bluuebunny/crossref_metadata_2025 huggingface dataset:
Hydrocephalus in Spina Bifida
Authors: Blount Jeffrey P , Maleknia Pedram , Hopson Betsy D , Rocque Brandon G , Oakes W J
Published: December 2021

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

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

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

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

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

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

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.
Subarachnoid Hemorrhage and Hydrocephalus
Authors: Bhattacharjee Suchanda , Rakesh Das , Ramnadha Reddy , Manas Panigrahi
Published: December 2021

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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.
Chronic Pregabalin Abuse with Subacute Encephalopathy Mimicking Autoimmune Encephalitis
Authors: Harsha Kamble Jayaprakash , Joshy EV , Aravinda Rangenahalli Vasudev , Poornima Rangareddy
Published: December 2021

Pregabalin, a gabapentinoid frequently prescribed for neuropathic pain, also increasingly identified as a drug for abuse. We describe a unique case of 31-year-old man presented with subacute neuro-psychiatric symptoms and a spectrum of movement disorders, suspicious of autoimmune encephalitis. Initial response to IV methylprednisolone followed by recurrence of symptoms strengthened our suspicion for autoimmune encephalitis. His autoimmune encephalitis workup was negative, however, his two MRIs showed parenchymal changes. The patient, finally, confessed to chronic pregabalin abuse. He recovered completely upon stopping pregabalin abuse and remained asymptomatic at follow-up. To the best of our knowledge, we are the first to describe parenchymal changes in MRI mimicking autoimmune encephalitis in a case of pregabalin abuse. Despite the limited number of reports of pregabalin abuse in India, it is time to consider restricting the pregabalin availability, in line with many Western countries. This is particularly relevant to India, where, one legitimate prescription can be used by many to buy medicines at multiple stores without any questions being asked by the pharmacists.
Perioperative Management of a Patient with Chin-On-Chest Deformity Presenting for Reconstructive Spine Surgery
Authors: Khandelwal Ankur , Sokhal Suman , Dube Surya K , Goyal Keshav , Singh Akanksha , Tandon Vivek , Phalak Manoj , Chaturvedi Arvind , Kale Shashank Sharad
Published: December 2021

Chin-on- chest deformity is not uncommon sequelae of ankylosing spondylitis. Apart from difficult airway, several other considerations might include co-existing cardio-respiratory embarrassment, osteoporotic bones, and neurological perturbations. We describe the successful anesthetic management of a case of chin-on-chest deformity with no access to midline neck structures and extremely difficult airway posted for corrective spine surgery.
Osteomalacic Myopathy in Children and Adolescents with Vitamin-D Deficiency
Authors: Sahni Suhalika S , Kakkar Shruti , Kumar Ruchika , Goraya Jatinder S
Published: December 2021

Background:

Osteomalacic myopathy secondary to vitamin-D deficiency is an under-recognized cause of muscle weakness in children and adolescents.

Aim:

To describe a cohort of children and adolescents with osteomalacic myopathy.

Settings and Design:

Pediatric neurology unit of a tertiary care hospital.

Methods and Material:

Charts of children and adolescents with osteomalacic myopathy were retrospectively reviewed for demographics, clinical presentation, laboratory investigations, and treatment response. Diagnosis of vitamin-D deficiency was made on the basis of a combination of clinical, biochemical, and radiographic findings. Response to treatment with vitamin-D confirmed vitamin-D deficiency as the cause of myopathic symptoms.

Results:

Twenty-six children—15 girls and 11 boys aged between 20 months and 19 years—with osteomalacic myopathy were identified. Fifteen (58%) children were between 10 years and 19 years of age. Twenty-one (81%) children presented with myopathic symptoms of progressive walking difficulty, with eventual loss of ambulation in six. Four children came to attention through hypocalcemic seizures. One nonambulatory child with cerebral palsy presented with loss of previously attained ability to roll over and sit. All children had proximal muscle weakness on examination. Fifteen (58%) children had clinical signs of rickets. All the children who underwent biochemical (n = 24) and radiographic (n = 16) investigations had results consistent with vitamin-D deficiency. Only in one child, the diagnosis of osteomalacic myopathy was made on the basis of clinical findings. Response to vitamin D was uniformly good.

Conclusions:

Vitamin-D deficiency should be considered in the differential diagnosis of proximal myopathy in children and adolescents.
Risk Factors, Recurrence and Short-Term Outcomes for Progressive Cerebral Infarction
Authors: Wang Yuanwei , Wang Ying , Du Li , Liu Ping , Fei Zhengdong
Published: December 2021

Background:

Only a few studies have investigated the risk factors for the prognosis of progressive cerebral infarction (PCI) and the relationship between PCI and cerebral infarction (CI) recurrence.

Objective:

The objective of this study is to analyze the risk factors for PCI and PCI prognosis and evaluate the relationship between PCI and CI recurrence, mortality, short-term outcomes.

Methods:

The retrospective study included 221 CI patients. PCI and non-PCI patients were divided into the observation (91) and control (130) groups, respectively. Patients' clinical data, including diabetes history, laboratory blood indices, National Institutes of Health Stroke Scale (NIHSS) scores at admission, and presence of carotid and intracranial artery stenoses, were retrospectively analyzed. Instances of CI recurrence, adverse short-term outcomes, and death within 1 year postanalysis were recorded.

Results:

Diabetes, homocysteine, NIHSS score at admission, fibrinogen, and intracranial artery stenosis were associated with PCI. Age, NIHSS score at admission, and pneumonia were associated with PCI prognosis. By 12-month follow-up, the mortality and adverse outcome rate in the observation group were significantly increased than those of the control group. There was no diference in CI recurrence rates between the groups. Adverse outcomes were associated with PCI, age, and homocysteine.

Conclusions:

PCI risk factors included diabetes, homocysteine, NIHSS score at admission, fibrinogen, and intracranial artery stenosis. The independent risk factors for PCI prognosis included age, high NIHSS score at admission, and pneumonia. PCI did not affect CI recurrence but may affect adverse short-term outcomes.
Predictive Factors of Postoperative Peritumoral Brain Edema after Meningioma Resection
Authors: Li Liang-Ming , Zheng Wen-Jian , Chen Ying-Zhi , Hu Zi-Hui , Liao Wei , Lin Qi-Chang , Zhu Yong-Hua , Huang Han-Tian , Lin Shao-Hua , Gong Jian
Published: December 2021

Background:

Postoperative peritumoral brain edema (PTBE) is the progressively exacerbating cerebral edema following meningiomas resection.

Objective:

The study aims to identify the predictive factors of postoperative PTBE.

Materials and Methods:

A retrospective study was conducted on the 117 cases of patients who underwent meningioma. The histopathological features of the tumors were re-assessed according to WHO 2016 classification. Clinical and pathohistological features were analyzed.

Results:

Thirteen patients (11.1%) were diagnosed having postoperative PTBE. Preoperative seizure (odds ratio [OR] = 6.125, P = 0.039) and histological prominent nucleoli (OR = 3.943, P = 0.039) were the independent risk factors for postoperative PTBE. Meningiomas with a parietal localization were more likely to develop postoperative PTBE (OR = 3.576, P = 0.054). Brain invasion and large tumor volume did not increase complication rate. Preoperative edema index was significantly higher in brain invasive meningiomas (3.0 ± 2.2 versus 1.8 ± 1.7, P = 0.001). Patients having moderate preoperative PTBE were prone to the complication (21.4% versus 7.9%, P = 0.100).

Conclusions:

Preoperative seizure were the predictive factors for postoperative PTBE. Careful venous protection during the operation may be helpful, especially for tumors locating in the parietal lobe. Prominent nucleoli observed in postoperative pathology should warrant surgeons' attention. Comprehensive perioperative management is essential for these patients.
Atlantoaxial and Subaxial Cervical Spinal Instability in Two Cases with Neurofibromatosis-Type 1
Authors: Goel Atul , Dandpat Saswat , Shah Abhidha , Bhambere Sagar , Darji Hardik
Published: December 2021

This is a report of two patients who were diagnosed to have NF-1. The patients had severe dystrophic soft tissue and bone changes leading to craniovertebral junction and subaxial cervical spinal instability and deformity. Both the patients underwent atlantoaxial and subaxial cervical spinal stabilization. No bone, soft tissue or tumor resection was done for decompression. Both patients had gratifying clinical recovery. Follow-up in both the patients is more than 12 months.
A Rare Case of Stroke in an Adolescent Violinist Due to Thoracic Outlet Syndrome
Authors: Kuril Sandeepkumar , Chopade Pramod R , Mandava Mamatha , Bhatia Sonal
Published: December 2021

Thoracic outlet syndrome (TOS), a rare condition, results from the compression of neurovascular structures traversing from the neck through the thoracic outlet into the axilla. It can develop from chronic repetitive activities of the upper extremities, commonly reported in athletes playing sports involving vigorous use of arms and shoulders. While symptoms of neurovascular compression can occur, stroke due to TOS in children is not commonly reported. We describe a rare case of a healthy 14-year-old boy, a competitive violinist, with acute limb ischemia from extensive occlusive thrombi involving several arteries in the right upper extremity as well as the right vertebral artery, which ultimately caused infarcts in the bilateral posterior circulation. The etiology was determined to be TOS leading to impingement of the right subclavian artery by a fused cervical rib aggravated by patient's prolonged violin practice. This case represents the first description of stroke from TOS in an adolescent violinist.
Embolectomy by SOLUMBRA Technique for Nontarget Intracranial Glue Migration— Complication and Bailout after Percutaneous Embolization of Orbital Meningioma
Authors: Chauhan Richa Singh , Prasad Chandrajit , Siddiqui Shahyan M , Srinivas Dwarakanath
Published: December 2021

Presurgical devascularization of neoplasms of the head and neck can be achieved by endovascular as well as direct percutaneous embolization techniques. We report a case of percutaneous glue embolization of an orbital meningioma, complicated by delayed acute stroke due to the distal migration of polymerized glue in the left middle cerebral artery. To the best of our knowledge, this is the first report to discuss the percutaneous embolization of orbital meningioma complicated by stroke due to intracranial glue migration.