bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long-Term Outcomes of Paediatric-Onset Craniopharyngioma
Authors: Harsha Gunna Sri , Dabadghao Preeti , Sudhanshu Siddhnath 
Published: August 2023

Background:
Craniopharyngiomas are associated with long-term morbidity in the form of hormone deficiencies, visual deficits, and hypothalamic obesity.

Objective:
To study the long-term outcomes, including cure rates, endocrine dysfunction, visual dysfunction, hypothalamic obesity, and mortality in pediatric-onset craniopharyngiomas.

Methods:
A retrospective data analysis of pediatric (onset <18 years) craniopharyngioma diagnosed between 2003 and 2018. Data were collected from electronic hospital records, case files, and direct patient interviews.

Results:
The mean age at presentation was 10.4 ± 4.5 years (n = 62). The median duration of symptoms at diagnosis was 6 months (3–13 months). At presentation, central diabetes insipidus was present in four (6.5%), central hypothyroidism in 27 (43.5%), secondary adrenal insufficiency in 20 (32%) and delayed puberty in 15 (24%) patients. Hypothalamus was involved in 59/60 patients (98%). At last visit, 22.6% were obese in comparison to 4.6% at presentation, and anterior pituitary deficiency was present in 90% of the patients. Sixty-one percent patients (n = 62) had delayed puberty and 67% (n = 53) had short-stature. Out of 35 short children, nine (14%) children who received growth hormone had significant increase in height SD score (−3.8 (1.4) at start vs. −2.9 (1.2) at last follow-up; P = 0.008). Tumor progression was significantly less in the group that received RT compared to those who did not (8% vs. 39%; P = 0.002).

Conclusion:
Childhood-onset craniopharyngioma results in significant morbidity. The prevalence of pituitary hormones deficiency, visual deficits, and obesity are high at long-term follow-up. Incomplete tumor removal is also frequent. Thus, long-term monitoring is necessary for the timely management of the morbidities associated with craniopharyngioma.
Schizencephaly and the Neurodevelopmental Model of Psychosis
Authors: Patwal Rahul , Pai Naveen Manohar , Ganjekar Sundarnag , Arshad Faheem , Alladi Suvarna , Sharma Manoj Kumar , Desai Geetha , Chaturvedi Santosh K 
Published: August 2023

Background:
Schizencephaly is a rare congenital disorder of cerebral cortical development. Clinical features vary based on the areas and severity of cortical involvement. The most prominent manifestations being motor deficits, seizures, and cognitive impairment. MRI has been the diagnostic modality of choice. Till now, a handful of case reports associating schizencephaly with psychiatric manifestations have been reported.

Methods:
In this brief report, we review the available literature and present the summary of a 50-year-old gentleman who presented with paranoia, irritability, impulsive spending, emotional lability, and circumstantial speech with perseveration of themes.

Results:
MRI findings are suggestive of closed lip schizencephaly.

Conclusion:
This case helps us in understanding the neurodevelopmental model and functional neuroanatomy of psychiatric symptom dimensions.
Preliminary Study on the Effect of Impaired Glucose Tolerance on Blood-Brain Barrier Permeability in Non-Neurosyphilis Patients
Authors: Wang Feng , Che Xingyu , Yang Qingwei , Wang Ru , Zeng Jianqi , Chen Yiqian , Su Xinhui , Miao Jiayin 
Published: August 2023

Background:
Blood-brain barrier (BBB) is frequently disrupted in patients with diabetes mellitus (DM) and/or neurosyphilis (NS). Clinical cases reflect a trend that non-neurosyphilis (non-NS) patients with impaired glucose tolerance (IGT) are likely to develop NS and/or DM.

Objective:
To investigate whether IGT promotes BBB disruption in patients with non-NS.

Methods and Material:
A total of 21 subjects were enrolled, including six with IGT, nine with non-NS, and six with both IGT and non-NS. BBB permeability was evaluated by dynamic contrast-enhanced (DCE) MRI and the secretion of biomarkers from cerebrospinal fluid (CSF) were measured by colorimetric method, immune turbidimetric method, and enzyme-linked immunosorbent assay (ELISA) method.

Results:
The non-NS patients with IGT have higher BBB permeability at cortex superior frontal gyrus, white matter, and thalamus than non-NS patients without IGT or IGT patients without non-NS. The CSF-serum albumin-quotient (Qalb) levels and CSF secretion are highest in non-NS patients with IGT, including matrix metalloproteinase 9 (MMP9), soluble intercellular cell adhesion molecule-1 (sICAM-1), and soluble vascular cell adhesion molecule-1 (sVCAM-1).

Conclusions:
Significant correlations between CSF biomarkers and BBB permeability were found.
Unilateral Biportal Percutaneous Transforaminal Endoscopic Lumbar Foraminal Decompression and Discectomy
Authors: Gandham Edmond Jonathan , Uvaraj Nalli Ramanathan , Eum Jin Hwa 
Published: August 2023

Background and Introduction:
Minimally invasive spine surgery (MISS) is a set of techniques and procedures that reduces the local tissue injury while achieving the same results through traditional open surgery. The techniques for the treatment of lumbar disc herniation and degenerative canal stenosis have evolved from tubular discectomy to endoscopic discectomy. This helps in preserving spine function. Good clinical outcomes have been reported with the use of an endoscope for degenerative lumbar disc disease.

Objective:
In this video abstract, we present a case lumbar foraminal stenosis in an elderly man that was treated successfully using unilateral biportal endoscopic spine surgery. (UBESS).

Surgical Technique:
A 70-year-old man presented with progressive neurogenic claudication and left L4 numbness and paresthesias. His claudication distance was 200 m. His MRI Lumbosacral spine showed severe left L4-5 foraminal stenosis due to a disc prolapse. The patient underwent a left-sided unilateral biportal endoscopic transforaminal decompression and discectomy at L4-5 successfully. Postoperative period was uneventful and the patient had significant relief in his symptoms. Postoperative MRI Lumbosacral spine showed no muscle damage with adequate decompression at left L4-5 level.

Results:
The patient had an uneventful recovery.

Conclusion:
Biportal percutaneous transforaminal endoscopic discectomy/decompression is an ideal alternative to the uniportal endoscopic and minimal invasive microsurgery for foraminal and paracentral disc herniations and lateral recess and foraminal stenosis.
Genetic Polymorphisms in DRD4 and Risk for Parkinson's Disease Among Eastern Indians
Authors: Dipanwita Sadhukhan , Arindam Biswas , Atanu Biswas , Kunal Ray , Jharna Ray 
Published: August 2023

Background:
Genetic factors, including causal gene and modifier genes, contribute significantly in PD pathogenesis in an ethnicity-dependent manner. Dopamine Receptor 4 (DRD4), involved in dopamine metabolism is one such modifier locus for PD.

Objective:
To identify the potential association of DRD4 polymorphic variants with PD among Eastern Indians.

Methods and Materials:
PD-related DRD4 variants were genotyped among 291 PD patients and 265 ethnically matched controls from Eastern India.

Results and Conclusion:
Among the three DRD4 variants, only the 120 bp duplicated allele [P = 0.036; Odds ratio: 1.323; 95% CI: 1.014–1.725] and its homozygous genotype [P = 0.034; Odds ratio: 1.452; 95% CI: 1.025–2.057] were found as risk factors for overall PD and sporadic PD among Eastern Indians. However, no other disease-associated variant or haplotype was identified. Therefore, in conclusion, our study demonstrates that DRD4 plays a small role in PD pathogenesis among Eastern Indians.
Protective Effect of L-Arginine in an Animal Model of Alzheimer's Disease Induced by Intra-Hippocampal Injection of AlCl3
Authors: Karami Manizheh , Geravand Samira , Rahimpour Mahnaz 
Published: August 2023

Background and Objective:
Aluminum chloride (AlCl3) can impair spatial memory recovery. We investigated the protective effect of L-arginine, a precursor of nitric oxide (NO), on memory retrieval in an Alzheimer's animal model induced by AlCl3 at intra-hippocampal CA1 using a seeking behavior practice.

Materials and Methods:
Wistar rats were deeply anesthetized and cannulated at CA1 (AP: -3.8 mm, L: ±2.2 mm, V: 3 mm), and received once AlCl3 (1–200 μg/rat, intra-CA1), on day of cannulation under stereotaxic device. After a week of recovery, they experienced the novelty task with a three-stage paradigm and injected L-arginine (0.05–25 μg/rat) intra-CA1, pretesting. L-NAME, the neuronal NO synthase inhibitor was administered before L-arginine effective doses in the test stage. Also, a reference group exclusively received beta-amyloid 2 μg/rat. Control group solely received saline. Finally, after euthanasia of rat, the hippocampal sample was collected on ice and evaluated by immunohistochemical marking and specific staining.

Results:
AlCl3 caused novelty-seeking behavior without meaningful change in animal locomotor activity. βA (2 μg/rat, intra-CA1) affected the rat's grooming, causing it to stop further in the new side. Pretest injection of L-arginine restored behavior in AlCl3-treated rats; however, this effect was stopped by L-NAME pretreatment, indicating NO involvement. CA1 did not show necrotic change due to AlCl3 exposure; however, neurofibrillary tangles were accumulated in the region.

Conclusions:
Prophylaxis with L-arginine probably due to NO has a protective role against the dangerous effect of AlCl3 on the function of neurons in the cortical hippocampus.
Reconsidering the Relationship Between Hand Preference and Cerebral Vascular Dominance
Authors: Bülbül Nazlı Gamze , Demir Serkan , Özkan Suat , Samancı Cesur , Atmaca Murat Mert , Çelik Didem , Köse Ercan , Alparslan Funda , Altıner Ayşenur , Arı Selma Akkaya , Doğan Ceyda , Yavuz Cansu Gulcihan , Arslan Zeynep Meltem , Akşahin Okan , Özdağ Mehmet Fatih 
Published: August 2023

Background:
To date, it has been suggested that there may be many genetic, environmental, and vascular factors that affect hand preference. In previous studies evaluating the relationship between cerebral dominance and hand preference, carotid and vertebral artery (VA) Doppler ultrasonography (USG) was generally preferred; and these studies only measured VA diameters. Unlike other studies, we aimed to reevaluate the relationship between hand preference and cerebral vascular dominance by measuring VA and internal carotid artery (ICA) diameters. In addition, we used carotid and VA computed tomography (CT) angiography instead of Doppler USG.

Methods and Material:
A total of 345 participants were included in the study. The results of carotid and VA CT angiography taken during hospitalization were retrospectively evaluated by two radiologists, and the Edinburgh Hand Preference Questionnaire was applied to these patients.

Results:
In right-handed patients, the diameter of the left VA was significantly larger than the diameter of the right VA (p = 0.005). In left-handed patients, the diameter of the left ICA was larger than the diameter of the right ICA, but the difference was not statistically significant (p = 0.055). There was no significant difference between the diameter of the right and left ICA in right-handed patients (p = 0.771).

Conclusions:
In our study, we found a correlation between the dominant hemisphere VA diameter and hand preference. Using CT angiography, we were able to eliminate many challenges of ultrasonography that make radiological evaluation difficult, such as differences of opinion between radiologists, and technical and anatomical reasons.
Therapeutic Plasma Exchange in Neuromyelitis Optica Spectrum Disorders—Experience from Tertiary Care Center in North India
Authors: Hans Rekha , Prakash Satya , Sharma Ratti R , Sankhyan Naveen , Takkar Aastha , Goyal Manoj , Saikia Biman , Lal Vivek , Marwaha Neelam 
Published: August 2023

Background:
Therapeutic plasma exchange (TPE) is indicated in the acute presentation of neuromyelitis optica spectrum disorders (NMOSD) as category II indication as per American Society for Apheresis guidelines 2016.

Aim:
To assess the efficacy of TPE in symptomatic patients of NMOSD not responding to high dose intravenous steroids.

Patients and Methods:
We retrospectively reviewed the record of TPE procedures in NMOSD patients over a period of 3 years (2013–2016). TPEs were done on Cobe® spectra (Terumo BCT, Lakewood Co., USA) using 5% human serum albumin or fresh frozen plasma as replacement fluid. Clinical improvement was recorded post-procedure and adverse reactions if any was noted.

Results:
We performed TPE in 11 clinically diagnosed patients of NMOSD. Three (27%) patients had only visual symptoms, five (46%) had both visual as well as muscular symptoms, whereas three (27%) patients presented with only muscular symptoms. Out of seven tested, three patients were aquaporin-4-immunoglobulin-G-positive (AQP4-IgG positive). Visual symptoms improved from no vision pre-TPE therapy to finger counting or 6/24 post-therapy. The muscle power of the limbs improved by grade one to grade two post-therapy. Adverse events were observed in 8% (5/62) of the procedures; allergic reactions to replacement fluid as the most common event (n = 3) followed by hypotension (n = 2). Follow-up was available in 81% (9/11) patients. Two patients died at 3 and 12 months of follow-up. One patient had relapsed and underwent a second TPE cycle.

Conclusion:
The addition of TPE as a part of therapy is effective and safe in acute exacerbations of NMOSD.
Preoperative Third Ventricle Floor Bowing is Associated with Increased Surgical Success Rate in Patients Undergoing Endoscopic Third Ventriculostomy – A Systematic Review and Meta-analysis
Authors: Pranata Raymond , Yonas Emir , Vania Rachel , Rachmadian Cut Vanessa , July Julius 
Published: August 2023

Background:
Endoscopic third ventriculostomy (ETV) is a procedure that involves devising an opening in the third ventricle floor, allowing cerebrospinal fluid to flow into the prepontine cistern and the subarachnoid space. Third ventricular floor bowing (TVFB) serves as an indicator of intraventricular obstruction in hydrocephalus and existence of pressure gradient across third ventricular floor, which is the prerequisite of a successful ETV.

Objective:
In this systematic review and meta-analysis, we aimed to synthesize the latest evidence on the TVFB as a marker for surgical success in patients undergoing ETV.

Material and Methods:
We performed a comprehensive search on topics that assesses the association of TVFB with the surgical success in patients undergoing ETV from several electronic databases.

Results:
There was a total of 568 subjects from six studies. TVFB was associated with 85% (81-88%) ETV success. TVFB was associated with OR 4.13 [2.59, 6.60], P < 0.001; I2: 6% for ETV success. Subgroup analysis on pediatric patients showed 86% (82-91%) success rate. In terms of value for ETV success compared to ETV Success Score (ETVSS), a high ETVSS does not significantly differ (P = 0.31) from TVFB and TVFB was associated with OR 3.14 [1.72, 5.73], P < 0.001; I2: 69% compared to intermediate/moderate ETVSS. Funnel plot analysis showed an asymmetrical funnel plot due to the presence of an outlier. Upon sensitivity analysis by removing the outlier, the OR was 3.62 [2.22, 5.89], P < 0.001; I2: 0% for successful surgery in TVFB.

Conclusions:
TVFB was associated with an increased rate of successful surgery in adults and children undergoing ETV.
Perioperative Management of Pediatric Brain Tumors
Authors: Mishra Nitasha , Rath Girija P. , Rajagopalan Vanitha , Doddamani Ramesh , Chaturvedi Arvind 
Published: August 2023

Background:
Brain tumors are the second most common malignancy in childhood and the surgical excision remains the cornerstone of management. The objective of this study was to analyze the factors associated with the length of intensive care unit (ICU) and hospital stay, and postoperative outcome in such children.

Materials and Methods:
Three years of data were collected, retrospectively, by detailed review of medical records pertaining to pre-anesthetic evaluation and perioperative course of children less than 16 years of age who underwent excision of intracranial tumors.

Results:
One hundred sixty-eight medical records were analyzed. One third of the children were found to have developed various intraoperative adverse events; the most common were hemodynamic changes following brainstem handling and brain swelling. 58% of children required postoperative mechanical ventilation. 82.7% of patients had favorable neurologic outcome which was comparable between the two tumor locations (supratentorial vs infratentorial). On multivariate analysis, re-exploration surgery and electrolyte disturbances, such as serum sodium, were found to be the independent risk factors affecting hospital stay. The amount of intraoperative blood loss and postoperative pulmonary complications (POPCs) were independent risk factors affecting the neurologic outcome.

Conclusions:
Adverse events are fairly common after excision of brain tumors in children. Intraoperative complications did not affect the ICU stay or neurological outcome; however, the postoperative complications increased length of ICU and hospital stays. POPC was the single most important factor responsible for poor neurologic outcome and was more so in children who underwent infratentorial surgery, prolonged mechanical ventilation, and who had a lower cranial nerve palsy.
OFF-Period Purposeless Groaning in Parkinsonism
Authors: Agarwal Vikash , Rajan Jeyalakshmi , Ramli Norlisah , Santiago Cecilia , Jasti Dushyanth B , Tan Ai Huey , Lim Shen-Yang 
Published: August 2023

Background:
Purposeless groaning is primarily encountered in patients with progressive supranuclear palsy and has also been reported to occur in advanced Parkinson's disease (PD).

Objective:
We describe a case of pronounced purposeless groaning occurring as a medication OFF-period nonmotor phenomenon in PD. To our knowledge, this has not been previously reported in the literature.

Methods and Materials:
We describe and provide video documentation of a patient with moderately advanced PD and motor fluctuations, in whom OFF-period groaning was reported by the family and observed during clinic consultations to be a prominent feature, occupying approximately 40% of his OFF periods as calculated from his PD diary.

Results and Conclusions:
Although rare, OFF-period purposeless groaning in PD can be very disruptive and add significantly to caregiver burden. It is postulated to be a disinhibitory and perseverative behavior related to overactivation of the cingulo-periaqueductal circuit; further study is needed to delineate the underlying pathophysiological mechanisms.

Conclusions:
Although rare, OFF-period purposeless groaning in PD can be very disruptive and add significantly to caregiver burden. It is postulated to be a disinhibitory and perseverative behavior related to overactivation of the cingulo-periaqueductal circuit; further study is needed to delineate the underlying pathophysiological mechanisms.
Translation and Validation of Penn Acoustic Neuroma Quality of Life Scale for Hindi-Speaking Population
Authors: Mohanty Manju , Kaur Rupinder , Gupta Sunil Kumar , Tripathi Manjul , Sharma Akhilesh , Munjal Sanjay 
Published: August 2023

Background:
The Penn Acoustic Neuroma Quality-of-life Scale (PANQOL) was specifically developed for assessing the quality of life (QOL) in patients with vestibular schwannoma (VS). Its utility has been established in many populations, but it has not been validated for use in India.

Objective:
The objective of this study was to translate PANQOL into Hindi and validate it for use in India.

Materials and Methods:
PANQOL was translated into Hindi by using the standardized procedure. Its conceptual equivalence was established by administering. For validation, PANQOL-Hindi and SF-36 were administered to 78 patients aged above 18 years and recently diagnosed to have VS. Test–retest reliability was established by readministering PANQOL-Hindi on 15 patients after 2 weeks.

Results:
Internal consistency was good for the composite scale (α = 0.87). The Cronbach's alpha for all domains except facial dysfunction was in an acceptable range. Intercorrelations between various domains showed that anxiety and energy were strongly correlated. All domains except pain had a strong correlation with the total PANQOL score. The intraclass correlation coefficient test indicated high test–retest reliability for the composite scale (ICC = 0.97; 95% CI, 0.93–0.99). Test–retest reliability for various domains was also good. The construct validity of PANQOL was assessed by correlating its specific domains with SF-36 domains. The meaningful correlations between domains of PANQOL and SF-36 indicated good construct validity.

Conclusions:
PANQOL-Hindi has been adequately translated and has satisfactory psychometric properties.
Is There a Role for Temozolomide in Glioma Related Seizures? A Systematic Review
Authors: Yue Jiong , Yin Cheng , Chen Longyi , Xu Ruxiang , Zhao Dongdong 
Published: August 2023

Background:
Seizures often herald the clinical appearance of glioma. Temozolomide (TMZ) is the first-line chemotherapeutic agent that has been used to treat glioma.

Objective:
We conducted a systematic review to determine seizure outcomes in glioma patients treated with TMZ.

Methods and Material:
We searched EMBASE and PubMed databases (January 1, 2003–August 26, 2021) by using search terms closely related to glioma, seizure, and temozolomide. Titles, abstracts, and full texts were screened and selected using previously established inclusion and exclusion criteria. The research team members reviewed potential articles and reached a consensus on the final articles to be included.

Results:
Nine studies containing data from three continents met our inclusion criteria. From several descriptive studies on low-grade gliomas (LGGs), the percentage of patients with partial seizure control after TMZ treatment ranged from 29% to 89.7%, and the percentage of patients with complete seizure control after TMZ ranged from 19.4% to 72%. In a retrospective cohort study of patients with LGGs, there was a marked difference in decreased seizure frequency between patients receiving TMZ and those who did not receive TMZ. In a randomized trial, TMZ seemed to have little effect on seizure control in elderly patients with glioblastoma.

Conclusions:
At present, there are few high-quality and well-designed clinical studies on TMZ for gliomas-related seizures. In terms of the literature included in this review, TMZ has an inhibitory effect on epilepsy. More randomized controlled trials are needed to elucidate the clinical benefits of TMZ in the treatment of gliomas-related seizures.
Stereotactic Radiosurgery with Gamma Knife in Recurrent Rathke's Cleft Cyst
Authors: Manzoni Andrea , Leocata Filippo , Picano Marco , Arienti Virginia Maria , Mainardi Hae Song , Cenzato Marco , Stefini Roberto , LA CAMERA Alessandro 
Published: August 2023

Background:
Rathke's cleft cyst is a benign expansive lesion of the sella turcica. If related to clinical disorders, the patient needs surgical treatment.

Objective:
To demonstrate the efficacy of radiosurgery in the treatment of relapse of Rathke's cleft cyst as an alternative to surgery.

Methods and Material:
The stereotactic radiosurgical treatment was performed at the Gamma Knife Center of the Niguarda Hospital in a patient with Rathke's cleft cyst subjected to two subsequent neurosurgical resections with early regrowth of the cyst. The cyst underwent radiosurgery with a prescription dose of 12 Gy at 50% (minimum dose 9.8, mean 17.3 and maximum 24.4).

Results:
Three years after stereotactic radiosurgical treatment the patient is asymptomatic and does not present disorders of the hypothalamic-pituitary axis or further visual alterations. The control MRI shows a reduction of the cyst's volume.

Conclusions:
Stereotactic radiosurgery resulted in a reduction of the cyst's volume and avoided further recourse to surgery.