bluuebunny/crossref_metadata_2025_split huggingface dataset:
Sporadic Inclusion Body Myositis
Authors: Challa Sundaram , Jakati Saumya , Narla Swethalakshmi , Uppin Megha S , Kannan Meena A , Jagarlapudi M K Murthy 
Published: December 2021

Background:
Sporadic inclusion body myositis (s-IBM) is rare in India.

Aim:
The aim of this study was to diagnose s-IBM according to the European Neuromuscular Center (ENMC) IBM research diagnostic criteria 2011.

Materials and Methods:
A retrospective review of patient records diagnosed as s-IBM according to the above criteria during the period from January 2010 to May 2015 was done with an emphasis on pattern of muscle weakness. Serumcreatine kinase (CK) andelectromyography (EMG) were noted. Muscle biopsy was evaluated with basic panel of histochemical stains including Congo red stain. Immunohistochemistry (IHC) with ubiquitin was done in 10 biopsies. IHC for major histocompatibility complex-1 and electron microscopy studies were not performed.

Results:
The diagnosis of s-IBM constituted 5 clinicopathologically defined, 12 clinically defined, and 10 probable IBM in the study period. There was male predominance with median age at 51 and duration of disease varying from 1–5 years. All the patients presented with insidious onset of muscle weakness of quadriceps and/or forearm flexors. CK varied from 57–2939 IU/L. EMG was myopathic in 22, mixed in 2, and neuropathic in 3. Endomysial inflammation was seen in 23 (85.19%) and rimmed vacuoles in 24 (88.89%). Amyloid was demonstrated in only 5 (18.52%) and ubiquitin in 2 biopsies. Mitochondrial abnormalities were seen in 92.59% biopsies.

Conclusions:
Application of the ENMC IBM research diagnostic criteria allowed diagnosis of clinically-defined and probable IBM in the absence of all pathology criteria. Rimmed vacuoles in 88.89% of biopsies indicate presentation at a late stage. Use of ancillary techniques can improve diagnostic yield.
Endoscopic-Assisted C2 Transverse Process Mass Excision
Authors: Bajaj Jitin , Ratre Shailendra , Yadav Yad Ram 
Published: December 2021

Background and Introduction:
C2 transverse process exostoses are rare lesions. Due to critical structures surrounding them, their excision is challenging. There are sparse reports of anterior retropharyngeal approach (ARPA) for high-cervical transverse process mass and none for endoscopic ARPA approach.

Objective:
A step-by-step technical report with its video is presented.

Surgical Technique:
A 14-year-old girl presented with chronic right-sided neck pain. The computed tomography scan revealed a 6.5 cm3 mass in the right transverse process extending into the lateral mass of the C2 vertebra. The mass was anterior and in direct contact with the vertebral artery. She underwent a minimally invasive endoscopic ARPA.

Results:
The mass could be excised along with its cartilaginous cap without any complications. The patient's symptoms resolved completely. The biopsy came out as osteochondroma.

Conclusion:
Endoscopic ARPA is a minimally invasive option for high-cervical tumors and was found safe and effective for C2 transverse process osteochondroma.
Cognitive Capacity Assessment
Authors: Bajpai Swati , Nehra Ashima , Pandey R M , Sati Hemchand , Singh Rajesh Kumar , Vishnu Venugopalan Y , Rajan Roopa , Singh Manmohan , Srivastava Achal , Srivastava Padma , Tripathi Manjari 
Published: December 2021

Background:
Disability evaluation as per World Health Organization includes assessment of impairments, activity limitations, and participation restriction, which unfortunately is not assessed by the existing guidelines of disability in India.

Aim:
The aim of this study wasto comparea new study criterion with the existing guidelines for assessing cognitive disability for chronic neurological conditions.

Methodology:
A cross-sectional pilot study was conducted on 41 participants. They were assessed on an existing guideline (Gazette India 200,1 assessing physical domain and Intelligence Quotient[IQ]) and study criterion assessing three aspects of cognitive capacity: IQ, neurocognitive functioning, and QOL.

Results:
The existing guideline underestimated 84% of cases for disability. The average percentage of disability measured by the study criterion was 33.2% more as compared to existing guidelines with S.D of 26.6.

Conclusion:
Cognitive capacity assessment is an important element to be measured in chronic neurological disability certification. However, a large sample is required to make an affirmative claim for the same.
Endothelial Nitric Oxide Synthase (Glu298Asp) Polymorphism is Associated Significantly with Ischemic Stroke Presenting with Seizures and Altered Sensorium
Authors: Saluja Alvee , Saraswathy Kallur N , Thakur Sunil , Margekar Shubhalaxmi , Goyal Ankit , Dhamija Rajinder K 
Published: December 2021

Background:
Endothelial nitric oxide synthase (eNOS) is an enzymatic marker whose genetic polymorphism might predispose to acute ischemic stroke (AIS) via vascular endothelial dysfunction. It has a potential role in atherosclerosis, making it a plausible risk factor for stroke. Prior studies have failed to prove a conclusive relationship between eNOS polymorphism and AIS.

Objective:
The aim of this study is to find an association between the presence of eNOS polymorphism (Glu298Asp) and the risk of developing AIS.

Materials and Methods:
We recruited 307 subjects including 153 AIS cases and 154 healthy controls. The eNOS (Glu298Asp) polymorphism was identified in EDTA blood by PCR amplification of the target region followed by restriction enzyme digestion, and genotyping on Agarose gel. GG, GT and TT genotypes were obtained. Statistical analysis was done using SPSS software version 20.

Results:
A significant association was found between the presence of TT genotype and the risk of AIS (Odd's ratio (OR): 2.43, P-value = 0.038). There was no significant association between the TT genotype and the traditional stroke risk factors. However, the TT genotype was significantly associated with the presence of altered consciousness (OR: 5.27, 95% CI: 1.59–17.04, P-value = 0.003) and with the occurrence of seizures at presentation (OR: 7.98, 95% CI: 1.99–32.09, P-value = 0.007).

Conclusions:
There is a significant association between the presence of eNOSpolymorphism (Glu298Asp) and the risk of AIS, and the TT genotype may predispose to a more severe initial presentation of ischemic stroke.
Calvarial Lesions
Authors: Attri Gagandeep , Maurya Ved Prakash , Srivastava Arun Kumar , Behari Sanjay , Bhaisora Kamlesh Singh , Sardhara Jayesh , Verma Pawan Kumar , Nazar Aftab Hasan , Jaiswal Sushila 
Published: December 2021

Background:
The human calvaria harbors a variety of pathology and majority of them are incidentally noticed as painless swelling. The aim of the present study is to describe the histopathological subtypes of calvarial lesions, their management and factors affecting their surgical outcome at a tertiary care referral center.

Material and Methods:
All patients who underwent excision of the calvarial lesions over the last 15 years (from January 2005 to July 2019) were included in this study. Patients having calvarial pathology of infective origin and recurrent lesions were excluded. Any patient with multiple calvarial lesions who have been operated more than one time for same histopathological diagnosis was counted as one patient. We studied Karnofsky Performance Status (KPS) scores and radiological changes at 3-month follow up.

Results:
Total 65 patients were recruited in this retrospective observational study. The median age of patients in the study was 29 years (range: 8 years to 68 years). Fibrous dysplasia 20 (30.7%) was the commonest lesion while metastatic thyroid carcinoma 3 (4.6%) was the most common malignant pathology. Complete excision was performed in 51 (78.5%) of patients while in 14 (21.5%) cases, subtotal or near total decompression were achieved. After three months of surgery, there was significant improvement in the KPS score (P < 0.00001). Duration of follow up ranges from 6 months to 5 years with 4 mortality in the study.

Conclusions:
Most of the calvarial tumors were benign and surgically addressable. The malignant lesions were scattered with diverse underlying pathology and required individualized holistic approach.
Role of Clinical Neuropsychologists in Awake-Craniotomy
Authors: Hande Vasudha H , Gunasekaran Harini , Hegde Shantala , Shashidhar Abhinith , Arimappamagan Arivazhagan 
Published: December 2021

Background:
Awake craniotomy encompasses surgical resection of focal zone of neurological impairment, using intraoperative functional zone mapping. The strength of the procedure is maximum retention of functionally significant zones to ensure better function and quality of life outcomes in patients. A clinical neuropsychologist plays a vital role in profiling the patient's cognitive and psychosocial functioning as well as increasing the efficacy of functional zone mapping procedures.

Objective:
Aim of this article is to summarize the literature on the role of clinical neuropsychologists in awake craniotomy and underscoring the need for establishing standardized operating procedures for neuropsychologists in awake craniotomy highlighting experiential anecdotes from a tertiary care facility.

Materials and Methods:
A review of articles that elucidate the role of clinical neuropsychologists was done and summarized to highlight the role of clinical neuropsychologists. An attempt was made to explain the implementation of this role in regular clinical practice at a tertiary care facility.

Results:
The role of a clinical neuropsychologist is highly crucial at pre-/during and postawake craniotomy, and has a significant bearing on the overall psychological outcome of the individual. The need for a standardized protocol to unify practice and increase the efficacy of the awake-craniotomy procedure is put forth. Recommendations for future directions in research to increase the scope of neuropsychologists in awake craniotomy have also been made.
Hydrocephalus in Spina Bifida
Authors: Blount Jeffrey P , Maleknia Pedram , Hopson Betsy D , Rocque Brandon G , Oakes W J 
Published: December 2021

Abstract
Hydrocephalus is the most important co-morbidity in myelomeningocele from a neurosurgical perspective. Historically, 75-80% of patients with myelomeningocele have required treatment with a shunt but recent advances including intra-uterine myelomeningocele closure and ETV-CPC are reducing this burden. The expression of hydrocephalus differs between patients and across the life span. Hydrocephalus impacts the clinical expression of other important co-morbidities including the Chiari II malformation and tethered spinal cord. Shunt failure is often the key stress to prompt symptomatic worsening of these other conditions. Shunt failure may occur with minimal ventricular change on CT or MRI in Spina Bifida patients. Waiting for radiographic changes in symptomatic SB patients with shunts may result in hydrocephalus related fatalities. It is hypothesized but not proven that shunt failure may contribute to respiratory insufficiency and be a risk factor for sudden death in adult patients with spina bifida. Excellence in hydrocephalus management in MMC is essential for proper care, good outcomes, and quality of life for patients and families.
Three Decades of Vellore Grading for Tuberculous Meningitis with Hydrocephalus
Authors: Rajshekhar Vedantam 
Published: December 2021

Abstract

Background:
This review documents the evolution of the Vellore grading system for tuberculous meningitis and hydrocephalus (TBMH), its evaluation by different authors, and analyzes the need for further modification in light of the published literature.

Methods:
Published literature was searched in PubMed and Google Scholar using the search terms, “tuberculous meningitis hydrocephalus” and “Vellore grading.” The retrieved articles were reviewed by the author and the appropriate ones were chosen for inclusion in the study.

Results:
Vellore grade (1–4, with 1 being the best grade and 4 being the worst grade) was found to be the sole statistically significant factor associated with outcome following VP shunt or ETV in several studies. Additionally, Vellore grades also correlate with the likelihood of success following ETV. However, the use of response to external ventricular drainage (EVD) in managing Vellore grade 4 patients has remained contentious as a small but significant proportion of patients have a good outcome following shunt, irrespective of their response to the EVD. The latter findings suggest that grade 4 patients might not constitute a homogenous group. It is proposed that grade 4 be subdivided into grades 4a and 4b, which might help in prognostication and in surgical management of the hydrocephalus in patients with TBMH.

Conclusions:
Vellore grading has proved its utility as a prognostic tool and can aid surgical decision-making. However, management of patients in grade 4 might be better rationalized with its division into grades 4a and 4b.
Endoscopic Third Ventriculostomy - A Review
Authors: Yadav Yad Ram , Bajaj Jitin , Ratre Shailendra , Yadav Nishtha , Parihar Vijay , Swamy Narayan , Kumar Ambuj , Hedaoo Ketan , Sinha Mallika 
Published: December 2021

Abstract

Background:
Endoscopic third ventriculostomy (ETV) has become a proven modality for treating obstructive and selected cases of communicating hydrocephalus.

Objective:
This review aims to summarize the indications, preoperative workup, surgical technique, results, postoperative care, complications, advantages, and limitations of an ETV.

Materials and Methods:
A thorough review of PubMed and Google Scholar was performed. This review is based on the relevant articles and authors' experience.

Results:
ETV is indicated in obstructive hydrocephalus and selected cases of communicating hydrocephalus. Studying preoperative imaging is critical, and a detailed assessment of interthalamic adhesions, the thickness of floor, arteries or membranes below the third ventricle floor, and prepontine cistern width is essential. Blunt perforation in a thin floor, while bipolar cautery at low settings and water jet dissection are preferred in a thick floor. The appearance of stoma pulsations and intraoperative ventriculostomography reassure stoma and basal cistern patency. The intraoperative decision for shunt, external ventricular drainage, or Ommaya reservoir can be taken. Magnetic resonance ventriculography and cine phase-contrast magnetic resonance imaging can determine stoma patency. Good postoperative care with repeated cerebrospinal fluid drainage enhances outcomes in selected cases. Though the complications mostly occur in an early postoperative phase, delayed lethal ones may happen. Watching live surgeries, assisting expert surgeons, and practicing on cadavers and models can shorten the learning curve.

Conclusion:
ETV is an excellent technique for managing obstructive and selected cases of communicating hydrocephalus. Good case selection, methodical technique, and proper training under experts are vital.
Spontaneous Intracranial Hypotension - A Dilemma
Authors: Shukla Dhaval , Sadashiva Nishanth , Saini Jitender , Kamath Sriganesh 
Published: December 2021

Abstract

Background:
Spontaneous intracranial hypotension (SIH) is a highly misdiagnosed and underdiagnosed disorder.

Objective:
Update evaluation and treatment of spontaneous intracranial hypotension.

Methods and Material:
Narrative review.

Results:
Traditionally, SIH is diagnosed when a headache has developed spontaneously and in temporal relation to a CSF leak (evident on imaging) and/or CSF hypotension (lumbar puncture opening pressure <60 mm CSF). However, lumbar puncture is not mandatorily required to diagnose SIH. Besides headache, other symptoms such as nausea/vomiting in 50.6%, neck pain/stiffness in 33%, tinnitus in 19%, dizziness in 14%, hearing disturbances in 10.7%, followed by visual disturbances, vertigo, back pain, and cognitive symptoms may be present. In suspected cases of SIH, brain and spine should be evaluated with MRI. Dynamic computerized tomographic myelography is required to demonstrate the site of spinal CSF leak. Epidural blood patch (EBP) is a minimally invasive treatment for spontaneous intracranial hypotension (SIH) refractory to medical management and provides symptomatic relief in up to 90% of patients even in patients with bilateral subdural hematomas. The CSF-venous fistulas do not respond well to EBP, and the most definitive curative treatment is the surgical closure of the fistula.

Conclusions:
The SIH is a distinct entity and requires a high index of suspicion for diagnosis. A post-contrast MRI should be included for evaluation of headaches. Spinal MRI should be done to demonstrate the site of leak. Epidural blood patch therapy is the most effective treatment of SIH. Most SDHs associated with SIH do not require treatment.
Hydrocephalus Associated with Posterior Fossa Tumors
Authors: Muthukumar Natarajan 
Published: December 2021

Abstract

Background:
Hydrocephalus associated with posterior fossa tumor (PFT) is a common neurosurgical problem, the management of which is still controversial.

Aim:
To provide an overview of the advantages and limitations of different management strategies for hydrocephalus associated with PFT both before and after resection of these tumors.

Methods:
Structured review of the literature on the management of hydrocephalus in PFT both in children and adults.

Results:
The incidence of hydrocephalus associated with PFT at the time of presentation is more in children (70–90%) than adults (10–21%). This difference is maintained for hydrocephalus after the resection of PFT (~30% for children and 1.2–6.9% for adults). Preresection hydrocephalus is obstructive while emerging evidence in the literature suggests that postresection hydrocephalus may have a communicating component. The treatment of preresection hydrocephalus associated with PFT has undergone a paradigm shift in the past two decades. Preoperative Cerebrospinal Fluid (CSF) diversion is less commonly used except when required by the clinical condition of the patient. Preresection hydrocephalus may be treated by steroid use and early tumor removal, perioperative use of external ventricular drainage, or endoscopic third ventriculostomy in selected patients. Various prediction scales are available to assess the risk of postresection hydrocephalus in PFT. Certain histological tumor types and molecular phenotypes of PFT are more commonly associated with hydrocephalus. CSF diversion through endoscopic third ventriculostomy or ventriculoperitoneal shunts remains the management strategies for postresection hydrocephalus. The failure rates and the time-to-failure of both endoscopic third ventriculostomy and CSF shunts in PFT are variable and surgeons should be aware of these while taking management decisions.

Conclusions:
Hydrocephalus associated with PFT affects the quality of life of patients with such lesions. Routine preoperative CSF diversion is not necessary for the vast majority of patients with posterior fossa tumor-related hydrocephalus. A high index of suspicion and aggressive surveillance is required for the early identification and appropriate management of postresection hydrocephalus. Future studies are needed to address several unanswered questions pertaining to the management of this condition.
Diagnostic Nuances and Surgical Management of Arrested Hydrocephalus
Authors: Panigrahi Manas K , Kodali Sandhya , Chandrsekhar Y B V K , Vooturi Sudhindra 
Published: December 2021

Abstract
Hydrocephalus is characterized by the increased volume of cerebrospinal fluid (CSF) with enlarged cerebral ventricles. In nearly 50% of the patients, if left untreated, the balance between CSF production and absorption is achieved, resulting in arrested hydrocephalus (AH). However, 15% of them who are diagnosed as arrested can progress over a period of time. Importantly, a large fraction of patients with hydrocephalus in India, may not have access to tertiary level care. Therefore, both progressive hydrocephalus and insidious progression of AH with related mortality and morbidity could be higher in India. The pathophysiology behind AH and insidious progression of AH are poorly established. Unfortunately, there are no established clinical or radiological parameters identifying or predicting AH from progressive hydrocephalous. Diagnosis is often based on a combination of neurological, psychometric, and magnetic resonance imaging (MRI) findings. Invasive monitoring of intracranial pressure (ICP) and telemetric ICP measurement is increasingly helping surgeons to detect insidious progressive AH in the early stages. In patients with AH, surgery may not be always necessary and a conservative approach is often adopted. On the contrary, AH that becomes progressive may require intervention. Surgical intervention should not be delayed and endoscopic third ventriculostomy (ETV) is preferable over shunt placement. Importantly, comprehensive counseling and the appropriate selection of patients are pivotal in improving outcomes and reducing complications.
Lumboperitoneal Shunts - Patient Selection, Technique, and Complication Avoidance
Authors: Sinha Mallika , Bajaj Jitin , Kumar Ambuj , Hedaoo Ketan , Sharma Sandeep , Konchada Kamesh , Ratre Shailendra , Parihar Vijay S , Swamy Narayan M , Yadav Yad R 
Published: December 2021

Abstract

Background:
Lumboperitoneal shunt is a known procedure for communicating hydrocephalus. Being an extracranial procedure, it can also be utilized in normal-sized ventricles.

Objective:
To report our experience of lumboperitoneal shunt done with a minimal follow-up of 12 months with an emphasis on patient selection, technique, and complication avoidance.

Methods:
This was a retrospective analysis of patients who underwent LP shunt during October 2014–October 2019 at the authors' institute. Inclusion criteria were patients with communicating hydrocephalus due to tubercular meningitis, normal pressure hydrocephalus, idiopathic intracranial hypertension, and postoperative refractory cerebrospinal fluid leaks. Data were collected for demographics, Glasgow coma scale and Glasgow outcome scale, vision, gait, memory, urinary incontinence, failed attempts, and complications.

Results:
A total of 426 patients underwent the LP shunt procedure. The commonest indication was tubercular meningitis followed by idiopathic intracranial hypertension and normal pressure hydrocephalus. Age ranged from 16 to 72 years. There were 255 male and 171 female patients. The mean follow-up was 41 ± 8 months. Overall, 301 patients (70.6%) had neurological improvement. Shunt-related complications occurred in 112 (26.29%) patients, of which shunt block was the commonest. Other complications were infection in 17 (3.9%) patients and extrusion in four (0.9%) patients. Transient postural headache was seen in 46 (10.7%) patients, which gradually improved.

Conclusion:
Lumboperitoneal shunt was found to be a safe and effective treatment in appropriately selected communicating hydrocephalus patients. A meticulous technique reduces the complication rate.