bluuebunny/crossref_metadata_2025_split huggingface dataset:
Subarachnoid Hemorrhage and Hydrocephalus
Authors: Bhattacharjee Suchanda , Rakesh Das , Ramnadha Reddy , Manas Panigrahi 
Published: December 2021

Abstract

Background:
Hydrocephalus associated with subarachnoid hemorrhage is a common neurosurgical problem, the management of which is tailor-made to the patient. It is usually seen with an aneurysmal bleed and is independent of the primary modality of treatment.

Aim:
This study aimed to provide a comprehensive overview of this important association and discuss the various available treatment modalities.

Materials and Methods:
A detailed review of the literature was done on the risk factors, pathogenesis, and treatment of hydrocephalus in the setting of subarachnoid hemorrhage.

Results:
Hydrocephalus occurs in 6% to 67% of subarachnoid hemorrhage (SAH). It may present as acute, subacute, or chronic at the time of presentation. Diagnosis is made with a plain computed tomography scan of the brain, and the treatment is observant, temporary, or permanent cerebrospinal fluid diversion.

Conclusion:
Hydrocephalus associated with SAH interferes with the outcome of SAH. It prolongs the hospital stay, besides causing additional morbidity. The various risk factors, if present, should warn us to be vigilant, and management is definitely not uniform and is custom made to the patients' needs.
Comparison of Programmable and Non-Programmable Shunts for Normal Pressure Hydrocephalus
Authors: Katiyar Varidh , Sharma Ravi , Tandon Vivek , Garg Kanwaljeet , Narwal Priya , Chandra P Sarat , Suri Ashish , Kale Shashank S 
Published: December 2021

Abstract

Background:
The use of programmable ventriculoperitoneal shunt (P-VPS) in idiopathic normal pressure hydrocephalus (iNPH) has increased over the last two decades, however, there is no definitive evidence to favor them over non-programmable VPS (NP-VPS). Thus, there is a growing need for studies comparing these two procedures for their efficacy and safety profile in iNPH.

Objective:
In this study, we attempt to quantitatively summarize the findings of all the prospective and retrospective studies that have directly compared the P-VPS and NP-VPS in terms of efficacy, complications, or overall healthcare expenditure.

Methods:
A systematic search was performed of PubMed, the Cochrane Library databases, and Google Scholar for studies till June 2021 comparing the outcomes of P-VPS with NP-VPS. Four studies were finally included in the quantitative analysis. A trial sequential analysis was done to evaluate the need for further studies.

Results:
The total rates of subdural collection (odds ratio (OR) 1.03; 95% Confidence interval (CI): 0.73–1.46; P = 0.85; I
2 = 12%) as well as surgically evacuated subdural collection (OR 0.46; 95% CI: 0.14–1.55; P = 0.21; I
2 = 75%) were not significantly different for P-VPS compared to NP-VPS with pooled data. Similarly, the rate of postoperative infection was found to be similar between the two types of VPS (OR 0.98; 95% CI: 0.39–2.5; P = 0.97; I
2 = 0%). The trial sequential analysis (TSA) for the need of surgical evacuation of subdural collection and shunt revision revealed that the meta-analysis of the currently accrued information is not conclusive.

Conclusions:
Though, associated with higher initial costs, P-VPS does not seem to result in increased healthcare costs in the long run while enabling the surgeon to titrate the opening pressure and avoiding additional surgical procedures like shunt revision or evacuation of subdural collection at least theoretically. However, further trials with a greater sample size are needed to confirm these findings as the current accrued information size is insufficient to reach an unequivocal verdict.
Malignant Meningitis Associated with Hydrocephalus
Authors: Kumar Ashutosh , Sardhara Jayesh C , Singh Guramritpal , Kanjilal Soumen , Maurya Ved P , Behari Sanjay 
Published: December 2021

Abstract
Malignant meningitis (MM) is the diffuse involvement of the leptomeninges by infiltrating cancer cells, most frequently from lung and breast cancers. This review is aimed to discuss the current advances in the diagnosis and management of MM, along with management of MM-associated hydrocephalus. We reviewed the literature using PubMed and Google Scholar search engines, focusing on various recent randomized controlled trials and clinical trials on MM. Given the hallmark multifocal involvement, the clinical symptoms and signs are also random and asymmetric. There are three important pillars for establishing a diagnosis of MM: clinical examination, neuroimaging, and CSF cytological findings. Several factors should be considered in decision-making, including performance status, neurological findings (clinical, MRI, and CSF flow dynamic), and evaluation of the primary tumor (nature and systemic dissemination). Response Assessment in Neuro-Oncology (RANO) working group recommended the objective assessment of disease for evaluating the progression and response to therapy. Pillars of current management are mainly focal irradiation and intrathecal or systemic chemotherapy. Symptomatic hydrocephalus is managed with a ventriculoperitoneal shunt, lumboperitoneal shunt, or endoscopic third ventriculostomy as palliative procedures, providing significant improvement in performance scores in the limited survival time of patients with MM. Studies using novel therapeutic approaches, such as new biological or cytotoxic compounds, are ongoing. Despite the use of all the combinations, the overall prognosis remains grim; therefore, decision-making for treatment should predominantly be based on attaining an optimal quality of life.
Natural History, Treatment Outcomes and Quality of Life in Idiopathic Normal Pressure Hydrocephalus (iNPH)
Authors: Isaacs Albert M , Hamilton Mark 
Published: December 2021

Abstract

Background:
The natural history and treatment outcomes in adult patients with hydrocephalus is a broad and heterogeneous topic that encompasses the natural history of the various subtypes of adult hydrocephalus with or without treatment; their surgical operative results, including symptom improvements, treatment failure, short- and long-term complications, and reoperations; and morbidity, mortality, and patient-centered health-related quality of life (HRQoL).

Objective, Methods, and Materials:
The objective of this review is to present a current update on the natural history and treatment outcomes, including QoL, for adults with hydrocephalus with a focus on patients with idiopathic normal pressure hydrocephalus (iNPH). A nonsystematic review of relevant literature was summarized.

Results and Conclusions:
The natural history for untreated patients with iNPH is poor, with both increased mortality and morbidity. It is strongly recommended that practitioners follow established guidelines to select patients with suspected iNPH while using objective measures of gait, balance, and cognition for consideration of treatment with a CSF shunt. Other factors such as patient-related medical comorbidities or frailty may need to be factored into the decision-making process before surgical treatment is offered. As a rule, failure to select patients based on the identified guidelines will result in a significantly lower positive response to treatment with a CSF shunt. Over 90% of iNPH patients who undergo CSF-shunt treatment demonstrate symptomatic relief after surgery, and long-term studies have shown that in most patients, the clinical improvements are long-lasting, with over 70% demonstrating improvement longer than 6 years after treatment. There is no evidence to support the routine use of endoscopic third ventriculostomy (ETV) to treat patients with iNPH. There is limited data regarding HRQoL in patients with iNPH. In addition to objective measures of outcomes focused on gait and cognition, it is equally important for future studies to assess patient-centered subjective measures of HRQoL.
Hydrocephalus in Low and Middle-Income Countries - Progress and Challenges
Authors: Enslin Johannes M N , Thango Nqobile S , Figaji Anthony , Fieggen Graham A 
Published: December 2021

Abstract
Hydrocephalus remains one of the most commonly treated neurosurgical conditions worldwide. Caring for patients with hydrocephalus requires infrastructure and political support and initiative; these are often difficult to obtain in low- and middle-income countries (LMICs). Some innovations that have arisen in LMICs have traveled up the financial gradient to high-income countries, such as the combination of endoscopic third ventriculostomy with choroid plexus coagulation to manage hydrocephalus. The development of neuro-endoscopy has played a major role in managing hydrocephalus worldwide; however, LMICs still face specific challenges, such as limited access to shunt hardware, a disproportionately high incidence of post-infectious hydrocephalus, unique microbiological spectra, and often poor access to follow-up care and neuroimaging. This has received increased attention since the Lancet Commission on Global Surgery. The goal of improving access to quality neurosurgical care through various initiatives in LMICs will be discussed in this manuscript. The need for neurosurgeons continues to grow in LMICs, where better access to neurosurgical care, adequate neurosurgical training and political support, and patient education are needed to improve the quality of life for patients with common neurosurgical conditions. Despite these challenges, treating hydrocephalus remains a worthwhile endeavor for many patients.
Fetal Ventriculomegaly and Hydrocephalus – What Shouldn't be Missed on Imaging?
Authors: Sira Liat B , Kozyrev Danil A , Bashat Dafna B , Constantini Shlomi , Roth Jonathan , Shiran Shelly I 
Published: December 2021

Abstract
Fetal ventriculomegaly is one of the most frequently diagnosed abnormalities detected prenatally. The finding of additional subtle abnormalities can facilitate accurate prognoses, which may range from normal outcomes to significant neurodevelopmental sequelae. Pathogenesis and imaging patterns of ventriculomegaly and hydrocephalus in the fetus based on the pattern-recognition approach using fetal MRI are reviewed in this paper. This radiological approach may shed light on clinical course prediction and therapeutic efficacy of hydrocephalus in the fetus.
Pre- and Post-stenting Cerebral Blood Flow Velocities in Patients with Carotid Artery Stenosis
Authors: Akkaya Efdal , Nazliel Bijen , Caglayan Batur Hale Z , Ilgit Erhan , Onal Baran , Akkan Koray , Irkec Ceyla 
Published: December 2021

Abstract

Purpose:
This study evaluated the hemodynamic effects of carotid artery stenting (CAS) on cerebral blood flow velocity (CBFV) in patients with carotid artery stenosis, before, 3 d, and 3 months after the procedure using transcranial Doppler ultrasound (TCD).

Methods:
The study included 36 patients with atheromatous carotid artery stenosis. Cerebral computed tomography (CT) or magnetic resonance imaging (MRI) was performed in every patient, and carotid stenosis was evaluated using duplex sonography, CT, and MRI angiography before the procedure. To obtain baseline values, the CBFV was evaluated 1 d before CAS. Follow-up TCD evaluations were performed 3 d and 3 months postoperatively.

Results:
The median degree of internal carotid artery (ICA) stenosis in the participants was 90% (range 50%–99%). The median CBFV at the anterior cerebral artery (ACA) was significantly lower on the ipsilateral side than on the contralateral side before stenting; however, there were no significant differences in CBFV in the ipsi and contralateral middle cerebral artery (MCA). The median CBFV in the ipsilateral MCA increased significantly 3 d after the procedure and remained higher than the basal values after 3 months.

Conclusions:
We observed significant increases in the median CBFV and pulsatility index (PI) in the MCA bilaterally, especially on the stented side, measured 3 d and 3 months after stenting in patients with severe ICA stenosis.
Cryptogenic Stroke in the Young
Authors: Salomi Bodda S B , Solomon Raja , Turaka Vijay Prakash , Aaron Sanjith , Christudass Christhunesa S 
Published: December 2021

Abstract

Context:
Strokes that remain without a definite cause even after an extensive workup, termed cryptogenic strokes, constitute up to 30–40% of ischemic strokes (ISs) in the young. Some of them can have a genetic basis. However, the well-established genetic causes account for only a small percentage of these cases.

Aim:
To evaluate the association of cryptogenic young IS with 16 candidate gene polymorphisms.

Settings and Design:
A case-control study with cryptogenic young IS patients (South and North Indians; n = 105) and age, sex, and ethnicity-matched controls (n = 215).

Subjects and Methods:
Genotyping was carried out by PCR-RFLP method using DNA extracted from the blood.

Statistical Analysis Used:
Association of the genotypes with the disease was studied using Chi-square test.

Results:
MTHFR rs1801133 and KNG1 rs710446 showed significant statistical association with cryptogenic young IS (P = 0.0261 and 0.0157, respectively) in the Indian population. Significant association of KNG1 rs710446 (P 0.0036) and FXII rs1801020 (P 0.0376) with cryptogenic young stroke in South Indian males, SERPINC1 rs2227589 in South Indian female patients (P = 0.0374), and CYP4V2 rs13146272 in North Indian males (P = 0.0293) was observed.

Conclusions:
Our study indicates that in the Indian population MTHFR rs1801133, KNG rs710446, FXII rs1801020, SERPINC1 rs2227589, CYP4V2 rs13146272, and FXIII V34L may be significant risk factors for cryptogenic IS in the young. In addition, ethnicity and gender play a significant role. Further studies with larger sample size are required to completely establish these polymorphisms as risk factors for cryptogenic IS in young Indians.
A case of adult-onset ophthalmoplegic migraine
Authors: Alam Anwar , Iqubal Md Shahid , Kumar Baidyanath , Azad Zeyaur R 
Published: December 2021

Abstract
Ophthalmoplegic migraine (OM) also called recurrent painful ophthalmoplegic neuropathy (RPON) is not a so common disorder. It is characterized by childhood onset, ophthalmoplegia and migraine type of headache. The most common involved nerve is third cranial nerve. Involvement of fourth and sixth cranial nerve is unlikely. Adult cases are not so common. This is a case report of a man who presented with left-sided severe headache and diplopia of left eye. He had left oculomotor nerve palsy. The patient responded to treatment and recovered.
An Atypical Presentation of Left Lateral Medullary Syndrome – A Case Report
Authors: Renjen Pushpendra N , Krishnan Ramesh , Chaudhari Dinesh , Ahmad Kamal 
Published: December 2021

Abstract
Lateral medullary syndrome (LMS), known as Wallenberg's syndrome and posterior inferior cerebellar artery syndrome, is a rare cause of stroke. It often results from thrombosis or emboli of the vertebral artery or posterior inferior cerebellar artery. The triad of Horner's syndrome, ipsilateral ataxia, and ipsilateral hyperalgesia clinically identify patients with LMS. We report a case of a 62-year-old diabetic, hypertensive male who presented with symptoms involving the left lateral dorsal medulla along with partial Horner's syndrome, left lateral rectus palsy, and left lower motor neuron–type facial palsy. Atypical presentation in LMS could be explained by infraction of left facial colliculus in addition to the left lateral medulla. Association of these entities with Wallenberg's syndrome is atypical features in our case, which questioned the diagnosis of a simple LMS.
COVID-19 in Patients with Myasthenia Gravis
Authors: Murthy Jagarlapudi M.K. , Gutta Abhinay K. , Yerasu Muralidhar Reddy , Boorgu Santhosh Kumar , Osman Syed , Jaiswal Shyam K. , Pidaparthi Lalitha , Gudavalli Bhavani P. 
Published: December 2021

Abstract
This report describes a patient with thymomatous myasthenia gravis (MG) with aplastic anemia in pharmacological remission and COVID-19 who developed respiratory failure in the course of the disease and reviews the published literature on this topic. Analysis of the clinical characteristics of the eight patients with MG including our patient suggests two possible mechanisms for respiratory failure: myasthenic crisis (MC) or pulmonary complications of COVID-19. Patients with MC were young women in high-grade MGFA Class whereas patients with respiratory failure due to pulmonary complications of COVID-19 were elderly men in pharmacological remission or MGFA Class I. These observations suggest that COVID-19, like other infections, may precipitate MC in patients with severe grade MG before COVID-19. The only differentiating feature between the two types of failure was severity myasthenic weakness. This clinical distinction has management implications. These observations need to be validated in a larger sample.
Traumatic Pseudoaneurysm of Middle Meningeal Artery with Delayed Presentation as Intracerebral Hematoma
Authors: Kulanthaivelu Karthik , Siddiqui Shahyan M , Prasad Chandrajit , Shashidhar Abhinith 
Published: December 2021

Abstract

Background:
Post-traumatic pseudoaneurysm of the middle meningeal artery is a rare entity. We report an atypical case of a delayed presentation as parenchymal hemorrhage due to a ruptured middle meningeal artery pseudoaneurysm.

Case Description:
A 22-year-old man with an alleged history of cranial trauma following a road traffic accident presented 10 days later with a new right temporal intraparenchymal hemorrhage. The CT revealed a differentially hypodense circumscribed structure in the anterior temporal location eccentrically in the hematoma. The cerebral angiogram depicted a pseudoaneurysm arising from the middle meningeal artery. The patient underwent craniotomy and excision of the aneurysm. On follow up, the patient was asymptomatic and had no focal neurological deficits.

Conclusion:
Despite its rare occurrence, meningeal artery pseudoaneurysm should be considered as a possible etiology of a post-traumatic delayed presentation as an intracerebral hematoma. Prompt diagnosis and management are warranted in view of the mortality and morbidity.
Assessing Utility of Clinical Exome Sequencing in Diagnosis of Rare Idiopathic Neurodevelopmental Disorders in Indian Population
Authors: Sheth Harsh , Pancholi Dhairya , Bhavsar Riddhi , Mannan Ashraf U , Ganapathy Aparna , Chowdhury Mayank , Shah Sudhir , Solanki Dhawal , Sheth Frenny , Sheth Jayesh 
Published: December 2021

Abstract

Background:
Neurological diseases are phenotypically and genotypically heterogeneous. Clinical exome sequencing (CES) has been shown to provide a high diagnostic yield for these disorders in the European population but remains to be demonstrated for the Indian population.

Objective:
The study aimed to understand the utility of clinical exome sequencing for the diagnosis of neurodevelopmental disorders.

Materials and Methods:
A cohort of 19 idiopathic patients with neurological phenotypes, primarily intellectual disability and developmental delay, were recruited. CES covering 4620 genes was performed on all patients. Candidate variants were validated by Sanger sequencing.

Results:
CES in 19 patients provided identified 21 variants across 16 genes which have been associated with different neurological disorders. Fifteen variants were reported previously and 6 variants were novel to our study. Eleven patients were diagnosed with autosomal dominant de novo variants, 7 with autosomal recessive and 1 with X-linked recessive variants. CES provided definitive diagnosis to 10 patients; hence, the diagnostic yield was 53%.

Conclusion:
Our study suggests that the diagnostic yield of CES in the Indian population is comparable to that reported in the European population. CES together with deep phenotyping could be a cost-effective way of diagnosing rare neurological disorders in the Indian population.
A Rare Cause of Globus Pallidus and Dentate Nucleus Hyperintensity in Childhood
Authors: Ozpinar Esra , Kaytan Ismail , Topcu Yasemin , Kılıc Betul , Aydin Kursad 
Published: December 2021

Abstract
Mutations in mammalian membrane-bound O-acyltransferase domain-containing (MBOAT) 7 gene are a rare cause for intellectual disability, developmental delay, autistic findings, epilepsy, truncal hypotonia with appendicular hypertonia, and below-average head sizes. Pathogenic variants in MBOAT7 gene show these nonspecific clinical features that are seen in many other neurometabolic diseases. Therefore, specific neuroimaging findings can be valuable key factors for differential diagnosis. Magnetic resonance imaging (MRI) findings of T2 hyperintensity in bilateral globus pallidi and dentate nuclei are seen in a few neurometabolic diseases with similar clinical features of developmental delay and hypotonia, as in our cases. While evaluating the patients with similar phenotypes and specific MRI findings, MBOAT7 deficiency should be kept in mind. Here, we identified two brothers who had a novel homozygous variant in MBOAT7 gene and aimed to raise awareness about this newly described disease.
Statins Therapy Improves Acute Ischemic Stroke in Patients with Cardio-metabolic Disorders Measured by Lipoprotein-Associated Phospholipase A2 (Lp-PLA2)
Authors: Al-kuraishy Hayder M , Hussien Nawar R , Al-naimi Marwa S , Al-Gareeb Ali I , Lugnier Claire 
Published: December 2021

Abstract

Background:
Acute ischemic stroke (AIS) leads to neuronal and endothelial damage that activate the release of proinflammatory mediators such as lipoprotein-associated phospholipase A2 (Lp-PLA2), which lead to the development of brain edema injury. Most of statins produce differential effects on Lp-PLA2 activity and mass with a comparable reduction in low-density lipoprotein (LDL) serum levels.

Aims:
The aim of this study is to evaluate the differential effect of different statins on the mass of level of Lp-PLA2 in patients with AIS.

Methods:
A total of 69 patients with AIS aged 40–70 years compared with matched 39 healthy controls were involved in this case-control study. The AIS patients were divided according to the statins therapy into 39 patients on statins therapy (statins on), and 30 patients were not on the statins therapy (statins off). Anthropometric variables including weight, height, body mass index (BMI), and blood pressure profile were estimated. Besides, biochemical variables including lipid profile[total cholesterol (TC), triglyceride (TG), LDL, very low-density lipoprotein (VLDL), high-density lipoprotein (HDL)], Lp-PLA2 mass levels, high-sensitive C-reactive protein (Hs-CRP) were estimated.

Results:
Patients with AIS had high Lp-PLA2 mass levels (P < 0.01) that positively correlated with high Hs-CRP, blood pressure, BMI, TC, TG, VLDL, LDL, and negatively correlated with HDL as compared with healthy controls. As well, statins on patients had lower Lp-PLA2 mass levels (9.82 ± 3.19 IU/mL) compared with statins off patients (16.55 ± 4.72 IU/mL), (P = 0.0001). Regarding the gender differences in the Lp-PLA2 mass level, it was higher in men patients with AIS compared with comparable females (P = 0.03).

Conclusions:
Lp-PLA2 mass level was higher in patients with AIS and linked with underlying poor cardio-metabolic disorders. Therefore, the Lp-PLA2 mass level is observed to be a surrogate biomarker of AIS mainly in patients with poor cardio-metabolic disorders. Statin therapy improves the Lp-PLA2 mass level and the poor cardio-metabolic profile in patients with AIS.