PEPITEM Treatment Ameliorates EAE in Mice by Reducing CNS Inflammation, Leukocyte Infiltration, Demyelination, and Proinflammatory Cytokine Production.

To investigate the effect of the therapeutic treatment of the immunopeptide, peptide inhibitor of trans-endothelial migration (PEPITEM) on the severity of disease in a mouse model of experimental autoimmune encephalomyelitis (EAE) as a model for human multiple sclerosis (MS), a series of experiments were conducted. Using C57BL/6 female mice, we dosed the PEPITEM in the EAE model via IP after observing the first sign of inflammation. The disease was induced using MOG35-55 and complete Freund's adjuvants augmented with pertussis toxin. The EAE score was recorded daily until the end of the experiment (21 days). The histological and immunohistochemistry analysis was conducted on the spinal cord sections. A Western blot analysis was performed to measure the protein concentration of MBP, MAP-2, and N-Cadherin, and ELISA kits were used to measure IL-17 and FOXP3 in the serum and spinal cord lysate. The therapeutic treatment with PEPITEM reduced the CNS infiltration of T cells, and decreased levels of the protein concertations of MBP, MAP-2, and N-Cadherin were observed, in addition to reduced concertations of IL-17 and FOXP3. Using PEPITEM alleviated the severity of the symptoms in the EAE model. Our study revealed the potential of PEPITEM to control inflammation in MS patients and to reduce the harmful effects of synthetic drugs.

Schistosomiasis Involving the Central Nervous System: Case Report of a Rare Complication.

Introduction: Schistosomiasis is a parasitic infection caused by schistosome invasion of blood circulation. Neuroschistosomiasis is a severe cerebral complication that accounts for less than 2.3% of reported cases. Patients present with progressive encephalitis, seizures, or both. Management includes antiparasitic medications, steroids, and surgical intervention. Case Presentation. We report a case of a 44-year-old female who presented to the ER with a history of transient loss of consciousness (LOC) and seizure. Radiological investigations revealed a right frontal brain lesion. Histopathological results confirmed the diagnosis of schistosomiasis. Conclusion: Schistosomiasis is a parasitic infection commonly diagnosed in patients who live in tropical areas. Early diagnosis with radiological and histopathological evaluation is required to identify patients at risk of developing severe neurological complications.

Mesentric lymphatic malformation presented with a small bowel internal hernia: A case report.

We report a case of a 2 years and 8 months boy presented with a small bowel obstruction occurring in a setting of mesenteric lymphatic malformation (LM). The case did not showed typical features of LM and thought to be a simple ascites. Nevertheless, the mass effect of the lesion was the salient finding and the case presented with a small bowel internal hernia which is a rare presentation. This emergent case was managed surgically.

Metastatic brain lesion as the initial presentation of follicular thyroid carcinoma.

Background: Metastatic brain lesions, of thyroid origin, are rare manifestations of differentiated thyroid cancer, with papillary thyroid carcinoma being the most common subtype. Considering the rarity of metastatic follicular thyroid carcinoma to the brain, the present article outlines its clinical presentation, neuroradiological findings, pathological features, and outcome. Case Description: A 52-year-old female presented with a 6-month history of progressive and holocephalic headache. Examination revealed a tracheal deviation to the left side due to an enlarged goiter. Brain CT scan showed a right occipital, slightly hyperdense lesion associated with a 0.4 cm midline shift to the left side. Brain MRI demonstrated a right occipital, avidly-enhancing, extra-axial lesion with disproportionate and extensive vasogenic edema. As the lesion was solitary, the patient underwent craniotomy and tumor resection. Histopathological examination revealed a tumor consistent of small follicles, composed of uniform round nuclei without papillary thyroid carcinoma nuclear features, suggestive of metastatic follicular thyroid carcinoma to the brain. Postoperatively, the patient was neurologically intact. She was discharged in a stable condition with laboratory/ radiological investigations and follow-up at neurosurgery, endocrine, radiotherapy, and thyroid surgery clinics. Conclusion: Follicular thyroid carcinoma may rarely metastasize to the central nervous system. A high index of suspicion is required to identify patients with thyroid cancer who initially present with neurological manifestations. Complete surgical resection of the metastatic brain lesion is safe, feasible and is associated with a prolonged overall survival.

Intracranial tuberculomas: A case report of clinical, radiological, and pathological characteristics.

BACKGROUND: Intracranial tuberculomas are uncommon yet devastating forms of extrapulmonary tuberculosis with a high mortality rate and morbidity risk. A high level of suspicion is required for a prompt diagnosis and treatment. CASE DESCRIPTION: A 67-year-old male, medically free, presented at the Emergency Department with a 1-day history of nausea and vomiting, and a 15-day history of imbalance and dizziness. Radiological imaging demonstrated right well-defined ring-enhancing lesions. He underwent a sup-occipital craniotomy with lesion resection. The diagnosis of an intracranial tuberculoma was confirmed histopathologically. Anti-tuberculosis therapy was prescribed, and the patient was discharged with mild cerebellar dysfunction. DISCUSSION: Intracranial tuberculomas have a high rate of mortality and morbidity. It is critical to consider tuberculoma in the differential diagnosis of intracranial lesions with such clinico-radiological characteristics, especially in developing countries. CONCLUSION: In this article, we are reporting an interesting case with multiple intracranial tuberculomas with an extensive review of the literature.

A rare cerebellar vermis high-grade neuroepithelial tumor: Radiological-pathological correlation.

Neuroepithelial tumors, formerly known as primitive neuroectodermal tumors of the central nervous system, are reclassified under embryonal tumors in the 2016 WHO Classification of Tumors of the Central Nervous System. The tumor has two known genetic alterations: HGNET-MN1 and HGNET-BCOR. Previously, radiological features of the tumor have been reported as large, intra-axial lesions in the cerebral or cerebellar hemisphere, which presents mild adjacent edema. Here, we report the first case of high-grade neuroepithelial tumor not elsewhere classified (HGNET-NEC) arising from the cerebellar vermis, demonstrating good outcomes in clinical follow-up when compared with previously known types.

Cervical epidural neurosarcoidosis: A case report and literature review.

BACKGROUND: Neurosarcoidosis is a rare disease. In the spine, it commonly presents as an intramedullary lesion. Epidural spinal lesions are extremely rare. CASE DESCRIPTION: A 29-year-old patient presented with a 22-month history of progressive neck, upper limb pain, and myelopathy. The cervical MRI showed a large epidural mass infiltrating the paraspinal soft tissue. After an open biopsy, the diagnosis of neurosarcoidosis was established and was followed-up by appropriate medical management. CONCLUSION: To manage cervical epidural neurosarcoidosis, first, you must obtain a tissue diagnosis and then follow with appropriate medical management.

Brentuximab-vedotin maintenance following chemotherapy without irradiation for primary intracranial embryonal carcinoma in down syndrome.

BACKGROUND: Germ cell tumors (GCT) are the most common central nervous system (CNS) tumors in individuals with Down syndrome. Patients with Down syndrome treated with CNS irradiation are at increased risk of developing cerebrovascular complications such as moyamoya disease. Embryonal carcinoma components are recognized to be more resistant to conventional chemotherapy and radiotherapy and confer a very poor prognosis. CD30 is a member of the tumor necrosis factor-receptor superfamily. CD30+ has a limited expression in normal cells but is the defining marker for embryonal carcinoma. Brentuximab-vedotin is a novel antibody-drug conjugate consisting of the chimeric anti-CD30 antibody conjugated to an anti-tubulin synthetic analog monomethyl auristatin E. METHODS: A retrospective review of the patient's records was conducted in September 2017. RESULTS: We report upon our management of a teenage girl with Down syndrome and a suprasellar pure embryonal carcinoma utilizing an intensive chemotherapy regimen followed by brentuximab-vedotin without irradiation. The patient received two cycles of carboplatin and etoposide interspersed with one cycle of cyclophosphamide and etoposide for induction followed by three cycles of marrow-ablative thiotepa and carboplatin rescued by autologous hematopoietic stem cell. Finally, She received six cycles of intravenous brentuximab-vedotin. The patient continues without evidence of recurrent tumor by MRI and tumor marker surveillance 24 months since diagnosis, with no adverse sequelae of her treatment. CONCLUSIONS: Brentuximab-vedotin may provide a selective and safe alternative (or adjunct) to radiotherapy in the management of patients with CD30-positive CNS embryonal carcinoma, especially for those patients at high risk of developing irradiation-related complications.

Severe Crohn's Disease Manifestations in a Child with Cystathionine ß-Synthase Deficiency.

Inflammatory bowel diseases (IBDs) are idiopathic autoimmune diseases that are characterized by inflammation of both the small and large intestine. Although IBD is common in the general population, the pathophysiology remains ambiguous. Clear understanding of IBD pathophysiology would be a major step toward curative treatment in the future. Hyperhomocysteinemia has been associated with multiple autoimmune diseases including IBD, but homocystinuria has not been associated with IBD before. We report a 9-year-old girl with Crohn's disease and homocystinuria. Her gastrointestinal symptoms improved significantly upon classical homocystinuria treatment, and her last colonoscopy showed a pronounced remission. This case supports the inflammatory role of homocysteine in the gastrointestinal tract and the association between hyperhomocysteinemia and IBD manifestations.

Preterm birth and cerebellar neuropathology.

Improved survival rates in premature infants and more sensitive neuroimaging techniques have expanded the scope of recognized neurodevelopmental disabilities in this vulnerable population and have implicated a role for cerebellar pathology in their origin. Although supratentorial pathologies are well studied, cerebellar pathology has been under-recognized in premature infants. The purpose of this review is to provide a concise description of established acquired cerebellar pathologies in premature infants including cerebellar atrophy/hypoplasia, hemorrhage, and infarction. The cerebellum develops over an extended period during which time cerebellar injury tends to occur with the potential to derail the cerebellum from its expected growth trajectory and perturb the establishment of cerebellar neural circuitry. The occurrence of cerebellar injury in this vulnerable period may have life-long implications that extend beyond the immediate damage sustained by the cerebellum, all of which needs to be considered as we research the causes and effects of neurodevelopmental disabilities in these patients.

Neuropathology of temporal lobe epilepsy.

Pathologic findings in surgical resections from patients with temporal lobe epilepsy include a wide range of diagnostic possibilities that can be categorized into different groups on the basis of etiology. This paper outlines the various pathologic entities described in temporal lobe epilepsy, including some newly recognized epilepsy-associated tumors, and briefly touch on the recent classification of focal cortical dysplasia. This classification takes into account coexistent pathologic lesions in focal cortical dysplasia.