Cerebrospinal Axis Research Articles

Case Study on the Role of Neuromotor Re-Education Methods in the Treatment of Charcot Marie-Tooth Disease

The isolated Charcot disease occurs in young people and has a motor onset, being a touch of motor cells, anywhere in the cerebrospinal axis. The paper is a case study of a subject diagnosed with Charcot Marie-Tooth disease. The physical therapy intervention aimed to apply elements of neuromotor re-education methods over a period of nine months. The results reveal that combining neuromotor re-education methods contributes to improving muscle elasticity and capsuloligamentous structures, thus preventing the formation of dysfunctional positions, and induces voluntary motor activity in the affected muscles, thus improving muscle imbalances specific to pathology.

Chronic subdural hematoma associated with type II and type III Galassi arachnoid cysts: illustrative cases.

Arachnoid cysts (ACs) are congenital abnormalities that can be located anywhere within the subarachnoid space along the cerebrospinal axis, although they are most often found on the left side in the temporal fossa and sylvian fissure. ACs comprise approximately 1% of all intracranial space-occupying lesions and are considered potential risk factors for subdural hematoma (SDH) in individuals of all age groups who have experienced traumatic brain injury. Although it is uncommon for an intracystic hemorrhage of an AC to occur without evidence of head trauma, it may be more common among children and young adults. Here, the authors present three cases of spontaneous AC intracystic hemorrhage with chronic SDH. Additionally, they provide a thorough review of the existing literature. All three patients with AC were adolescent males. In all cases, AC was identified using the Galassi classification (type II or III) and associated with spontaneous intracystic hemorrhage and chronic SDH as seen on imaging. Spontaneous intracystic hemorrhage is a rare complication and occurs most commonly on the left side. Surgery is the definitive treatment, requiring either craniotomy or burr hole for hematoma evacuation and microsurgical fenestration to drain the cyst into the subarachnoid cisterns.

Survival and prognostic factors in adult medulloblastoma: the Salah Azaiz Institute experience

Background Medulloblastoma is the most common malignant brain tumor in children. This entity in adulthood is rare. The aim of our study is to evaluate therapeutic results and prognostic factors of adult medulloblastoma treated at our institute with post-operative radiotherapy. Methods We retrospectively reviewed a cohort of 55 patients with medulloblastoma who underwent radiation in the department of radiation oncology of institute Salah Azaiz (Tunis) over a 18-year period (1994–2012). Results The surgery was total or subtotal resection in 73% of cases. Forty-eight patients received radiotherapy to the entire craniospinal axis as part of the curative treatment. The median interval from surgery to the initiation of radiotherapy was 83 days. Etoposide-cisplatin chemotherapy was only performed in metastatic patients (n = 4). The 5-years and 10-years overall survival rates were respectively 53 and 34%. The dose of radiotherapy to the craniospinal axis was a prognostic factor. The 5-years and 10-years event-free-survival rates were 64 and 41%. Reduction in the dose of radiotherapy to the craniospinal axis and fourth ventricular floor involvement were correlated with a worse event-free survival. Conclusion Our results, compared to those of the literature, conclude that the reduction in the dose of radiotherapy to the craniospinal axis (<34 Gy) in the standard risk group of adult medulloblastoma could not be done without chemotherapy. In the high-risk group of adult medulloblastoma, radiotherapy to the cerebrospinal axis at the dose of 36 Gy with chemotherapy, is required for disease control.

Abstract A08: IGF1R as a key target in high risk, metastatic medulloblastoma

Abstract The risk or presence of metastasis in childhood medulloblastoma represents a substantial cause of morbidity and mortality. Relatively little has been known about the molecular mechanisms of medulloblastoma dissemination throughout the cerebrospinal axis. To determine potential interventions that address both metastasis and tumor growth, we examined the expression of cytokines and growth factors in the CSF of metastatic medulloblastoma patients that were also present in the conditioned media of metastatic, MYC amplified medulloblastoma cell lines and/or cell culture models of the leptomeninges. The presence of IGF1, the IGF-sequestering protein Igfbp3, which can be cleaved by metal metalloproteases (MMP) and the serine protease tPA, and the presence of corresponding protease modulators TIMP1 and SerpinE1/PAI-1, led us to explore the bioactivity of IGF1 in medulloblastoma. IGF1 led not only to receptor phosphorylation but also increased migration and increased relative cell growth. A tumor cell viability screen of 60 clinically-related compounds prioritized IGF1R inhibitors. Our findings support a model whereby medulloblastoma tumor cells actively migrate, rather than arrive passively, to the leptomeningeal cell surface and provide a rationale for exploring IGF1R as a target for therapeutics in future clinical trials. Citation Format: Matthew N. Svalina, Ken Kikuchi, Jinu Abraham, Sangeet Lal, Monika A. Davare, Jennifer L. Peckham, Yoon-Jae Cho, Melanie Jackson, Daniel J. Guillaume, Nathan R. Selden, Darell D. Bigner, Kellie J. Nazemi, Sarah C. Green, Christopher L. Corless, Sakir Gultekin, Brian P. Rubin, Randall Woltjer, Charles Keller. IGF1R as a key target in high risk, metastatic medulloblastoma. [abstract]. In: Proceedings of the AACR Special Conference: Advances in Brain Cancer Research; May 27-30, 2015; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2015;75(23 Suppl):Abstract nr A08.

Case Report on Removing Large Schwannoma in Axilla

Introduction Schwannomas can achieve huge sized masses along the cerebrospinal axis and peripheral nerves.

Altered Functionality, Morphology, and Vesicular Glutamate Transporter Expression of Cortical Motor Neurons from a Presymptomatic Mouse Model of Amyotrophic Lateral Sclerosis.

Amyotrophic lateral sclerosis (ALS) is a lethal disorder characterized by the gradual degeneration of motor neurons in the cerebrospinal axis. Whether upper motor neuron hyperexcitability, which is a feature of ALS, provokes dysfunction of glutamate metabolism and degeneration of lower motor neurons via an anterograde process is undetermined. To examine whether early changes in upper motor neuron activity occur in association with glutamatergic alterations, we performed whole-cell patch-clamp recordings to analyze excitatory properties of Layer V cortical motor neurons and excitatory postsynaptic currents (EPSCs) in presymptomatic G93A mice modeling familial ALS (fALS). We found that G93A Layer V pyramidal neurons exhibited altered EPSC frequency and rheobase values indicative of their hyperexcitability status. Biocytin loading of these hyperexcitable neurons revealed an expansion of their basal dendrite arborization. Moreover, we detected increased expression levels of the vesicular glutamate transporter 2 in cortical Layer V of G93A mice. Altogether our data show that functional and structural neuronal alterations associate with abnormal glutamatergic activity in motor cortex of presymptomatic G93A mice. These abnormalities, expected to enhance glutamate release and to favor its accumulation in the motor cortex, provide strong support for the view that upper motor neurons are involved early on in the pathogenesis of ALS.

Microbiota disorders and food hypersensitivity in autism spectrum disorders; what do we know?

Pathogenesis of autism spectrum disorders (ASD) is probably multifactorial. Many studies have shown intestinal dysbiosis in children with autism. Moreover, gastrointestinal disturbances, probably resulting from abnormal microbiota composition, are commonly reported in children with autism. Another aspect, that may have negative influence on ASD children’s behavior are enzymopathies (enzymatic deficiency). Lack of selected gastrointestinal enzymes leads to inappropriate nutrients decomposition (mainly gluten and casein) and formation of so called exorphins, i.e. substances with opioid activity (opioid therapy). Because of increased permeability of the intestinal barrier and cerebrospinal axis, exorphins and others improperly digested food particles may be transported to the central nervous system. Consequently, disorders in brain development and children’s behavior deterioration may be observed. Studies have shown that both microflora disorders and increased intestinal permeability may contribute to behavioral impairment.

Arachnoid cyst presenting with endocrine manifestations: Tale of two patients

Arachnoid cysts are benign cysts that occur in the cerebrospinal axis in relation to the arachnoid membrane. These are usually congenital and asymptomatic. We herein report endocrine manifestations associated with large intracranial arachnoid cyst in two of our patients, the first case presented with the combination of precocious puberty and growth hormone deficiency and the second case with precocious puberty; subsequently, we discuss the relevant literature on this association.

Familial Replicating Arachnoidal Cysts: Case Series and Review of Literature

Background: Arachnoid cysts are intra-arachnoid fluid collections covered by a thin membrane that may develop throughout the cerebrospinal axis. Although the precise causative mechanism is unknown, arachnoid cyst (AC) are now generally accepted to be developmental anomalies of arachnoid. These lesions have commonly been described in the literature; however the presence of familial arachnoid cysts is quite rare. Most genetically related AC have been documented in patients with a known genetic syndrome. The current case report describes a family with four members affected by an arachnoid cyst in the same region. Methods: In addition to reviewing the current case, a literature search was conducted using National Library of Medicine and National Institutes of Health databases to identify articles pertaining to familial Arachnoid cysts. Overall, 32 published articles fit the established review criteria Results: We describe a family whose members (father and three siblings) present an arachnoid cyst in the same region (the left middle cranial fossa). The general physical findings in the four members were normal and no clinical suggestion of a genetic syndrome. None of the members had an increased head size or abnormal cutaneous findings. Histories of prenatal and perinatal periods were unremarkable. All were born normally at term and none had any history of intrauterine exposure to infection, drugs, teratogens or trauma. Karyotyping failed to reveal abnormalities. Conclusion: This report extends previous observations that AC can be familial and supports the hypothesis that some cases have a genetic aetiology. The lack of chromosomal or genetic studies in these patients supports the need for additional research into the mechanism of AC formation.

Long term follow up after surgery for benign intracranial cysts in children

Arachnoid cysts are cystic malformations in cerebrospinal axis found in both adults and children. While most arachnoid cysts are asymptomatic and usually go undetected, some cause symptoms and warrant surgical treatment. In this prospective study we aimed to describe the result of short- and long term follow up in children referred to our center with a cystic malformation.

Paramedic Suicide with Vecuronium Bromide � A Case Report

Paramedic Suicide with Vecuronium Bromide – A Case Report Skeletal muscle relaxants are drugs that act peripherally at neuromuscular junction/ muscle fiber itself or centrally in the cerebrospinal axis to reduce muscle tone and/ or cause paralysis. The neuromuscular blocking agents are used primarily in conjunction with general anesthesia to provide muscle relaxation for surgery, while centrally acting muscle relaxants are used mainly for painful muscle spasms and spastic neurological conditions.

Neuropeptides Y, YY, PP and their clinical significance

peripheral nervous system. Considering the structure and evolutionary origin, neuropeptide Y (NPY) is a peptide of the same family as peptide YY (PYY) and pancreatic polypeptide (PP). These proteins were discovered relatively recently, however, knowledge about them is deepened. They are 36-amino acid peptide acting through G-protein coupled receptors, Y1, Y2, Y3, Y4, Y5 and Y6. The diverse structure C-terminus of the peptide and protein binding to receptors affect the biological activity and the physiological effects on the digestive system, blood vessels, and the center of hunger and satiety in the hypothalamus. Peptides have anorexic properties, they regulate appetite and food intake mainly through the intestinal cerebrospinal axis and the hypothalamus. These substances represent an important potential target of new drugs in the long-term treatment and prevention of obesity. Furthermore, neuropeptide Y affects many processes depending on the central nervous system modifies ethanol consumption, affect circadian rhythms, memory processes, anxiety behavior. Peripherally NPY affects smooth muscle contraction of the blood vessels, blood pressure, and atherogenic processes. Conducted more thorough research trying to define the role and participation of various neuropeptides in the development of diseases of the pancreas and the gastrointestinal tract, cardiovascular system and use it for diagnosis.

Sox2 Requirement in Sonic Hedgehog-Associated Medulloblastoma

The transcription factor Sox2 has been shown to play essential roles during embryonic development as well as in cancer. To more precisely understand tumor biology and to identify potential therapeutical targets, we thoroughly investigated the expression and function of Sox2 in medulloblastoma, a malignant embryonic brain tumor that initiates in the posterior fossa and eventually spreads throughout the entire cerebrospinal axis. We examined a large series of tumor samples (n = 188) to show that SOX2 is specifically expressed in Sonic hedgehog (SHH)-associated medulloblastoma with an interesting preponderance in adolescent and adult cases. We further show that cerebellar granule neuron precursors (CGNP), which are believed to serve as the cell of origin for this medulloblastoma subgroup, express Sox2 in early stages. Also, Shh-associated medulloblastoma can be initiated from such Sox2-positive CGNPs in mice. Independent of their endogenous Sox2 expression, constitutive activation of Shh signaling in CGNPs resulted in significantly enhanced proliferation and ectopic expression of Sox2 in vitro and Sox2-positive medulloblastoma in vivo. Genetic ablation of Sox2 from murine medulloblastoma did not affect survival, most likely due to a compensatory overexpression of Sox3. However, acute deletion of Sox2 from primary cultures of CGNPs with constitutive Shh signaling significantly decreased proliferation, whereas overexpression of Sox2 enhanced proliferation of murine medulloblastoma cells. We conclude that Sox2 is a marker for Shh-dependent medulloblastomas where it is required and sufficient to drive tumor cell proliferation.

Inguinal lymph nodal metastasis of myxopapillary ependymoma confirmed by fine‐needle aspiration cytology, biopsy, and immunohistochemistry: Case report

Ependymoma (EP) rarely metastasizes outside the central nervous system. Inguinal nodule metastasis of EP more than 10 years after surgical resection and radiotherapy is extremely rare. We report a man aged 38 years who underwent surgery for lumbosacral myxopapillary EP at the age of 22 years and was treated with several cycles of radiotherapy. The patient was reoperated for residual tumor and received two complete cycles of radiotherapy for 11 years. Biopsies were always diagnosed as myxopapillary EP. Five years after the last surgical excision, the patient developed abdominal pain and inguinal lymphadenopathy. Biopsy was performed by fine-needle aspiration and was proven malignant epithelial neoplasm with a myxoid background, was diagnosed as metastasis of EP. Biopsy showed an anaplastic EP grade III. EP is often recurrent at the primary site but can seed on the entire cerebrospinal axis. We describe the clinical features of this rare lesion and particularly emphasize the need for long-term follow-up, for more than 10 years after the initial treatment, in patients with EP and malignant transformation after radiotherapy.

A case of choroid plexus carcinoma with spinal metastasis in an adult

The author presents a rare case of choroid plexus carcinoma originated from right lateral ventricle and progressive multiple spinal seeding through cerebrospinal pathway after gross total resection of brain lesion in a 60-year old woman. The patient presented with one-year history of headache. nausea and vomiting. In spite of radical resection of the ventricular tumor followed by chemotherapy and radiation therapy over the cerebrospinal axis. the patient expired with respiratory failure one and half year after surgery.

Face-Related Ictal Hallucinations and Illusions and Reflex-Type Phenomena

Face-Related Ictal Hallucinations and Illusions and Reflex-Type Phenomena

SU‐FF‐T‐269: Comparison of Out‐Of‐Field Doses in Pediatric Patients From Craniospinal Irradiations Using Photon, Electron and Proton Spinal Fields

Purpose: Compare the out‐of‐field secondary doses to critical organs in pediatric patients resulting from three different craniospinal irradiation (CSI) treatments. Methods and Materials: A pediatric anthropomorphic Rando phantom was CT scanned for planning purposes following MDACC protocols. Based on the images three different treatment plans employing photon, electron and proton spinal fields, were designed. A common dose prescription for pediatric patients with CNS disease of 36 Gy to the cerebrospinal axis was used for all three plans. The photon and electron spinal fields plans were each delivered three times and the doses at several critical organ sites were measured with TLD. The proton delivery was simulated with MCNPX to calculate the out‐of‐field secondary neutron contribution to the doses at the same critical organ sites. Measured doses for all three treatments were compared. Results: In the majority of the investigated critical organ sites, the doses from the photon spinal fields were higher than from the electron spinal fields. Many of the critical organ doses beyond the spinal target were greater for the photon delivery than for the electron delivery by several orders of magnitude. The entrance doses, as well as the doses lateral to and outside of the target, were up to a factor of two greater for the electron delivery. The expectation for the proton delivery is that the out‐of‐field doses from the secondary neutrons will be less than the conventional electron and photon treatments. Conclusions: An improved clinical treatment decision for pediatric patients can be made using the comparisons between the out‐of field doses for three different treatment methods.Work supported by CA010953 awarded by NCI, DHHS

Lumbar epidural abscess caused by brucella species: report of two cases

Spinal epidural abscess due to Brucella species is usually associated with spondylodiscitis. Urgent surgical decompression should be performed in cases with moderate to severe neurological deficits particularly if progressive. We report clinical features of two cases operated for lumbar epidural abscess caused by Brucella species. Early surgical decompression combined with medical treatment could decrease progression of neurological findings or the severity of complications. Iatrogenic dural tear at the operation should be repaired immediately with fine sutures and fibrin tissue glue to prevent further innoculation into the cerebrospinal axis. These cases should be cautiously followed for any recurrence or neurobrucellosis.

Total resection of inferiorly located sacral chordoma with posterior only approach: case report and review of the literature

Chordoma is a primary sacral neoplasm of ectodermal origin and makes up %1- 4 of all primary bone tumors. It is usually present on the midline cerebrospinal axis and the most common locations are the spheno-clival region and the sacrum. The treatment of primary sacral tumors represents a challenge because of a large tumor mass at presentation and a hemorrhage risk in surgery. Sacral tumors may present a difficult problem to the surgeon who desires to obtain a clear margin of excision. Using the retrorectal fat tissue as a cleavage line in the posterior approach guides the neurosurgeon to resect the tumor totally and reduce the hemorrhage in sacral chordomas. In this case report, we tried to discuss the advantages of using of retrorectal fat tissue as a cleavage line in sacral chordomas under the literature.

Spontaneous Intracystic Hemorrhage of the Arachnoid Cyst: A Case Report and Review of Literature

Arachnoid cysts represent benign cysts that occur in the cerebrospinal axis in relation to the arachnoid membrane and do not communicate with the ventricular system. We report a case of a years right handed lady, who presented to the emergency department with the complaints of headache and vomiting for one week CT scan showed extraaxial cystic lesion in the left fronto-parietal region. On the fifth day of admission, patient had sudden onset of severe headache associated with loss of consciousness for about 3-4 minutes with neck rigidity. A CT scan of head was repeated, which showed left fronto-parietal cystic lesion with intracystic bleed and SAH. Intraoperatively, there was intradural cystic lesion containing xanthochromic fluid with normal brain surface and there were no evidence of any vascular malformations. Marsupilization of the cystic lesion was carried out and she improved. The literature regarding arachnoid cyst with spontaneous intracranial haemorrhage is reviewed.