Inpatient Burden of Neurological Disorders: A Route Map for Allocation of Resources and Postgraduate Training.

Introduction There are limited data regarding the profile of inpatient neurological disorders in India. Understanding the spectrum of diseases and the profile of patients admitted in an inpatient setting will help to streamline services, allocate resources, develop management protocols, design curricula, and improve training programs of postgraduate students in neurology training. Objective The objective of this study is to study the profile of inpatient neurological disorders in 1000 consecutive patients admitted to a tertiary care neurological center. Methods Data from 1000 consecutive inpatients admitted to the Neurology Department at St. John's Medical College Hospital, Bengaluru from January 2018 to October 2018 were collected from the medical records. The data obtained from the case records were entered into a Microsoft Excel spreadsheet for descriptive analysis. Results The average age of the patients was 48 years (±18.18) and 606 of the 1000 patients were males. Strokes, including arterial and venous strokes, formed the major inpatient caseload, accounting for 48.7% of cases. Of these, 84% had ischemic arterial strokes, 7.4% had intracranial hemorrhage, and 8.4 % had cerebral sinus venous strokes; 19.3% of patients were admitted for seizures while 8.2% of patients were admitted for headache. Meningitis was diagnosed in 5.2% of patients; 4.8% of patients had central nervous system demyelinating and autoimmune diseases. A number of other diagnoses comprised less than 2.5% each and included movement disorders, peripheral nerve, spine and nerve roots disorders, neuromuscular diseases, neurodegenerative diseases, and medical and functional illness. Conclusion The most common disorders in the inpatient setting are stroke, seizure, headache, meningitis, and autoimmune/demyelinating disorders. These disorders should receive priority while planning the allocation of resources, educational curriculum, training, and teaching programs.

Intrathecal Rituximab as a Rescue Therapy in Refractory Pure CSF Positive, Non-Teratomatous Type Anti-NMDAR Encephalitis.

NMDAR antibody encephalitis is the most common autoimmune encephalitis characterized by a myriad of neuropsychiatric symptoms. It predominantly affects females and is associated with ovarian teratoma (58%). Nineteen percent do not respond to treatment and are left with serious neurological deficits. A subset of NMDAR encephalitis with antibody positivity in CSF alone without ovarian teratoma is often found to be refractory to treatment. We name them Pure CSF positive, non-teratomatous type anti-NMDAR encephalitis . We report two such cases who responded to intrathecal rituximab, to highlight a novel treatment as a rescue therapy to prevent long-term disability and improve clinical outcomes.

A Novel Mutation in KMT2B Gene Causing Childhood-onset Generalized Dystonia with Expanded Phenotype from India.

Mutations in KMT2B (lysine-specific methyltransferase 2B) gene, which is primarily involved in methylation of Histone3lys4 (H3K4), has been recently described to cause early-onset generalized progressive dystonia (DYT28) by two independent researchers. Unlike other primary dystonias, mutations in KMT2B gene is associated with additional features like dysmorphism (elongated face, bulbous nose), microcephaly, short stature, and multisystemic involvement. Herein, we describe a 13-year-old boy with early-onset, generalized, progressive complex severe dystonia, along with mild intellectual disability, dysmorphism, and dermatological manifestations associated with a novel missense variation in KMT2B gene and also expand the phenotypic spectrum of the same.

Effective Early Treatment of AChR Antibody-Positive Myasthenia Gravis with Rituximab; the Experience from a Neuroimmunology Clinic in a Developing Country.

BACKGROUND: Rituximab is reserved for treating refractory myasthenia gravis (MG) patients. Here we report our experience with rituximab in AChR antibody positive generalized MG (gMG) and impending myasthenic crisis (IMC). METHODS: This retrospective, observational study, conducted at a tertiary care, neuroimmunology clinic, analyzed the data of patients with AChR antibody positive gMG, treated with rituximab between 1st January 2016 and 30th October 2018. RESULTS: Eleven patients with AChR antibody positive gMG received rituximab. Mean age of the cohort was 50.54 ± 18.71 years with 9 males. Seven out of 11 patients received rituximab in the early stage (<2 years from onset) and had good response to treatment. Four of the 5 patients with IMC improved with rituximab alone. In the 10 patients who regularly followed up, there was a significant difference between the QMG scores at baseline and at 1, 2, 6, 12, and 18 months (P < .0001). CONCLUSION: Rituximab appears to be a potentially effective early treatment option for AChR antibody positive generalized MG and impending myasthenic crisis.

Prevalence and associations of moderately increased albuminuria in patients with type 2 diabetes in United Arab Emirates.

BACKGROUND AND AIMS: Our study sought to investigate the prevalence of moderately increased albuminuria in United Arab Emirates (UAE) nationals with type 2 diabetes, and to identify the associated factors. METHODS: This prospective cross-sectional study was conducted in two hundred and seven UAE nationals with type 2 diabetes mellitus attending the internal medicine department of a secondary care hospital. Moderately increased albuminuria was estimated in random spot urine samples and was defined as urinary albumin-to-creatinine ratio (UACR) of 3-30 mg/mmoL. Prevalence and associations of moderately increased albuminuria were evaluated. RESULTS: The study population had a mean UACR of 7.2 ± 10.2 mg/mmoL with mean eGFR of 94.5 ± 11.7 mL/min/1.73 m2. Prevalence of moderately increased albuminuria in our study population was found to be 44.0%. Multivariate logistic regression analysis showed that duration of diabetes (OR:1.72, 95% CI:1.34-2.19; p<0.001), presence of hypertension (OR:3.42, 95% CI:0.96-12.20; p=0.050) and neuropathy (OR:2.85, 95% CI:1.03-7.84; p=0.042), BMI (OR:1.08, 95% CI:1.01-1.16; p=0.019), HbA1c (OR:1.39, 95% CI:1.00-1.93; p=0.045), CRP (OR:1.10, 95% CI:1.00-1.22; p=0.035), serum creatinine (OR:1.04, 95% CI:1.02-1.06; p<0.001) and HDL-C (OR:0.10, 95% CI:0.01-0.28; p<0.001) were independently correlated with moderately increased albuminuria. Stepwise multiple linear regression analysis demonstrated that duration of diabetes, HbA1c, CRP and serum creatinine were independent predictors of UACR. CONCLUSION: We report a high prevalence of moderately increased albuminuria in UAE nationals with type 2 diabetes in a secondary care setting. Routine screening and timely management of moderately increased albuminuria in type 2 diabetes mellitus can lead to better patient outcomes.

Efficacy and safety of rituximab in multiple sclerosis: Experience from a developing country.

BACKGROUND: Rituximab is increasingly being used in treatment of multiple Sclerosis (MS) in our centers due to its easy availability, efficacy and favorable side effect profile. Here we describe experience with rituximab over a period of 4 years from three MS centers from south India. METHODS: The data of MS patients who were treated with rituximab in three MS centers at Bangalore, India, from December 2015 to December 2019 were collected and evaluated with respect to relapse rate, EDSS score and adverse events. RESULTS: Over the four-year study period 118 MS patients were evaluated, 80 of whom were on rituximab. 58 (72%) had RRMS, 15 (19%) SPMS and 7 (9%) PPMS. Most patients (89%) received rituximab at a dose of 500 mg every 6-12 months. Nine patients (11%), all with progressive MS were on 1 gm to 2 gm every 6 months. Follow up ranged from 1 year to 3 years, with a median of 2 years. 56 (97%) RRMS patients had no relapses during follow up. EDSS score improved by a score of 0.5-2.0 in 68 (85%) patients, remained same in 10 (12.5%) and worsened in 2 patients (2.5%). Most patients (91%) tolerated rituximab infusions well. There were no opportunistic infections or neoplasms. CONCLUSION: Anti B cell therapy with rituximab appears effective, safe and affordable in the treatment of MS in developing countries like India with resource limited settings.

Post herpes zoster infection neuromyelitis optica spectrum disorder.

Neuromyelitis optica is a severe autoimmune demyelinating disease; however, the trigger for autoimmunity in an individual patient is unclear. Here, we report the case of a 48 year old female presenting with longitudinally extensive transverse myelitis in the cervical region of the spinal cord two weeks post herpes zoster infection in right C6 dermatome. Though post herpetic myelitis was provisionally diagnosed, serological evaluation revealed aquaporin-4 antibody positivity, confirming the diagnosis of neuromyelitis optica spectrum disorder. Herpes zoster infection appears to have triggered the onset of autoimmunity, leading to the development of neuromyelitis optica spectrum disorder in this patient.

Ultrasound-Guided Parasternal Block Allows Optimal Pain Relief and Ventilation Improvement After a Sternal Fracture.

INTRODUCTION: Sternal fractures are a painful condition which can result in pulmonary morbidity if not treated promptly. The management of isolated fractures has changed from hospital to home-based treatment, provided other major injuries have been excluded. Pain management is the mainstay of treatment. In this case report, we describe how a parasternal block under ultrasound guidance for sternal fracture provided better analgesia thereby improving ventilation. CASE REPORT: A 26-year-old man was admitted to the emergency department following a road traffic accident. His initial evaluation revealed a radio-cubital displaced fracture at the elbow level with severe tenderness over the sternum. Chest X-ray on admission did not reveal any abnormality. On preoperative checkup he was found to have altered chest mechanics with severe pain and tenderness over the sternum. Arterial blood gas (ABG) analysis showed respiratory acidosis. Pulmonary electrical impedance tomography showed hypoventilation of anterior portions of both lungs. An ultrasound examination of the sternum showed a fractured sternum with complete disjunction. An ultrasound-guided bilateral parasternal block was performed which resulted in efficient analgesia and thereby improved his ventilation as indicated by the improvement in ABG. CONCLUSION: Timely and proper analgesia can reduce the pulmonary morbidity in sternal fractures. Of the various analgesic techniques, parasternal block under ultrasound guidance is a relatively simple, safe, and target-specific procedure that can provide efficient pain relief.

New antiplatelet agents prescribed to patients with ischemic heart disease: implications for treatment of stroke.

OPINION STATEMENT: Stroke continues to be a significant public health problem worldwide. Despite a number of clinical trials testing various therapeutic agents, we are still left with a small armamentarium of options. Aspirin, clopidogrel and combination aspirin-dipyridamole remain the mainstay for prevention of recurrent ischemic stroke. Tissue plasminogen activator (tPA) or alteplase is the sole agent used in the acute phase up to 4.5 h from the onset of stroke symptoms. A greater understanding of pathophysiologic mechanisms produced an array of acute experimental treatments including intravenous magnesium and free radical scavengers. However, they did not stand up to the scrutiny of phase III randomized clinical trials. Secondary prevention of stroke benefitted more from epidemiologic studies focusing on risk factor modifications, rather than antiplatelet or other stroke specific agents. One must ask if new treatments for stroke are exhausted. Despite the frustrations of stroke neurologists, new avenues for treatment continue to be explored. One comes from our colleagues in cardiology. Development of new medications for treating ischemic heart disease, acute or chronic, may provide opportunity to cross over into stroke. Cardiovascular trials usually encompass stroke as an outcome measure. As of yet, there have not been the data to support use of these agents in stroke. Recent medications for acute and chronic phases of ischemic heart diseases include desmoteplase, tenecteplase, tirofiban, prasugrel, and ticagrelor. Though some of these medications may fail to show a benefit in stroke patients, we feel there is always potential for a breakthrough.

Antiplatelet therapy for secondary prevention of acute coronary syndrome, transient ischemic attack, and noncardioembolic stroke in an era of cost containment.

Physicians are aware of the profound impact of oral antiplatelet therapy for secondary prevention of acute coronary syndrome (ACS), transient ischemic attack, and noncardioembolic stroke. Numerous clinical studies have compared the benefits of aspirin (ASA) alone with those of combination therapy with extended-release dipyridamole or with those of clopidogrel, with or without ASA, for secondary stroke prevention; and of ASA monotherapy compared with ASA plus clopidogrel combination therapy for secondary prevention in various ACS populations. More recently, ASA plus prasugrel has been compared with ASA plus clopidogrel in a high-risk ACS population. However, given the different treatment modalities and methods used in the various trials, it is difficult to make generalizations as to which therapy is most effective with the lowest risk of bleeding. Further complicating physician's decision making are cost considerations, particularly with the newer oral antiplatelet agents, which are considerably more expensive than ASA. This review provides a brief overview of the clinical data on each of the currently marketed oral antiplatelet agents and the available data on cost-effectiveness for the secondary prevention of ACS, transient ischemic attack, and noncardioembolic stroke.

An examination of economic outcomes associated with misdiagnosis or undertreatment of TIA.

Transient ischemic attack (TIA) is a common cerebrovascular event that is associated with a high risk for secondary stroke, possibly higher than stroke itself. Nevertheless, a TIA is often described as a "mini-stroke," implying a less serious event requiring less urgent treatment. In reality, TIA and subsequent secondary stroke are associated with a very high economic burden. Cerebrovascular disease is the most financially costly of all major disease states, while morbidity and mortality risk is highly elevated in TIA patients. TIA is also associated with a very severe quality-of-life burden, which manifests both in terms of diminished functional independence and high rates of depression. Furthermore, TIA is commonly misdiagnosed and underdiagnosed, often leading to inappropriate treatment. More than half of patients who experience TIA are undiagnosed, while those who do receive a diagnosis are often offered neither diagnostic imaging nor hospital admission. Much of the morbidity and mortality associated with TIA and secondary stroke might be avoided by the application of timely and appropriate treatment. Clinical data have shown that accelerated treatment of TIA during its acute phase is associated with an 80% reduction of secondary stroke risk for the following 3 months, the period of greatest risk.

Development of high-risk newborns ? a follow-up study from birth to one year.

We followed 55 high risk newborns from birth till one year using Trivandrum Development Screening Chart (TDSC) and Denver Development Screening Test (DDST). We also assessed their muscle tone, vision and hearing. Babies were classified into mild, moderate, and severe risk groups using a scoring system. Babies with developmental delay were categorized as having mild, moderate, or severe delay. The risk score was significantly associated with the severity of developmental delay (Plt; 0.001). Abnormalities of tone were also associated with development delay (P< 0.001).

Leptomeningeal collateral volume flow assessed by quantitative magnetic resonance angiography in large-vessel cerebrovascular disease.

BACKGROUND/PURPOSE: Leptomeningeal collateral volume flow has not been previously quantified. Quantitative magnetic resonance angiography (QMRA) can determine flow in the large vessels of the intracranial circulation. METHODOLOGY: We reviewed consecutive QMRA studies performed between December 1, 2004 and August 30, 2005, for cases showing asymmetrically higher flow in a posterior cerebral artery (PCA) just distal to the origin of the posterior communicating artery ipsilateral to a hemodynamic middle cerebral artery (MCA) or internal carotid artery lesion. The mean, range, and standard deviation (SD) of the flow rate in the PCAs, MCAs, and PCA ipsilateral-contralateral difference were calculated. Ipsilateral and contralateral PCA flow rates were compared using the Student's t-test. RESULT: Sixteen studies met selection criteria. Mean age was 52 years (range 21-79) and 9 were female. MCA flow was below QMRA detection limits in 6 studies. Mean measurable ipsilateral MCA flow reduction was 84 mL/min (range 9-147, SD 51.4). Mean ipsilateral PCA flow was 118 mL/min (range 72-206, SD 38.5) and mean contralateral PCA flow was 68 mL/min (range 35-144, SD 30.5, P < .001); mean difference was 50 mL/min (range 10-93, SD 24.3). CONCLUSION: Leptomeningeal collateral flow can be assessed with QMRA and may be substantial.

Epidemiology of ischemic and hemorrhagic stroke: incidence, prevalence, mortality, and risk factors.

The epidemiology of ischemic and hemorrhagic stroke is an ongoing exploration to identify risk factors that continue to expand with the advent of technological advancements and preventative medical practices. Identification of risk factors that can or cannot be modified is a crucial step in determining stroke risk. Many of the modifiable risk factors are well established, and specific interventions to reduce stroke risk have been established. Some risk factors are less established, and intervention to reduce risk is yet to be determined by evidence-based medicine. Data from ongoing randomized clinical trials continue to enhance our ability to prevent a first stroke.

Thoracic paravertebral block for implantable cardioverter-defibrillator and laser lead extraction.

Implantable cardioverter defibrillators (ICDs) are routinely placed in the cardiac electrophysiology laboratory. Previously, these procedures were performed in the operating room during general anesthesia. In recent years, electrophysiologists have been performing these procedures using local anesthetics in conjunction with intravenous sedation. We report a case in which thoracic paravertebral blocks with mild sedation were successful for infected ICD and laser lead extraction. Randomized, controlled trials are required to determine whether thoracic paravertebral block with sedation is more effective than local anesthesia with sedation in providing adequate anesthesia for ICD placement and extraction.

Dexmedetomidine related cardiac arrest in a patient with permanent pacemaker; a cautionary tale.

Dexmedetomidine (Precedex), an alpha-2 adrenergic receptor agonist is frequently and safely used as sedative agent during surgical procedures. We report a case of a 76-year-old woman who developed cardiac arrest from the use of dexmedetomidine during pacemaker lead extraction procedure.

Restricted magnetic resonance diffusion-weighted imaging with mass lesions presenting as acute lesions.

Diffusion-weighted imaging is useful for diagnosis of acute ischemic stroke. However, metastatic brain lesions may also demonstrate restricted diffusion on magnetic resonance imaging. We describe 4 cases (3 with metastatic neoplasms and 1 with a meningioma) with positive findings by diffusion-weighted imaging.