The management of medulloblastoma, a pediatric brain tumor, has evolved significantly with the advent of genomic subgrouping, yet morbidity and mortality remain high in LMICs like Pakistan due to inadequate multidisciplinary care infrastructure. This paper aims to establish evidence -based guidelines tailored to the constraints of such countries. An expert panel comprising neuro-oncologists, neurosurgeons, radiologists, radiation oncologists, neuropathologists, and pediatricians collaborated to develop these guidelines, considering the specific challenges of pediatric brain tumor care in Pakistan. The recommendations cover various aspects of medulloblastoma treatment, including pre-surgical workup, neurosurgery, neuropathology, chemotherapy, radiation therapy, and supportive care. They offer both minimum required and additional optional protocols for more advanced centers, ensuring comprehensive patient management with attention to complications and complexities encountered in Pakistan. The paper's consensus guidelines strive for uniformity in healthcare delivery and address significant gaps in diagnosis, treatment, and follow-up of pediatric medulloblastoma patients.

Abstract BACKGROUND AND OBJECTIVE Alveolar soft part sarcoma is a rare malignancy of the lower extremities considered to be of unknown cellular origin. Common in young female patients, the ASPS usually has a strong metastatic potential, mostly involving the lungs at the time of presentation. Cases involving the CNS primarily, without any evidence of systemic disease have emerged i.e. the primary intracranial alveolar soft part sarcoma (PIASPS). We aim to determine the clinical manifestations, natural progression and management of primary intracranial alveolar soft part sarcoma. MATERIALS AND METHODS After an extensive literature search using PubMed Central and Google Scholar, seven case reports and two case series of 14 patients with PIASPS were retrieved and included using the Preferred Reporting Items for Systemic review and Meta-analyses guidelines, published between 2000 and 2023. RESULTS Female preponderance of the neoplasm (57.14%) with an average age of 24.9 years was noted. Headache (36.36%) and papilledema (25%) were the most common presenting complaint and sign, respectively. Brain parenchymal involvement was noted in 71.42% cases, mostly the frontal lobe and left hemisphere. The lesion appeared isointense to hypointense on T1-weighted images and hyperintense on T2-weighted images (66.67%) on MRI. Gross Total Resection was possible in 57.14% lesions. On immunohistochemistry, the tumor cells manifested TFE3 and PAS positivity in 25% and 20% of the cases respectively. An average survival after follow up duration was found to be 31.79 months. CONCLUSION Surgery and radiotherapy are the mainstay of treatment of PIASPS, while meningioma an important differential diagnosis. TFE3 fusion product is readily been helpful in diagnosis. Evidence of a primary lesion in the CNS cannot rule out the presence of an occult primary elsewhere in the body. This, along with the slow growing nature of the neoplasm and delayed localized recurrences, require a long-term follow-up.

Abstract BACKGROUND AND OBJECTIVE The most commonly reported congenital intracranial space occupying lesions are teratoma, glioma and neuroblastoma. Primary intracranial congenital glioblastoma (PICG) is one of the most lethal and extremely rare neoplasms. Most pediatricians and gynecologists are unable to detect it congenitally, posing an ominous risk of spread and disease severity. We aim to determine the clinical manifestations, number of reported cases, radiological findings and neuro-oncological management of PICG. MATERIALS AND METHODS A comprehensive literature search using PubMed Central and Google Scholar was done, retrieving 17 case reports and five case series of 31 patients with PICG and included using the Preferred Reporting items for Systematic Reviews and Meta-Analyses guidelines, published between 2000 and 2023. RESULTS The mean age at presentation was 86.00 ± 88.36 days, with both genders affected equally, i.e. 45.16%. The most common symptoms and signs at presentation were seizures (38.70%) and hydrocephalus (51.61%), respectively. Left hemispheric (48.38%), with frontoparietal involvement (16.12%) was the frequently affected site. MRI findings were suggestive of low intensity signal significant of hemorrhage or calcification and a heterogenous mass in 35.58% and 32.25% cases, respectively. Gross total resection (GTR) was achievable in 48.38% patients. Glial fibrillary acid protein and vimentin were positive in 45.16% and 29.03% cases, respectively. Intraoperative mortality and recurrence were noted in 25.80% and 9.68% of patients, respectively. The average follow up duration was 20.57 months. CONCLUSION PICG has an extremely aggressive course of natural progression. Congenital presentation and its lack of awareness among clinicians is itself a challenge for disease management. Adjuvant chemo-radiotherapy has proven fruitful along with GTR. Post-operative CSF diversion is warranted.

Background & Objective:Takotsubo cardiomyopathy (TCM), manifests as left ventricular dysfunction triggered by physical or emotional stress. It leads to higher morbidity in epileptic patients and can progress to complications. To find out the correlation between Takotsubo cardiomyopathy and epilepsy and to investigate pathophysiology and associated types of epilepsy. Methodology:This systematic review adhered to PRISMA guidelines and was sourced from the PubMed Central database. Search terms were pertinent to cardiomyopathy and epilepsy. Sixteen studies, comprising case reports and a case series were selected from 2000 to 2023 for data extraction. The quality evaluation was executed via the Joanna Briggs Institute Critical Appraisal checklist. Results:The review included 18 female patients with a mean age of 57.22 years. Predominant symptoms included tonic-clonic seizures (66.66%). Seizure-induced TCM pathophysiology implicates catecholamine surge, precipitating myocardial stunting and characteristic apical ballooning. Most patients had a history of epilepsy (38.88%). ECG findings showed tachycardia (38.88%) and ST-segment elevation (38.88%). Elevated troponin levels were noted in 83.33% of patients. Echocardiography showed reduced ejection fraction (72.22%), hypokinesia (38.88%) and akinesis (27.77%). Treatment involved Benzodiazepines (50%), Beta-blockers (61.11%) and Phenytoin (38.88%). The majority of patients showed improvement in echocardiography findings (55.55%) and ECG findings (11.11%). Conclusion:TCM in epilepsy patients evinces significant female predominance, with pathophysiology rooted in seizure-induced catecholamine surge. Early recognition in high-risk patients is essential in preventing complications.

Abstract BACKGROUND AND OBJECTIVE Primary CNS lymphoma accounts for three percent of all newly diagnosed CNS tumours and 1-3% of all non-Hodgkin’s lymphomas. The oncologic origin of primary intramedullary lymphoma of the spinal cord (PILSC) is an enigma. We aim to determine the clinical manifestations, disease course and management of PILSC. MATERIALS AND METHODS A comprehensive literature search using PubMed Central and Google Scholar, was done, retrieving 17 case reports and two case series of 21 patients with PILSC and included using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, published between 2000 and 2023. RESULTS About 90.48% patients inflicted with PILSC were adult, with male predominance, 61.90%. The most common complaint at presentation was neurological deficit of motor type in 32.14% patients. MRI proved to be helpful in 76.19% cases, with spinal cord enlargement and high signal intensity in 19.04% cases. Adjuvant chemotherapy and radiotherapy was acquired by 52.38% and 38.09% patients. Non-Hodgkin type was reported in 90.47% cases and Hodgkin lymphoma was seen in 9.52% patients. Diffuse large B cell lymphoma was culprit in 41.10% cases. Five out of 21 cases had variable post-operative complications. The average follow-up was 16.16 months. CONCLUSION PILSC is a multifaceted neoplasm in all its clinico-pathological aspects of presentation. Although rare, PILSC can involve adult and paediatric populations with a clear male preponderance. The MRI findings are not reliable to make a preliminary diagnosis. Like other lymphomas, the role of chemoradiotherapy is clearly more significant than surgical intervention in improving patient prognosis. Histopathologically, it can be Non-Hodgkin or Hodgkin, B-cell type or T-cell type and has a wide array of immunohistochemistry positivity.

Abstract BACKGROUND Pilomyxoid Astrocytoma (PMA), new variant of pilocytic astrocytoma, is more aggressive and relatively rare, mostly seen in children and commonly found in brain. Primary intramedullary pilomyxoid astrocytoma of spinal cord (PIPASC) is extremely rare. The distinct aggressive and pathological behavior, poor prognosis and the diversity in response to various treatment modalities makes the PIPASC an oncologic enigma. We aim to evaluate the clinical manifestations, histopathologic spectrum and onco-surgical management of this neoplasm. MATERIALS AND METHODS After an extensive literature search using PubMed Central and Google Scholar, eight case reports and one case series of patients with spinal PA were retrieved and included using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines without applying any filter of time, study type or place. Case presentations of 12 patients were analyzed in this review. RESULTS Pediatric preponderance of the neoplasm (75%) with the mean age of 7.90 ± 4.51 years was noted. Sensory deficits and scoliotic deformity were the most common presenting symptom and sign in 83.33% and 33.33% of patients, respectively. Thoracic spine (50%) was most commonly affected region. MRI showed the most consistent findings of cord compression in 100% cases and the lesion appeared hypointense on T1- weighted images (T1WI) and hyperintense on T2-weighted images (33.33%). The average post treatment survival duration was found to be more than 2 years. CONCLUSIONS Occurrence of PIPASC among pediatric population is rare. They have possibility of extraneural metastasis, can differentiate into more malignant glioma and get recurrent due to which they require a long-term follow-up of the patients. Further studies are required to investigate the utility of chemotherapy and radiotherapy in its management.

Background Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, enabling them to perform tasks. The advancements in AI have also improved virtual reality (VR), augmented reality (AR) and mixed reality (MR) experience allowing a greater opportunity for use in the field of medicine. Objective To evaluate the knowledge, attitude and practice of AI and types of realities among Pakistani healthcare professionals (HCPs). Materials and methods This was a prospective, nationwide study designed at the Department of Neurosurgery at Punjab Institute of Neurosciences (PINS), Lahore, was conducted between January 2024 to February 2024. More than 500 HCPs were approached, out of which 176 participated in this survey consensually. A pre -formed general questionnaire based on knowledge, attitude and practices of AI and types of realities was modified according to local conditions. Google Forms (Google Inc., USA) was used to conduct the one-time sign up response. Statistical Package for Social Sciences (IBM SPSS Statistics for Windows, Version 24, USA) was used to analyze submitted responses. Results About 69.9% respondents were male HCPs. Most of the respondents were from the fields of neurosurgery, medicine and general surgery, i.e., 10.80%, 10.20% and 4%, respectively. More than 90% HCPs used Internet and electronic devices daily. A majority of 62.50% respondents agreed that AI brings benefits for the patients, while at the same time, 45.50% agreed that they would not trust the assessment of AI more than that of HCPs. 61% HCPs feared that AI -based systems could be manipulated from the outside sources, like terrorists and hackers. Although 90% respondents knew the definition of AR and VR, a strikingly low 40% respondents could only identify the practical applications of these realities when asked in a mini -quiz. About 61.40% HCPs never used any AI -based application throughout their clinical practice, but Google Health was used by 29.50% respondents, followed by Remote Patient Monitoring AI application used by 3.4% individuals. Conclusion There is an evident under -utilization of AI and types of realities in clinical practice in Pakistan. Lack of awareness, paucity of resources and conventional clinical practices are the key reasons identified. Pakistan is on the path towards the point where the developed world is currently. There is a potential to move past the initial stages of AI implementation and into more advanced modes of adopting AI and types of realities.

Moyamoya disease (MMD) is considered a primary disorder of an unknown etiology. In contrast, Moyamoya syndrome (MMS) refers to MMD associated with other underlying diseases, such as meningitis in childhood, neurofibromatosis type II, Down syndrome, cranial irradiation, and different types of anemias, particularly hemoglobinopathies. We aimed to provide a comprehensive clinicopathological overview of MMS. All case reports and case series published from 2000 to 2023 pertaining to MMD were included in the study. Case studies, original articles, editorials, letters to editors, and clinical images were excluded. The search was conducted using the Boolean operators ("AND" and "OR") on PubMed and Google Scholar. A total of 13 case reports and one case series study were included. The study suggests infection might be a trigger in susceptible individuals. The autoimmune antibody findings (anti-double stranded DNA IgG) suggest a potential autoimmune component in some cases. There were diverse presentations and outcomes of post-infectious MMS, with a striking predominance of pediatric cases (66.66%) and a possible female predominance. Both computerized tomography (CT) and magnetic resonance imaging (MRI) showed evidence of restricted blood flow. CT showed that stenosis, occlusion, and collateral formation were frequent vascular findings, but often unspecified in severity. Infarction, hypodensities, and hematoma were the most common parenchymal findings (22.22% each). The findings on MRI were stenosis (50%) and collateral formation (44.44%). Infarction was the most common finding (66.66%) in parenchyma. Hydrocephalus, encephalomalacia, and atrophy were less frequent. Lesions were most frequent in the internal carotid artery (66.66%), middle cerebral artery (66.66%), and anterior cerebral artery (50%). Lesions were less frequent in the posterior cerebral, vertebral, and basilar arteries. The frontal lobe (38.89%) and basal ganglia (33.33%) were commonly affected parenchymal regions. The most common risk factor was human immunodeficiency virus (HIV) infection (50%), followed by trisomy 21, cryptococcal, and other types of meningitides. Aspirin (50%) and antiretroviral therapy (38.89%) were the cornerstones of treatment for MMS. This review accentuates the noteworthy obstacles presented by post-infectious MMS, especially its catastrophic effect on children and its correlation with HIV/AIDS. According to our elaborate literature search using PubMed and Google Scholar, this is the first narrative review in the existing scientific literature summarizing the literature on post-infectious MMS.

BACKGROUND:Brain tumors, traumatic head injury, and other intracranial processes including infections, can cause increased intracranial pressure and lead to overstimulation of the vagus nerve. As a result, increased secretion of gastric acid may occur which leads to gastro-duodenal ulcer formation known as Cushing's ulcer.METHODS:A review of original records of Dr. Harvey Cushing's patients suffering from gastro-duodenal ulcers was performed followed by a discussion of the available literature. We also reviewed the clinical records of the patients never reported by Cushing to gain his perspective in describing this phenomenon. Dr. Cushing was intrigued to investigate gastro-duodenal ulcers as he lost patients to acute gastrointestinal perforations following successful brain tumor operations. It is indeed ironic that Harvey Cushing developed a gastro-duodenal ulcer in his later years with failing health.RESULTS:Clinically shown by Cushing's Yale Registry, a tumor or lesion can disrupt this circuitry, leading to gastroduodenal ulceration. Cushing said that it was "reasonable to believe that the perforations following posterior fossa cerebellar operations were produced in like fashion by an irritative disturbance either of fiber tracts or vagal centers in the brain stem."CONCLUSION:Harvey Cushing's pioneering work depicted in his Yale registry serves as a milestone for continuing research that can further discern this pathway.

Background: Endovascular procedures are associated with injuries to the vasculature. Interventionists have adopted the use of intravenous anti-platelet agents. Eptifibatide drip has been utilized in advanced stroke treatment to increase recanalization in ischemic strokes. This study compares the outcome and mortality between those individuals that received Eptifibatide drip and those who did not. Materials: This is a retrospective analysis of all patients (n= 161) who presented to the hospitals with ischemic stroke and considered for advanced stroke therapy between January 2009 and July 2014. Statistical analysis was performed using GraphPad Prism. We divided the groups into two: (A) Eptifibatide drip (B) No Eptifibatide drip. We compared the effect of using Eptifibatide drip on the functional outcome using modified Rankin Scale (mRS). mRS score of 2 or less defines a good functional outcome and an mRS of 6 defines mortality. All variables were included in the analysis. Results: 161 patients included in this study had all the variables and received Endovascular treatment. Group analysis including n, gender, mean age, mean NIHSS, Afib%, DM% and Prior Stroke % was as follows: A) 66, 47%, 71.6±16.7, 16.0±7.1, 28.3%, 25% and 31.7% B) 95, 62.5% female, 74.5±16.3, 16.5±7.7, 44.2%, 30.5% and 23.2%. We found that patients who underwent endovascular procedures in group A experienced a better outcome and lower mortality as compared to those patients in group B (95% CI, 0.1761-0.3953, P<0.001); (95% CI,-0.4761- -0.2569, P<0.001). Conclusion: The use of Eptifibatide drip during endovascular procedures may result in a better functional outcome and lower mortality after the procedures as compared to other devices. We plan to further investigate the use of Eptifibatide drip in prospective study.