The role of susceptibility-weighted imaging & contrast-enhanced MRI in the diagnosis of primary CNS vasculitis: a large case series.

Primary CNS Vasculitis (PCNSV) is a rare, diverse, and polymorphic CNS blood vessel inflammatory condition. Due to its rarity, clinical variability, heterogeneous imaging results, and lack of definitive laboratory markers, PCNSV diagnosis is challenging. This retrospective cohort analysis identified patients with histological diagnosis of PCNSV. Demographic data, clinical presentation, neuroimaging studies, and histopathologic findings were recorded. We enrolled 56 patients with a positive biopsy of CNS vasculitis. Most patients had cerebral hemisphere or brainstem symptoms. Most brain MRI lesions were bilateral, diffuse discrete to confluent white matter lesions. Frontal lobe lesions predominated, followed by inferior cerebellar lesions. Susceptibility-weighted imaging (SWI) hemorrhages in 96.4% (54/56) of patients, either solitary microhemorrhages or a combination of micro and macrohemorrhages. Contrast-enhanced T1-WIs revealed parenchymal enhancement in 96.3% (52/54 patients). The most prevalent pattern of enhancement observed was dot-linear (87%), followed by nodular (61.1%), perivascular (25.9%), and patchy (16.7%). Venulitis was found in 19 of 20 individuals in cerebral DSA. Hemorrhages in SWI and dot-linear enhancement pattern should be incorporated as MINOR diagnostic criteria to diagnose PCNSV accurately within an appropriate clinical context. Microhemorrhages in SWI and venulitis in DSA, should be regarded as a potential marker for PCNSV.

Bilateral pallidotomy for acquired or heredodegenerative generalized dystonia in children.

OBJECTIVE: Dystonias are relatively rare disorders characterized by sustained or intermittent muscle contractions causing abnormal movements or postures. Generalized dystonia is a therapeutic challenge because medications are unable to control dystonia adequately in most patients. These patients may be candidates for surgical therapy. The commonly used surgical procedures in these patients are pallidotomy and deep brain stimulation. Limited studies are available on the role of pallidotomy in children with acquired/heredodegenerative generalized dystonia. The objective of this study was to describe the authors' experience with bilateral pallidotomy in this group of patients. METHODS: The authors retrospectively reviewed all pediatric patients (less than 18 years of age) with acquired/heredodegenerative generalized dystonia who underwent bilateral simultaneous pallidotomy at their center between January 2014 and January 2021. Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) scores before and after surgery were recorded. Complications arising after the procedure were recorded as well. RESULTS: Ten patients (8 male and 2 female) with a mean (range) age of 11.1 (5-17) years were included in this study. The mean duration between disease onset and surgical intervention was 3.9 years. Two patients presented in status dystonicus. The mean ± SD (range) preoperative BFMDRS score of the patients without status dystonicus (n = 8) was 80 ± 18.9 (59.5-108). The mean ± SD BFMDRS score at the time of discharge from the hospital after surgery was 58.8 ± 37.9. Three patients had more than 20% change in BFMDRS score at the time of discharge from the hospital. The mean improvement was 25.5% at the end of 1 year. Of 5 surviving patients in the non-status dystonicus group, 3 patients had more than 40% change in BFMDRS score while the other 2 patients developed recurrence at the last follow-up (4.5 years). Status dystonicus abated after bilateral pallidotomy in both patients. Permanent bulbar complications were seen in 2 patients. CONCLUSIONS: Bilateral pallidotomy may result in clinically significant improvement in children with acquired/heredodegenerative generalized dystonia, although the benefits should be closely weighed against the risk of irreversible bulbar dysfunction. It is a viable option for children in resource-limited settings.

Primary CNS vasculitis (PCNSV): a cohort study.

Primary CNS Vasculitis (PCNSV) is a rare inflammatory disorder affecting the blood vessels of the central nervous system. Patients present with a combination of headaches, seizures, and focal neurological deficits. There is usually a diagnostic delay. Treatment is based on observational studies and expert opinion. Our objective was to identify clinical, laboratory, neuroimaging, pathologic or management-related associations with 2 year outcome in patients with primary CNS vasculitis. We conducted a cohort study at a single tertiary care referral centre of prospectively (2018-2019) and retrospectively (2010-2018) identified individuals with primary CNS vasculitis (diagnosis was proven by either brain biopsy or cerebral digital subtraction angiography). Clinical, imaging and histopathologic findings, treatment, and functional outcomes were recorded. Univariate and stepwise multiple logistic regression were applied. P-value<0.05 was considered statistically significant. The main outcome measures were documentation of clinical improvement or worsening (defined by mRS scores) and identification of independent predictors of good functional outcome (mRS 0-2) at 2 years. We enrolled eighty-two biopsy and/or angiographically proven PCNSV cases. The median age at presentation was 34 years with a male predilection and a median diagnostic delay of 23 months. Most patients presented with seizures (70.7%). All patients had haemorrhages on MRI. Histologically lymphocytic subtype was the commonest. Corticosteroids with cyclophosphamide was the commonest medication used. The median mRS at follow-up of 2 years was 2 (0-3), and 65.2% of patients achieved a good functional outcome. Myelitis and longer duration of illness before diagnosis were associated with poorer outcomes. The presence of hemorrhages on SWI sequence of MRI might be a sensitive imaging marker. Treatment with steroids and another immunosuppressant probably reduced relapse rates in our cohort. We have described the third largest PCNSV cohort and multi-centre randomised controlled trials are required to study the relative efficacy of various immunosuppressants.Study registration: CTRI/2018/03/012721.

COVID-19 Vaccine-Induced Immune Thrombotic Thrombocytopenia (VITT) and Cerebral Venous Sinus Thrombosis (CVST)- Lessons for India.

The COVID-19 pandemic evolved rapidly, overwhelming health care systems around the world. The cost to life and socioeconomic burden prompted a search for new treatments and vaccines. Several collaborations developed and could deliver state-of-the-art vaccines with acceptable efficacy and safety in record time. Recently, vaccination with Oxford-AstraZeneca and Johnson and Johnson vaccines was halted due to the reported adverse effects of vaccine-induced immune thrombotic thrombocytopenia (VITT) and cerebral venous sinus thrombosis (CVST). Although a detailed risk-benefit analysis led to their reinstitution, physicians across the world are still trying to understand the pathophysiology and mechanisms of these neurological adverse effects in order to better identify, diagnose, and treat them. One of the mechanisms that have been implicated is related to the adenovirus-based vector of these vaccines. COVISHIELD, which is the most widely administered vaccine in India, also shares the same vector. As India enters the next phase of vaccine distribution for younger adults, there are chances that such adverse effects may emerge. In this review, we analyze the temporary suspension of the administration of the vaccines due to VITT/CVST, summarize the existing guidelines about diagnosis and treatment of these neurological disorders as well as the need for increasing pharmacovigilance and awareness among physicians. Screening for potential risk factors, avoiding aggravating factors like dehydration, and providing choices in vaccinating the high-risk populations could help in avoiding these rare but potentially fatal adverse outcome.

Role of diffusion tensor imaging in the evaluation of ulnar nerve involvement in leprosy.

OBJECTIVE: Early detection of peripheral neuropathy is extremely important as leprosy is one of the treatable causes of peripheral neuropathy. The study was undertaken to assess the role of diffusion tensor imaging (DTI) in ulnar neuropathy in leprosy patients. METHODS: This was a case-control study including 38 patients (72 nerves) and 5 controls (10 nerves) done between January 2017 and June 2019. Skin biopsy proven cases of leprosy, having symptoms of ulnar neuropathy (proven on nerve conduction study) were included. MRI was performed on a 3 T MR system. Mean cross-sectional area, fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values of ulnar nerve at cubital tunnel were calculated. Additional ancillary findings and appearance of base sequences were evaluated. RESULTS: Ulnar nerve showed thickening with altered T2W signal in all the affected nerves, having an average cross-sectional area of 0.26 cm2. Low FA with mean of 0.397 ± 0.19 and high ADC with mean of 1.28 ± 0.427 x 10 -3 mm2/s of ulnar nerve in retrocondylar groove was obtained. In the control group, mean cross-sectional area was 0.71cm2 with mean FA and ADC of 0.53 ± 0.088 and 1.03 ± 0.24 x 10 -3 mm2/s respectively. Statistically no significant difference was seen in diseased and control group. Cut-off to detect neuropathy for FA and ADC is 0.4835 and 1.1020 × 10 -3 mm2/s respectively. CONCLUSION: DTI though is challenging in peripheral nerves, however, is proving to be a powerful complementary tool for assessment of peripheral neuropathy. Our study validates its utility in infective neuropathies. ADVANCES IN KNOWLEDGE: 1. DTI is a potential complementary tool for detection of peripheral neuropathies and can be incorporated in standard MR neurography protocol.2. In leprosy-related ulnar neuropathy, altered signal intensity with thickening or abscess of the nerve is appreciated along with locoregional nodes and secondary denervation changes along with reduction of FA and rise in ADC value.3. Best cut-offs obtained in our study for FA and ADC are 0.4835 and 1.1020 × 10 -3 mm2/s respectively.

Enhancing Quality of Life in Indian Parkinson's Disease Patients with Improved Measurement of Psychological Domains: A Perspective.

Parkinson's disease (PD) is the second most common progressive neuro-degenerative disorder. Research in PD is gradually increasing in India due to increased clinical cases, which could double by 2030 worldwide. Although its prevalence is low in India as compared to other countries, the total burden is much higher due to the large population size. PD is progressively debilitating, with pronounced motor and nonmotor symptoms (NMSs) that severely affect the quality of life (QoL) of patients and their caregivers. The progressive nature of the disease lays great emphasis on doctors to focus on the patients' QoL. As a consequence, Health-related QoL (HRQoL) has gradually become one of the main indicators for assessing health-related outcome. There is a growing need to pay attention to the NMSs and a pressing need to look at the QoL of Indian patients with PD through a culture and value specific lens. Research into the holistic QoL assessment with emphasis on psychological domains may allow for the early evaluation and intervention of depressive and cognitive symptoms in PD. This could result into increased productivity, reduced morbidity, and healthcare cost, which would in turn result into better QoL of Indian PD patients.

Odontogenic sinusitis publication trends from 1990 to 2019: a systematic review.

PURPOSE: Odontogenic sinusitis (ODS) is underrepresented in the literature compared to other forms of rhinosinusitis, specifically in sinusitis guidelines and position statements. ODS publication characteristics could help explain why ODS has received less attention in sinusitis guidelines and position statements. The purpose of this study was to explore trends in the quantity and quality of ODS studies over 3 decades from 1990 to 2019. METHODS: A systematic review was performed to identify all ODS studies from 1990 to 2019. The following variables from all ODS studies were compared between and across the 3 decades: authors' specialties, journal specialties, authors' geographic origins (continents), study topics, study designs, and evidence levels. RESULTS: From 1990 to 2019, there were 254 ODS studies that met inclusion criteria. Numbers of publications increased each decade, with 161 being published from 2010 to 2019. Otolaryngologists and dental authors published over 75% of ODS studies each decade, with 60-75% of ODS articles being published in otolaryngology or dental journals. European and Asian authors published the most ODS studies each decade. Overall, 92-100% of ODS publications per decade were level 4 and 5 evidence, with no significant changes between or across decades. CONCLUSION: While numbers of ODS publications increased each decade from 1990 to 2019, evidence levels remained low without significant changes over time. Otolaryngologists and dental authors published the majority of ODS studies each decade, with a minority of these studies being multidisciplinary. More ODS studies are needed across all aspects of the condition, and future projects would benefit from improved study designs and multidisciplinary collaboration.

Predicting Odontogenic Sinusitis in Unilateral Sinus Disease: A Prospective, Multivariate Analysis.

BACKGROUND: Odontogenic sinusitis (ODS) is a common cause of unilateral sinus disease (USD), but can be challenging to diagnose due to nonspecific clinical presentations, potentially subtle to absent dental pathology on sinus computed tomography (CT), and underrepresentation in the sinusitis literature. OBJECTIVE: Identify sinonasal clinical variables predictive of ODS in patients presenting with unilateral maxillary sinus opacification on sinus CT. METHODS: A prospective cohort study was conducted on 131 consecutive patients with USD and at least partial or complete maxillary sinus opacification on sinus CT. Patients' demographics, sinonasal symptoms (anterior and posterior drainage, nasal obstruction, facial pressure, smell loss, and foul smell), 22-item sinonasal outcome test, nasal endoscopy findings, CT findings, and histopathology were collected. Patients' diagnoses included ODS, chronic rhinosinusitis with or without nasal polyps, and inverted papilloma. Demographic and clinical data were compared between patients with unilateral ODS and non-odontogenic disease using univariate and multivariate analyses. RESULTS: Of the 131 USD patients, 65 had ODS and 66 had non-odontogenic disease. The following variables were significantly associated with unilateral ODS on multivariate analysis: middle meatal pus on endoscopy (OR= 17.67, 95% CI-5.69, 54.87; p = 0.001), foul smell (OR= 6.11, 95% CI-1.64, 22.82; p=.007), facial pressure (OR= 3.55, 95% CI-1.25, 10.12; p = 0.018), and any frontal opacification on CT (OR= 5.19, 95% CI-1.68, 16.06; p = 0.004). Any sphenoid opacification on CT was inversely related to ODS (OR = 0.14, 95% CI-0.03, 0.69; p = 0.016). The study was adequately powered. CONCLUSION: With unilateral maxillary sinus disease, the following features were significantly associated with ODS: foul smell, ipsilateral facial pressure, middle meatal pus on endoscopy, and any frontal sinus opacification on sinus CT. Additionally, any sphenoid sinus opacification on CT was inversely related to ODS. Presence or absence of these clinical variables can be used to increase or decrease one's suspicion of an odontogenic source of sinusitis.

Neonatal Lateral Epiglottic Defects.

INTRODUCTION: Multiple congenital abnormalities of the epiglottis have been reported and iatrogenic injuries to the larynx and subglottis are well known. We present a new pattern of defect not previously reported in the literature. METHODS: Epiglottic abnormalities at two institutions are reviewed. Cases of defects involving the lateral aspect of the epiglottis and aryepiglottic fold are identified. A literature review of known epiglottic defects is performed. RESULTS: Two children possessing lateral notch injuries at the aryepiglottic attachment to the epiglottis are described. Both children have a history of multiple laryngeal instrumentation attempts and prolonged intubation. Both have swallowing difficulties and are gastrostomy dependent. Congenital epiglottic defects include aplasia and midline bifidity, however, no lateral congenital epiglottic defects have been reported. CONCLUSION: Epiglottic defects, while rare, should be part of the differential for children with aspiration and feeding difficulties. A new pattern of defect is described and iatrogenic etiology proposed.

Surgical and Neurological Outcome of Minimally Invasive Thymectomy in Patients With Myasthenia Gravis: An Experience of 100 Cases Over 6 Years at a Tertiary Care Center in North India.

BACKGROUND: Minimally invasive thymectomy (MIT) is emerging as an effective alternative to open thymectomy in the management of patients with myasthenia gravis (MG). The primary objective of our study is to assess the surgical and neurological outcome of MIT in patients with MG. MATERIALS AND METHODS: It is a retrospective evaluation of prospectively collected data of 100 patients with MG, who underwent MIT from April 2012 to January 2018 at a tertiary care center in India. Surgical outcome was assessed for success of minimal invasive approach, conversion, perioperative morbidity, and postoperative hospital course. Neurological outcome was assessed, after at least 1 year of follow-up, according to Myasthenia Gravis Foundation of America postintervention status. Factors predicting complete stable remission (CSR) were evaluated. RESULTS: MIT was successfully performed in 98% patients with 2% conversion. There was no mortality. Overall, 10% of patients had perioperative morbidity with 5% having exacerbation of neurological symptoms. Two of these needed postoperative ventilation, whereas 3 recovered on conservative treatment. Median operative time and hospital stay were 140 minutes and 3 days, respectively. At a median follow-up of 47 months, CSR was seen in 20% with improvement in 73.3%. Overall, 63% patients were taken off steroids and patients requiring 3 drugs decreased by 70.7%. There was significant reduction in the dosage of pyridostigmine (P<0.001), prednisolone (P<0.001), and azathioprine (P=0.002) after thymectomy. Milder disease (Myasthenia Gravis Foundation of America class 1 and 2) predicted CSR on multivariate analysis. CONCLUSIONS: MIT is a safe and effective procedure that leads to improvement in neurological status with significant reduction in number and dosage of medications after thymectomy. Mild disease predicts CSR.

Community-based burden, warning signs, and risk factors of cancer using public-private partnership model in Kerala, India.

BACKGROUND: According to the GBD report published in 2016, the burden of cancer in Kerala is 135.3/100,000 population in contrast to the national average of 100/100,000 population. Cancer is a complex disease that requires broad engagement of various departments and organizations to implement a community based health promotion strategy. OBJECTIVE: To estimate the prevalence of diagnosed cancers, warning signs and selected risk factors of cancer in Niranam Panchayath of Pathanamthitta district, Kerala. METHODOLOGY: A total of 13,736 population was covered by door to door survey using a structured questionnaire. The questionnaire collected information on the sociodemographic variables of the residents, source of water supply, warning signs of cancer and details of diagnosed cancer cases. RESULTS: The mean age of the population was 39.7 ± 21 years. The prevalence of diagnosed cases of cancer in our study was 652/100,000 population. Most common type of cancer identified was Breast cancer (37.3%). The prevalence of any warning sign among the study population was 400/100,000 population. Breast lump was the common warning sign identified. Increasing age, female gender and occupational status were the factors found to be significantly associated with cancer. RECOMMENDATIONS: Community based health education to increase awareness, screening for cancers and breast self-examination in the community could help in early diagnosis and prevention at primary level. Scientific study to assess the risk factors of cancers using case control design could be done in this population along with soil and water sample testing for carcinogens.

Thymectomy in Non-Thymomatous Myasthenia Gravis: Does An RCT Solve The 75-Year-Old Controversy?

The role of thymectomy in thymomatous myasthenia gravis (MG) is unambiguous. However, its role in non-thymomatous MG (NTMG) remains debatable. The evidence till date is not robust. In this short review, we try to see the pros and cons of thymectomy in NTMG and critically appraise the evidence available till date.

Sestrin2 as Serum Protein Marker and Potential Therapeutic Target for Parkinson's Disease.

Sestrin2 (Sesn2) appears to mediate neuroprotection against Parkinson's disease (PD)-associated pathophysiology, however, the mechanism is unknown. This pilot study examines serum Sesn2 level in PD patients and older adult control and also interrogates the rescue effect of Syzygium aromaticum extract on the neurotoxicity by paraquat in neuroblastoma cells. The blood sample was collected from 36 PD patients and 54 older adult control and concentration of serum Sesn2 was measured by surface plasmon resonance and western blot. A significantly elevated level of Sesn2 (p < .0001) was observed in sera of PD group (15.96 ± 2.428 ng/µL) than the control (13.65 ± 2.125 ng/µL) which was further confirmed by western blotting. The receiver operating characteristic (ROC) curve (0.76) determined the threshold value of ≥14.58 ng/µL for differentiating PD from control. The S aromaticum extract exhibited the rescue effect from paraquat induced toxicity in SH-SY5Y cells. Further, these cells showed dose-dependent downregulation of p53, Sesn2, and phosphorylated-AMPK with concomitant increase in phosphorylated-p70S6K level than paraquat-treated cells. The differential level of Sesn2 in study subjects proposes its utility as one of the potential serum markers in PD. The ethanolic extract of S aromaticum may serve as a novel platform for management of PD-associated neurotoxicity.

Multiple Neurologic Deficits and Cognitive Decline in a Young Woman.

We present the clinicopathologic conference of a 34-year-old lady with history of facial palsy 14 years ago who developed new deficits of mononeuritis multiplex, maculopapular rash, pancytopenia, splenomegaly, lung involvement and cognitive decline rapidly over three years. Investigations revealed pancytopenia, reversal of albumin globulin ratio, mediastinal adenopathy, ANA positivity, low C3 levels with the CSF being inflammatory and MRI showing extensive hemorrhagic lesions with mass effect. She had a rapidly progressive fatal course over three years with the disease being undiagnosed. This case was presented in the annual meeting of the Indian Academy of Neurology in September 2018.