was conducted on 17 fetuses with an abnormal posterior fossa including, Dandy Walker malformation (DWM) (n = 6), vermian hypoplasia (VH) (n = 3), mega cisterna magna (MCM) (n = 8), and 99 healthy control fetuses from 20 to 34 weeks' gestation. Measurement of the fetal cisterna magna and cerebellar volume was performed in the standard transcerebellar plane through the VOCAL™ method. To establish the correlation of volumes with gestational age, polynomial regression analysis was performed. For comparison between groups, univariate ANCOVA was performed using gestational age as a covariate. The reliability was analyzed by the intraclass correlation coefficient (ICC).Results: Cerebellar volume and cisterna magna volume were correlated with gestational age. Posterior fossa volume was significantly larger in DWM (p < .0001) and MCM (p < .0001) in comparison to the control group. In VH group, cisterna magna volume does not seem to expand (p = .298). Cerebellar volume does not seem to change in subgroups when the influence of gestational age is discarded (p = .09). The ratio of cerebellar volume to the cisterna magna volume decreases significantly in abnormal fetuses (p < .0001). Good intraobserver and interobserver reliabilities were found for both cerebellum and cisterna magna measurements.Conclusions: Volume analysis may have a role in discrimination of different posterior fossa pathologies.

Is Cisterna Magna Width a Useful First-Trimester Marker of Aneuploidy?

OBJECTIVE: To assess whether the cisterna magna (CM) width measured in first-trimester fetuses is a useful marker for aneuploidy detection. METHODS: This was a prospective study in 2 different cohorts in a tertiary referral center. The first cohort comprised 913 fetuses from the general pregnancy population during the period 2012-2016 and was used to construct the CM reference ranges applying the λ-µ-σ (LMS) method. The second cohort included 714 high-risk fetuses undergoing chorionic villus sampling during the period 2012-2016. Mean detection rates using the 95th percentile for CM width observed in chromosomal anomaly groups were compared with those obtained in chromosomally normal fetuses. RESULTS: The 50th percentile for CM ranged from 1.66 to 2.75 mm when crown-rump length (CRL) increased from 45 to 84 mm. Among high-risk fetuses, the following chromosomal anomalies were diagnosed in 125 (17%) fetuses: trisomy 21 (n = 63), trisomy 18 or 13 (n = 21), monosomy X (n = 9), submicroscopic anomalies (n = 11), and other anomalies (n = 22). The mean CM width for euploid fetuses was 2.4 mm (1.13 multiples of the median, MoM). While CM width was significantly increased in trisomy 21 (mean 2.7 mm; 1.23 MoM; p > 0.05), no differences were found in the other anomaly groups. Among the 63 fetuses with trisomy 21, a CM width above the 99th percentile was observed in 23 fetuses (37%). CONCLUSIONS: The new reference range for CM width at 11-13 weeks of gestation did not differ from previous studies. In first-trimester fetuses with trisomy 21, CM width appears to be increased, although its value as an ultrasound marker is limited, because of its detection rate of 37%.

Lymphatic outflow of cerebrospinal fluid is reduced in glioma.

Glioblastoma is a malignant brain tumor with mean overall survival of less than 15 months. Blood vessel leakage and peritumoral edema lead to increased intracranial pressure and augment neurological deficits which profoundly decrease the quality of life of glioblastoma patients. It is unknown how the dynamics of cerebrospinal fluid (CSF) turnover are affected during this process. By monitoring the transport of CSF tracers to the systemic blood circulation after infusion into the cisterna magna, we demonstrate that the outflow of CSF is dramatically reduced in glioma-bearing mice. Using a combination of magnetic resonance imaging (MRI) and near-infrared (NIR) imaging, we found that the circulation of CSF tracers was hindered after cisterna magna injection with reduced signals along the exiting cranial nerves and downstream lymph nodes, which represent the major CSF outflow route in mice. Due to blockage of the normal routes of CSF bulk flow within and from the cranial cavity, CSF tracers were redirected into the spinal space. In some mice, impaired CSF clearance from the cranium was compensated by a lymphatic outflow from the sacral spine.

Efficacy of cyst-cisternal shunt for refractory cyst regrowth of cystic vestibular schwannomas.

Vestibular schwannomas (VSs) are generally benign and slow-growing tumors, and microsurgical resection is the commonly recommended treatment. Some reports suggested that inserting a cystoperitoneal shunt was effective for treatment of cystic VSs; however, there was no report of a cyst-cisternal shunt which diverts cyst fluid into cistern. We report a case of cystic VS with repeated cyst regrowth within weeks after repeated surgeries. We prevented further recurrence using cyst-cisternal shunt. This technique may be a new treatment option for refractory cyst regrowth of cystic VSs.

The Clinical and Molecular Characteristics of Molybdenum Cofactor Deficiency Due to MOCS2 Mutations.

BACKGROUND: We explored the clinical and molecular characteristics of molybdenum cofactor deficiency due to MOCS2 muations. METHODS: We summarize the genetic and clinical findings of previously reported patients with a MOCS2 mutation. We also present a new patient with novel neuroradiological findings associated with molybdenum cofactor deficiency due to a novel homozygous variant in the 5' untranslated region of the MOCS2 gene. RESULTS: The study population comprised 35 patients with a MOCS2 gene mutation. All reported children had delayed motor milestones. The major initial symptom was seizures in neonatal period. Facial dysmorphism was present in 61% of the patients. Only one patient had ectopia lentis. Agenesis of the corpus callosum and an associated interhemispheric cyst in our case are novel neuroradiological findings. CONCLUSIONS: The occurrence of neonatal seizures and feeding difficulties can be the first clinical signs of molybdenum cofactor deficiency. Although there is no effective therapy for this condition, early diagnosis and genetic analysis of these lethal disorders facilitate adequate genetic counseling.

Sintomatología derivada de los pares craneales: Clínica y topografía / Cranial nerve disorders: clinical manifestations and topography

La detección de la patología relacionada con los doce pares craneales representa un importante desafío, tanto para los clínicos como para los radiólogos. Las técnicas de imagen son fundamentales para el manejo de muchos de los pacientes. Adicionalmente al conocimiento anatómico y de las entidades patológicas que potencialmente puedan afectarlos, la evaluación por imagen de los pares craneales requiere protocolos de exploración y técnicas de adquisición y procesado específicas. En este artículo se efectúa un repaso de los principales síntomas y síndromes relacionados con los nervios craneales que pueden precisar la realización de pruebas de imagen y la patología subyacente, así como una breve revisión de la anatomía y de las técnicas de imagen más adecuadas a la indicación

The detection of pathological conditions related to the twelve cranial pairs represents a significant challenge for both clinicians and radiologists; imaging techniques are fundamental for the management of many patients with these conditions. In addition to knowledge about the anatomy and pathological entities that can potentially affect the cranial pairs, the imaging evaluation of patients with possible cranial pair disorders requires specific examination protocols, acquisition techniques, and image processing. This article provides a review of the most common symptoms and syndromes related with the cranial pairs that might require imaging tests, together with a brief overview of the anatomy, the most common underlying processes, and the most appropriate imaging tests for different indications

Comparative study of intracisternal kaolin injection techniques to induce congenital hydrocephalus in fetal lamb.

PURPOSE: Kaolin (aluminum silicate) has been used to generate hydrocephalus by direct cisterna magna injection in animal models. The aim of the present study is to compare which method of Kaolin injection into fetal cisterna magna is feasible, safer, and more effective to induce hydrocephalus in fetal lambs. METHODS: Twenty-five well-dated pregnant ewes at gestational 85-90 days (E85-90) were used to compare three different kaolin injection puncture techniques into the fetal cisterna magna. Group 1, ultrasound guidance in a maternal percutaneous transabdominal (TA); group 2, without opening the uterus in a transuterine (TU) technique; group 3, by occipital direct access after exteriorizing fetal head (EFH); and group 4, control group, was normal fetal lambs without injection. The fetal lambs were assessed using lateral ventricle diameter ultrasonographic measurements prior the kaolin injection and on the subsequent days. We analyzed the effectivity, mortality, and fetal losses to determine the best technique to create hydrocephalus in fetal lamb. RESULTS: After fetal intracisternal kaolin (2%, 1mL) injection, lateral ventricle diameters increased progressively in the three different interventional groups compared with the normal values of the control group (p ≤ 0.05). We observed that the transabdominal method had a 60% of fetal losses, considering failure of injection and mortality, compared with the 12.5% in the open group (EFH), and 0% for the transuterine group. CONCLUSIONS: Based on our study, we believe that both, open uterine (EFH) and transuterine approaches are more effective and safer than the transabdominal ultrasound-guided method to induce hydrocephalus.

Risk factors for shunt dependency in patients suffering from spontaneous, non-aneurysmal subarachnoid hemorrhage.

Patients presenting with spontaneous, non-aneurysmal subarachnoid hemorrhage (SAH) achieve better outcomes compared to patients with aneurysmal SAH. Nevertheless, some patients develop shunt-dependent hydrocephalus during treatment course. We therefore analyzed our neurovascular database to identify factors determining shunt dependency after non-aneurysmal SAH. From 2006 to 2016, 131 patients suffering from spontaneous, non-aneurysmal SAH were admitted to our department. Patients were stratified according to the distribution of cisternal blood into patients with perimesencephalic SAH (pSAH) versus non-perimesencephalic SAH (npSAH). Outcome was assessed according to the modified Rankin Scale (mRS) at 6 months and stratified into favorable (mRS 0-2) versus unfavorable (mRS 3-6). A multivariate analysis was performed to identify predictors of shunt dependency in patients suffering from non-aneurysmal SAH. Overall, 18 of 131 patients suffering from non-aneurysmal SAH developed shunt dependency (14%). In detail, patients with npSAH developed significantly more often shunt dependency during treatment course, when compared to patients with pSAH (p = 0.02). Furthermore, patients with acute hydrocephalus, presence of intraventricular hemorrhage, presence of clinical vasospasm, and anticoagulation medication prior SAH developed significantly more often shunt dependency, when compared to patients without (p < 0.0001). However, "acute hydrocephalus" was the only significant and independent predictor for shunt dependency in all patients with non-aneurysmal SAH in the multivariate analysis (p < 0.0001). The present study identified acute hydrocephalus with the necessity of CSF diversion as significant and independent risk factor for the development of shunt dependency during treatment course in patients suffering from non-aneurysmal SAH.

Non-dysraphic intramedullary lipoma of the craniocervical region in an adult: Case report.

Intradural spinal lipomas with intracranial extension are very rare and are typically diagnosed in childhood. Radical surgical excision usually causes a high rate of morbidity because of the firm adherence between the lipoma and neural tissues. In this report, we present a case of craniocervical intradural intramedullary lipoma in an adult patient. The patient underwent surgery with excision of the mass, leaving a sheet of lipoma on the tumor bed.