Novel concepts in the pathogenesis of hydrocephalus.

PURPOSE: Hydrocephalus is a multifactorial neurological disorder and one of the most common neurosurgical conditions characterized by excessive cerebrospinal fluid (CSF) accumulation within the brain's ventricles. It can result in dilatation of the ventricular system caused by the inadequate passage of CSF from its point of production within the ventricles to its point of absorption into the systemic circulation. Recent findings on the genetics and molecular studies of hydrocephalus have the potential to improve treatment and quality of life. METHODS: Review of literature on the novel studies of the pathogenesis of hydrocephalus. CONCLUSION: Molecular studies on the pathogenesis of hydrocephalus have provided a means to improve the treatment and follow-up of patients with hydrocephalus.

Exploring the Neuroprotective Effects of Spirulina platensis: Insights Into Hemorrhagic Volume and Histological Outcomes.

Background Hemorrhagic events can result in significant neurological damage, and identifying effective strategies for neuroprotection is crucial. Several studies have directed their attention to the alterations in perilesional parenchymal tissue. These investigations have sought to modify ischemic and metabolic changes by utilizing potential neuroprotective agents and to develop strategies that effectively mitigate secondary perilesional neuronal damage. By gaining a deeper understanding of its mechanisms and efficacy, Spirulina platensis can emerge as a promising therapeutic intervention for various neurological disorders. Methodology This controlled and blinded experimental study was conducted on adult male Wistar rats. The rats were divided into the treatment group, which received Spirulina platensis extract for 30 days before the hemorrhagic event, and the control group, where all animals underwent the same experimental hemorrhage model using collagenase. Each group was divided into the following three subgroups based on the sacrifice time: six hours, 24 hours, and 30 days. The brain section with the largest hemorrhage volume was selected for histological analysis. The number of viable neurons was analyzed in the perilesional zone and the cortical fields along the puncture trajectory. Neurofunctional evaluations were conducted on animals sacrificed 15 and 30 days after the procedure. Results Initial analysis showed no significant difference in viable neurons between groups (p = 0.63). Still, after 24 hours, the treatment group had a significantly higher number of viable neurons per peripheral fields (18.5) compared to the control group (13.4; p < 0.05). Neurofunctional tests at 15 days indicated a trend toward significance in absolute discrimination (p = 0.054), with the control group showing higher mean values (5.5, SD = 3.1) than the treatment group (-1, SD = 5.1). The discrimination index exhibited a significant difference (p < 0.01), with higher mean values in the control group (0.59, SD = 0.34) compared to the treatment group (-0.05, SD = 0.21). No significant differences were found in other neurofunctional parameters at this time point. At 30 days, no significant differences were observed in absolute discrimination, discrimination index, contralateral paw elevation, rearing time, and wire hanging time test (p > 0.1); however, the treatment group presented a better motor performance in the open field test (14.2, SD = 9.02) compared to the control group (5.25, SD = 2.06), approaching significance (p = 0.06). Conclusions The group treated with Spirulina platensis demonstrated significantly more viable neurons in the perilesional fields 24 hours after the induced hemorrhage. The treatment group also had a relatively better motor performance in the open field test 30 days after the hemorrhage (p = 0.06). These findings suggest a potential neuroprotection effect and warrant further investigations to explore the effects of Spirulina platensis and its active component phycocyanin in acute neurological conditions.

Unusual Presentation in Infratentorial Superficial Siderosis: Acute Intracranial Hypertension.

Superficial siderosis (SS) of the nervous system is a rare acquired condition related to hemosiderin deposits in subpial layers of the brain, brainstem, cerebellum, cranial nerves, and spinal cord, leading to brain iron-mediated neurodegeneration. The cardinal neurological features are slowly progressive hearing loss, ataxia, and pyramidal signs. Here we describe an atypical case of infratentorial SS evolving with acute intracranial hypertension in the absence of typical chronic signs.

Suprasellar Germinomas: 2 Case Reports and Literature Review.

BACKGROUND: Germinomas are rare malignant central nervous system tumors, a type of germ cell tumor, according to the 2016 World Health Organization Classification of Brain Tumors. Most of these tumors develop along the midline, most often from the pineal gland, followed by tumors arising in the suprasellar cisterns. Suprasellar germinomas commonly manifest with diabetes insipidus, visual impairment, and hypothalamic-pituitary failure. We present a literature review from the PubMed database and report 2 cases of suprasellar germinomas. CASE DESCRIPTION: Both pediatric patients presented with progressive visual loss; one did not show endocrinologic impairment, and the other presented with hypothyroidism, diabetes insipidus, and generalized edema. Magnetic resonance imaging showed expansive suprasellar masses in both cases, confirmed as germinomas at histopathologic examination. The patients were treated with the same surgical approach, although their outcomes were different because of endocrinologic and post surgical complications. CONCLUSIONS: Suprasellar germinomas are highly curable lesions when diagnosed early. The treatment remains controversial and should be individualized, but the association of chemotherapy and radiotherapy allows reduce adverse effects and shows great results.

Paracoccidioidomycosis in the Central Nervous System

Paracoccidioidomycosis is a systemicmycosis caused by the Paracoccidioides brasiliensis fungus, which is endemic in Latin America. Brazil is the country with the highest number of cases. The affection of the central nervous system (CNS), a potentially fatal condition, occurs in 12% of the cases. The following forms of presentation are identified:meningeal, which is unusual;meningoencephalitic; and pseudotumoral, the latter two being more frequent. Imaging tests are essential for the diagnosis, but the histological identification of the fungus is required for confirmation of the pathology. The clinical picture depends on the neuraxial location.We present a case of amale rural worker, with expansive lesions in the CNS compatible with paracoccidioidomycosis.

Olfactory Groove Schwannoma ­ A Case Report and Literature Review

Olfactory groove schwannomas (OGSs) are rare benign tumors of the anterior skull base region. Considering the lack of Schwann cells in the optic and olfactory nerves, their origin remains enigmatic. Despite the precursor cell, total resection of the lesion is curative, as long as the histopathological features of the tumor are compatible with schwannoma. We report the case of a 32-year-old woman, addicted to crack, who was brought to the hospital presenting with cognitive dysfunction after being physically assaulted, whose neuroimaging revealed a large extra-axial mass in the subfrontal sagittal region. The presentation, immunohistochemical markers and histogenesis are discussed in the present study, along with a literature review.

Supratentorial Atypical Rhabdoid Teratoid Tumor ­ A Case Report

Atypical rhabdoid teratoid tumors (ARTTs) are rare embryonic tumors, usually localized in the posterior fossa and diagnosed in children under 3 years-old. The treatment includes surgical resection, radio and chemotherapy, and the prognostic is unfavorable, with an average median survival of 1 year. We present the case of a 3-year-old patient, with history of headache and vomiting, followed by absence seizures, temporal automatism, syncope accompanied by sialorrhea and sphincteric loss succeeded by a postictal period. Surgical excision was performed and the anatomopathological study confirmed ARTT. The ARTTs are embryonic tumors, a category in which medulloblastoma and primitive neuroectodermal tumors (PNETs) represent the most common central nervous system (CNS) malignancies in childhood.

Tumores teratóides rabdóides atípicos (TTRA) são tumores embrionários raros, geralmente localizados na fossa posterior e diagnosticados em crianças com menos de 3 anos de idade. O tratamento inclui ressecção cirúrgica, radio e quimioterapia. Contudo, o prognóstico é desfavorável, com uma sobrevida média de 1 ano. Apresentamos o caso de um paciente de 3 anos de idade, com quadro de cefaléia e vômitos, companhados por automatismo temporal e perda de consciência, seguidos por período pós-ictal. A ressonância nuclear magnética (RNM) do encéfalo evidenciou lesão frontal compatível com tumor intra-axial, efeito de massa local e invasão do corpo caloso. Foi realizada excisão cirúrgica, e o estudo anatômico-patológico confirmou TTRA. Os TTRA são tumores embrionários, categoria na qual o meduloblastoma e os tumores neuroectodermais primitivos representam as malignidades mais comuns no sistema nervoso central de crianças.

Intracranial Mature Teratoma: A Case Report

Teratomas are the most common type of Germ Cell Tumors (GCTs). GCTs are classified as extragonadal, if there is no evidence of a primary tumor in neither the testicles, nor in the ovaries. Intracranial Mature Teratomas are tumors with a very low incidence, and clear male predominance. We present the case of a 21 year-old female patient, with a history of two seizures 15 days prior to admission, without any abnormalities upon physical examination. The Magnetic Resonance Imaging (MRI) performed at the admission evidenced an expansive, heterogeneous lesion in the frontal lobe, hypointense on T1-weighted images, hyperintense on T2-weighted images, and restriction on the diffusion imaging and ADC-mapping. The patient underwent microsurgical resection, and it was possible to achieve a near-total resection. During surgery, a welldefined capsule was identified, which was removed after adequate debulking. Tissues resembling hair were taken from inside the lesion. The patient recovered well, without any neurological deficits, and no further intervention was necessary. The authors aim to describe this rare pathology and their option for a surgical approach.

Os teratomas são o tipo mais comum de Tumores de Células Germinativas (TCG). TGC são classificados como extragonadais, quando não há evidências de um tumor primário em nos testículos, ou nos ovários. Os Teratomas Maduros Intracranianas são tumores com uma incidência muito baixa, e com uma predileção pelo sexo masculino. Nós apresentamos o caso de uma paciente, feminina, 21 anos de idade, com história de dois episódios de convulsões tônico-clônicas generalizadas, 15 dias antes da admissão, sem nenhum déficit focal ao exame físico. A Ressonância Magnética realizada na chegada mostrou uma lesão sólida, expansiva, grande e heterogênea, hipointensa em T1, e hiperintensa em T2, com restrição a difusão e ADC-mapping. Foi realizado uma cirurgia, e foi possível obter uma ressecção quase total. Havia uma cápsula bem definida, a qual foi removida após adequada redução do volume tumoral. Dentro da lesão foi encontrado tecido semelhante a cabelo. A paciente se recuperou bem, e foi dado alta sem novos déficits neurológicos, não foi realizado mais nenhuma intervenção, e ela está sendo acompanhada regularmente. Os autores visam descrever essa patologia rara e sua opção por uma abordagem cirúrgica.

Chiari IV Malformation, the Lückenschädel and Sagittal Craniosynostosis Association: Case Report and Literature Review / Associação entre malformação de Chiari IV, Lückenschädel e craniossinostose sagital: relato de caso e revisão de literatura

The association between Lückenschädel and craniosynostosis is unusual and unknown. Genetic origin is a possibility, representing one of many possible phenotypes for mutation. To the best of our knowledge, the association of such anomalies in a type IV Chiari malformation has never been reported before. The authors present the case of a patient with obstructive hydrocephalus, diagnosed with Chiari IV malformation associated with Lückenschädel and sagittal craniosynostosis. The Lückenschädel is the bone abnormality least commonly associated with Chiari malformation. It consists of a defect in the bones ofmembranous origin that formthe cranial vault. This anomaly arises from periosteal dysplasia, and is characterized by rounded and irregular gaps in the skull, bound by bony ridges. Craniosynostosis is due to premature fusion of the cranial sutures and is sometimes associated with the Chiari complex of malformations. The diagnostic of Lückenschädel and craniosynostosis is done by imaging, through which the skull assumes the aspect of a hive, characteristic of Lückenschädel and it is possible to see the premature fusion of the sutures. The Lückenschädel usually does not require treatment because of spontaneous resolution; whereas, craniosynostosis warrants surgery for aesthetic and functional reasons. The Chiari malformation IV only requires treatment when it results in syringomyelia or hydrocephalus.

O Lückenschädel e a craniossinostose são uma associação incomum e pouco conhecida, com possível origem genética, o que representa um dos muitos fenótipos possíveis para uma mutação. Até onde sabemos, a associação destas anomalias em um caso de malformação de Chiari tipo IV não foi relatada antes. Os autores apresentam o caso de um paciente com hidrocefalia obstrutiva, que foi diagnosticado com malformação de Chiari tipo IV associada com Lückenschädel e craniossinostose sagital. O Lückenschädel é a anormalidade óssea menos comum associada com a malformação de Chiari. É constituída por umdefeito nos ossos de origem membranosa que formam a abóbada craniana. Esta anomalia surge de displasia do periósteo e é caracterizada por falhas arredondadas e irregulares no crânio, delimitadas por cristas ósseas. A craniossinostose se deve à fusão prematura das suturas cranianas e é por vezes associada com o complexo de malformações de Chiari. Os diagnósticos de Lückenschädel e craniossinostose são feitos por imagem, onde o crânio assume um aspecto de colmeia, característico de Lückenschädel, e é possível ver a fusão prematura das suturas. O Lückenschädel geralmente não requer tratamento por causa de resolução espontânea e craniosynostosis necessita de cirurgia para fins estéticos e funcionais. A malformação de Chiari IV só requer tratamento quando resulta em siringomielia ou hidrocefalia.