Search

Many physicians are pursuing additional advanced degrees, though no study has evaluated the characteristics and career choices of physicians who have pursued graduate policy degrees. We therefore searched employment and alumni data from the Harvard Kennedy School of Government in addition to publicly available information to identify physicians who graduated with a policy degree from 1964 to 2002. We identified 258 physicians with policy degrees and found that they are increasing in number, though females are underrepresented when compared to the female physician workforce; likely to pursue clinical training in specialties highly proximate to public policy challenges, with most physicians remaining medically licensed after residency; and more likely to hold nonclinical roles in academia, clinical leadership, and the private sector than in nonprofits and government. We conclude that the importance of integrating physicians with policy training throughout various organizations warrants further research into the growth, characteristics, and career choices of these physicians., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: Neuromuscular disorders could have respiratory involvement early or late into illness. Rarely, patients may present with a hypercapnic respiratory failure (with minimal motor signs) unmasking an underlying disease. There are hardly any studies which have addressed the spectrum and challenges involved in management of this subset, especially in the real-world scenario., Methods: A retrospective study comprising consecutive patients hospitalized with hypercapnic respiratory failure as the sole/dominant manifestation. The clinical-electrophysiological spectrum, phrenic conductions, diaphragm thickness, and outcomes were analyzed., Results: Twenty-seven patients were included, the mean age was 47.29 (SD 15.22) years, and the median duration of respiratory symptoms was 2 months (interquartile range [IQR] 1-4). Orthopnea was present in 23 patients (85.2%) and encephalopathy in 8 patients (29.6%). Phrenic nerve latencies and amplitudes were abnormal in 83.3% and 95.6%, respectively. Abnormal diaphragm thickness was noted in 78.5%. Based on a comprehensive electrophysiological strategy and paraclinical tests, an etiology was established in all. Reversible etiologies were identified in 17 patients (62.9%). These included myasthenia gravis (anti-AChR and MuSK), inflammatory myopathy, riboflavin transporter deficiency neuronopathy, Pompe disease, bilateral phrenic neuritis, and thyrotoxicosis. Respiratory onset motor neuron disease was diagnosed in 8 patients (29.6%). Despite diaphragmatic involvement, a functional respiratory recovery was noted at discharge (45%) and last follow-up (60%). Predictors for good outcomes included female sex, normal nerve conductions, and recent-onset respiratory symptoms., Discussion: A good functional recovery was noted in most of the patients including respiratory onset motor neuron disease. A systematic algorithmic approach helps in proper triaging, early diagnosis, and treatment. Clinical and electrodiagnostic challenges and observations from a tertiary care referral center are discussed., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Medicaid is a critical antipoverty program. Since the Affordable Care Act expanded Medicaid eligibility, millions of newly eligible people have enrolled, creating positive financial improvements for low-income families. We examined the association of Virginia's 2019 Medicaid expansion and changes in health care-related and non-health-care-related financial needs among newly eligible Medicaid enrollees. Our unique survey collected responses between December 2018 and April 2019 from newly enrolled members reporting on experiences in the year before enrollment and between July 2020 and May 2021 from members reporting on experiences one year after enrollment. The follow-up period coincided with the COVID-19 pandemic. Medicaid enrollment was associated with decreases in concern about all financial needs assessed: housing, food, monthly bills, credit card and loan payments, and health care costs. These reductions were broadly similar across demographic subgroups and across the months of the pandemic that overlapped with the follow-up period. We add to the evidence that Medicaid expansion is a social safety-net policy that could improve equity among low-income families, potentially encouraging states that have yet to expand to do so.

Background and Objectives: To determine the real-world data regarding the use of Rituximab (RTX) in neuroinflammatory disorders (NIDS) and assess the outcomes following RTX treatment., Methods: A cohort of consecutive patients with NIDS started on RTX (2018-2020) was included. The outcomes assessed were the proportion with favourable clinical response (FCR) as defined by clinical remission/ improvement using disease specific disability scores, comparative efficacy based on timing of initiation and B cell kinetics., Results: A total of 97 patients with NIDS were included. The mean age was 36.43 (±14.4) years and median duration of follow - up being 15 months (IQR 12-16). Forty patients (41.2%) were initiated on RTX "early" in disease course. Favourable clinical response at last follow-up was seen in 94.9% (n = 92). The mean change in disability score (mRS) was 1.89 (SD 1.30) (p < 0.001). RTX appeared more effective when initiated "early" with higher remission rates (75% vs. 42%, p 0.015). B cell kinetics varied across NIDS, with 73% having adequate depletion at 6 months. Minor adverse events including infusion related reactions were reported in 9%., Conclusions: RTX has a favourable efficacy and safety profile. Future prospective studies are needed to establish the optimal timing of initiation and need for disease-based dosage regimens., (Copyright © 2022 Elsevier B.V. All rights reserved.)

This paper compares health policy responses to COVID-19 in Canada, Ireland, the United Kingdom and United States of America (US) from January to November 2020, with the aim of facilitating cross-country learning. Evidence is taken from the COVID-19 Health System Response Monitor, a joint initiative of the European Observatory on Health Systems and Policies, the WHO Regional Office for Europe, and the European Commission, which has documented country responses to COVID-19 using a structured template completed by country experts. We show all countries faced common challenges during the pandemic, including difficulties in scaling-up testing capacity, implementing timely and appropriate containment measures amid much uncertainty and overcoming shortages of health and social care workers, personal protective equipment and other medical technologies. Country responses to address these issues were similar in many ways, but dissimilar in others, reflecting differences in health system organization and financing, political leadership and governance structures. In the US, lack of universal health coverage have created barriers to accessing care, while political pushback against scientific leadership has likely undermined the crisis response. Our findings highlight the importance of consistent messaging and alignment between health experts and political leadership to increase the level of compliance with public health measures, alongside the need to invest in health infrastructure and training and retaining an adequate domestic health workforce. Building on innovations in care delivery seen during the pandemic, including increased use of digital technology, can also help inform development of more resilient health systems longer-term., (Copyright © 2021. Published by Elsevier B.V.)

As of November 2020, the United States leads the world in confirmed coronavirus disease 2019 (COVID-19) cases and deaths. Over the past 10 months, the United States has experienced three peaks in new cases, with the most recent spike in November setting new records. Inaction and the lack of a scientifically informed, unified response have contributed to the sustained spread of COVID-19 in the United States. This paper describes major events and findings from the domestic response to COVID-19 from January to November 2020, including on preventing transmission, COVID-19 testing and contact tracing, ensuring sufficient physical infrastructure and healthcare workforce, paying for services, and governance. We further reflect on the public health response to-date and analyse the link between key policy decisions (e.g. closing, reopening) and COVID-19 cases in three states that are representative of the broader regions that have experienced spikes in cases. Finally, as we approach the winter months and undergo a change in national leadership, we highlight some considerations for the ongoing COVID-19 response and the broader United States healthcare system. These findings describe why the United States has failed to contain COVID-19 effectively to-date and can serve as a reference in the continued response to COVID-19 and future pandemics.

Therapeutic plasma exchange (TPE) is an effective and affordable treatment option in most parts of Southeast Asia (SEA). In 2018, the SEA TPE Consortium (SEATPEC) was established, consisting of regional neurologists working to improve outcome of various autoimmune neurological diseases. We proposed an immunotherapeutic guideline prioritizing TPE for this region. We reviewed disease burden, evidence-based treatment options, and major guidelines for common autoimmune neurological disorders seen in SEA. A modified treatment algorithm based on consensus agreement by key-opinion leaders was proposed. Autoimmune antibody diagnostic testing through collaboration with accredited laboratories was established. Choice of first-line immunotherapies (IVIg/corticosteroid/TPE) is based on available evidence, clinicians' experience, contraindications, local availability, and affordability. TPE could be chosen as first-line therapy for GBS, CIDP, MG (acute/short term), IgG, A paraproteinemic neuropathy, and NMDAR encephalitis. Treatment is stopped for acute monophasic conditions such as GBS and ADEM following satisfactory outcome. For chronic immune disorders, a therapy taper or long-term maintenance therapy is recommended depending on the defined clinical state. TPE as second-line treatment is indicated for IVIg or corticosteroids refractory cases of ADEM, NMOSD (acute), MG, and NMDAR/LGI1/CASPR2/Hashimoto's encephalitis. With better diagnosis, treatment initiation with TPE is a sustainable and effective immunotherapy for autoimmune neurological diseases in SEA., Competing Interests: Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2021.)

Objectives: To study the clinical profile and outcomes of patients with paraproteinemic neuropathy (PPN) and to explore the utility of nerve conduction studies (NCSs) to differentiate between the demyelinating subtypes., Methods: We did a retrospective analysis of patients diagnosed with PPN between January 2010 and December 2019 in an inpatient setting. The study population consisted of patients above 16 years of age presenting with clinical features suggestive of chronic peripheral neuropathy and on evaluation was found to have PPN., Results: A total of 74 patients were identified. The patients were predominantly in the 6th decade, and the majority were males. The subtypes of PPN were monoclonal gammopathy of undetermined significance (MGUS) (45.9%), POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) (24.3%), solitary plasmacytoma (17.6%), multiple myeloma (8.1%), and AL amyloidosis (4.1%). There are specific features on NCS which can help in identifying POEMS syndrome and IgM MGUS. The majority of patients with PPN tend to stabilize or improve with treatment; however, many have a severe residual disability. New terminology and classification of these entities as 'monoclonal gammopathies of neurological significance' can aid in early diagnosis and the development of effective treatment, to prevent residual disability., Conclusion: PPN has a heterogeneous spectrum of clinical, biochemical, and electrophysiological features. NCS can help distinguish POEMS syndrome and IgM MGUS from other demyelinating subtypes.

Background: Guillain-Barre syndrome can be electrophysiologically classified into demyelinating and axonal subtypes and nerve conduction studies remain the mainstay in electrodiagnosis. Accurate electrodiagnosis has both therapeutic and prognostic significance and different criteria sets have been proposed for classification., Objectives: To electrophysiologically classify GBS patients into AIDP and axonal subtypes according to various published criteria (Cornblath, 1990: Hadden, 1998, Rajabally, 2015), investigate if serial NCS changes the classification, and to identify additional parameters which may support the electrodiagnosis., Materials and Methods: In a retrospective study, we included all patients aged 15 to 80 years, admitted with a diagnosis of GBS between August 2015 and July 2017, who had at least two serial NCS. The various published criteria were applied to the two serial NCS and subtype classification along with diagnostic shifts on serial NCS were ascertained., Results: At the first test, the established criteria gave a yield of 45.2% to 71% for AIDP, while 29% to 54.8% of patients were classified as axonal GBS. In the second study, there was a change in electrodiagnosis, ranging from 9.6% to 16.1%. The resolution of reversible conduction failure and misclassification of subtypes were the major reason for diagnostic shifts. Sural sparing pattern, facial nerve dysfunction, abnormal blink reflex, and phrenic nerve dysfunction were more common in AIDP., Conclusions: Serial nerve conduction studies allow an accurate electrodiagnosis of GBS subtypes, which has both therapeutic and prognostic implications. Also, the use of additional parameters such as blink reflex facial and phrenic nerve conduction may supplement routine NCS., Competing Interests: None

Limited literature is available on stimulus induced after discharges (SIAD) in patients with peripheral nerve hyperexcitability (PNH). The aim of the study was to examine the diagnostic utility of SIAD in the diagnosis and monitoring of primary PNH disorders. In this retrospective study, we studied 26 patients who were admitted with a diagnosis of primary PNH to the department of Neurology from January 2013 to April 2019. Their clinical profile, immunological characteristics were extracted from the database and nerve conduction studies were relooked for the presence of SIAD. 76% of patients in the primary PNH cohort had SIAD with 90% of them being voltage-gated potassium channel complex antibody positive; predominantly against contactin-associated protein-2 antigen and rest being paraneoplastic. There was also resolution of SIAD following treatment indicating reversible hyperexcitability. SIAD is a sensitive marker for Primary PNH syndrome with monitoring and diagnostic implications., (© 2020 Peripheral Nerve Society.)

Introduction  Infection with Burkholderia pseudomallei , a gram-negative bacterium found in soil and surface water, is termed melioidosis and is commonly reported to occur in Southeast Asia and Northern Australia, where it is endemic. It is being increasingly reported in India, and transmission occurs through inhalation, inoculation, and ingestion. The neuroparenchyma, the adjacent soft tissue, and bone are known to be affected in both the acute and chronic disease forms. Involvement of these structures is rare but causes significant mortality and morbidity. Material and Methods  Eighteen culture-proven cases of neuromelioidosis were identified between January 2008 and December 2019. The patients were retrospectively identified via search of the hospital's electronic database. Results  Cranial disease was in the form of parenchymal abscesses ( n = 4), cerebritis/encephalitis ( n = 5), and extradural ( n = 4) and dural disease ( n = 1). Acute myelitis ( n = 1) and spondylodiscitis ( n = 3) were seen in the spinal disease form. Neuroparenchymal involvement ranged from cerebritis/encephalitis to early and mature parenchymal abscesses. Extradural involvement was in the form of extradural abscesses and/or thick irregular enhancement in the extradural region. Early diagnosis and initiation of appropriate therapy had favorable outcomes in 15 out of 18 patients. Two patients with parenchymal abscesses and one with myelitis succumbed to the illness. Conclusion  Neuromelioidosis is a rare manifestation of melioidosis with significant morbidity and mortality, necessitating a high index of clinical suspicion, especially if there has been travel to endemic regions. Imaging plays a key role in facilitating early diagnosis and initiation of therapy., Competing Interests: Conflict of InterestFinancial Support and Sponsorship The authors declare conflict of interest. Nil., (Indian Radiological Association. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

Background: Paraneoplastic Neurological Syndromes (PNSs) are a heterogeneous group of immune-mediated disorders that often precede tumor diagnosis. There are few systematic studies on the spectrum and follow-up of PNSs., Objective: To analyze the clinical spectrum, associated tumors, antibody profile, outcomes, and prognostic predictors in a cohort of PNSs admitted in a tertiary care center., Methods: This retrospective study included 97 patients (2008-2019). PNSs were further classified as "classical," "nonclassical," "definite," and "possible." Clinical profile, diagnostic strategies, therapeutic options, and predictors of outcomes were identified., Results: The median age was 54 years (range 17-81). Thirty-nine (40.2%) had classical PNS, and 58 (59.8%) had nonclassical PNS, 74 (76.3%) had "Definite" PNS while 23 (23.7%) had "Possible" PNS. Cerebellar degeneration, peripheral neuropathy, and encephalopathy were the three most common neurological syndromes. Tumors were diagnosed in 66 (68%) patients; Lung cancer was the most common primary tumor. Antibodies were positive in 52 (53.6%). Anti-Yo antibody and anti-Ma2 antibody were the most common antibodies. The majority (57.7%) received immunotherapy in addition to definitive treatment for the tumor. A good outcome was seen in 53 (54.6%). Factors associated with good outcome were: early diagnosis, mRS <3 at presentation, absence of metastatic disease, and adjuvant immunotherapy., Conclusion: A high index of clinical suspicion is essential for early diagnosis and prompt management of PNS, especially the nonclassical syndromes. Multimodality diagnostic imaging techniques and antibody profiling play a crucial role in the diagnosis. A favorable prognosis can be expected with the judicious use of immunotherapy and definitive treatment of malignancy., Competing Interests: There are no conflicts of interest., (Copyright: © 2006 - 2021 Annals of Indian Academy of Neurology.)

Background and Purpose: Studies looking at seasonal variation on cerebral venous thrombosis (CVT) are few with conflicting conclusions. In this region-specific study, we looked for climatic influence and seasonal trends on the incidence of CVT., Methods: Imaging proven adult CVT cases treated over a period of 18 years from a specific geographical location with similar seasons and climatic conditions were studied. Metrological parameters prepared using 30 years of data was used. Quantum geographical information system (QGIS software) and SPSS v 22 were used for patient plotting and analysis., Results: Total of 970 cases were studied. The incidence was significantly higher in summer 411 (42.3%) compared with autumn 317 (32.7%) and winter 242 (25.05); P = 0.038. This trend was consistent across all the 18 years in time series analysis. Mean age was 33.5 years (range 18-88 years). A significant majority 673 (69.4%) were below 40 years of age; P = 0.012. Females constituted 394 (40.6%) of cases. Postpartum CVT cases constituted 237 (30%). Interaction analysis showed younger age (<40 years) were more vulnerable for CVT in summer; P = 0.009. There was no seasonal influence on postpartum CVT. Apart for a weak positive correlation between rain fall ( r = 0.18, P < 0.01); humidity and cloud cover was not influencing the incidence., Conclusions: Higher ambient temperatures were consistently associated with higher incidence of CVT. This is the largest region-specific study on CVT in the world. These results may be applicable to other regions with similar climatic conditions., Competing Interests: There are no conflicts of interest., (Copyright: © 2006 - 2019 Annals of Indian Academy of Neurology.)

Context: Neurosarcoidosis (NS) is a chronic disease with a diverse clinical spectrum, therapeutic response, and outcome. There is scarce literature from our country regarding the same., Aims: The aim of this study was to evaluate the clinical spectrum, therapeutic responses, and outcomes of NS in an Indian cohort., Settings and Design: In a cross-sectional study, we included all patients with NS treated at a quaternary care teaching hospital in India from January 2007 to October 2019., Subjects and Methods: Patients older than 18 years of age fulfilling the diagnostic criteria for NS from the Neurosarcoidosis Consortium Consensus Group were included in the study. The therapeutic response and the degree of disability at last follow-up were assessed., Results: We identified 48 patients, among them 3 were categorized as having definite NS, 30 probable NS, and 15 possible NS. Cranial neuropathy was the most common presentation (47.9%), followed by myelopathy (25%). Systemic involvement was identified in 95.83% and mediastinal lymph nodes were the most common site. Clinical improvement was seen in 65.8% and disease stabilized in 28.9%, while 5.26% deteriorated. Fifty percent recovered without any residual disability, while 26.3% had minor and 23.7% had major residual sequelae., Conclusions: NS is a diverse illness, with a heterogeneous spectrum of clinical presentation, treatment response, and outcome. Cranial neuropathy is the most common presenting feature and has a good prognosis while myelopathy has an unfavorable prognosis. Meningeal and brain parenchymal disease is difficult to diagnose accurately unless systemic involvement is present. The diagnosis of NS should be clinically suspected in the appropriate clinical setting, the presence of systemic involvement should be investigated, and histologic confirmation should be attempted., Competing Interests: There are no conflicts of interest., (Copyright: © 2006 - 2020 Annals of Indian Academy of Neurology.)

Background: Tolosa-Hunt Syndrome (THS) is one of the causes of cavernous sinus syndrome causing painful ophthalmoplegia. Literature on long-term outcome of this rare condition is scarce., Aims and Objectives: The aim is to study the recurrence and role of steroid-sparing agents in THS., Methodology: All cases of THS treated at a tertiary-level teaching hospital during a 10-year period were studied. Clinical and radiological profile, response to treatment and recurrences were noted., Results: A total of 44 cases were studied. The mean age was 49.5 years, Males constituted 23/44 (52%). The first symptom was pain in 90%. Ptosis with ophthalmoplegia was the most common deficit 29/44 (66%). Lesions confined to cavernous sinus 27/44 (61%) was the most frequent magnetic resonance imaging finding. All patients received steroids as the initial treatment and 15/44 (34%) received steroid-sparing agents. Follow-up ranged from 6 to 120 months (Mean 39 months). Two patients had alternative diagnosis of leptomeningeal malignancy and hypertrophic pachymeningitis on follow-up. Recurrences occurred in 18/37 (48.6%). Time for recurrence varied from 8 months to 7 years. (Mean 18 months). No clinical or radiological predictors for recurrence were identified. Patients who received steroid-sparing agents had a significantly lower recurrence 3/15 (20%) versus 14/26 (53.8%)P < 0.034., Conclusions: Around 50% of patients with THS can have recurrence. Steroid-sparing agents appear to prevent recurrence. A prospective multicenter randomized controlled trial may help to evaluate the risk and benefits of steroid-sparing therapy and to identify any possible predictors for recurrence., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Annals of Indian Academy of Neurology.)

Purpose: Progressive language dysfunction due to a selective neurodegeneration of the language networks is called primary progressive aphasia (PPA). However, demographic data on PPA is limited. In this study from India, we determined the prevalence and clinical profile of patients presenting with PPA and its subtypes. Method: Patients who were admitted to the neurosciences department during the period between January 2012 and December 2016 were screened, and patients who presented with slowly progressive aphasia for at least 2 years without other significant cognitive or behavioural symptoms and preservation of daily living activities were included. Patients had to fulfil the international consensus group criteria for PPA. All patients were evaluated with the mini-mental status examination (MMSE) and Strub and Black battery for neuropsychological testing. The language was tested using the progressive aphasia language scale (PALS). Result: During the study period from January 2012 to December 2016, 23 patients fulfilled the international consensus criteria for PPA. Of these, 16 (69.6%) patients were diagnosed with PPA-G, 6 (26%) patients had PPA-S and 1 (4.4%) patient had PPA-L. Conclusion : PPA is not an uncommon entity in India and the most common subtype in this study was PPA-G.

Introduction: There is scarce literature regarding the clinical course, comorbidities and long-term outcomes after myasthenic crisis (MC). The natural history of myasthenia gravis (MG) in this subset remains uncertain., Methods: The study included a cohort admitted with MC (2007-2017) in a tertiary care hospital. The comorbidities, outcomes after discharge, and prognostic factors were analyzed., Results: Sixty-two patients (89 episodes of MC) were included. Demographic data was comparable between the early- (<50 years) and late-onset (≥50 years) groups. Comorbidities included stress cardiomyopathy (14.5%), arrhythmias (6.4%), neuropathy (17.7%), pancytopenia (12.9%), encephalopathy (11.2%), neuromyotonia (4.8%), myelopathy (3.2%), and myositis (3.2%). Pulmonary embolism ( P < 0.008), dysautonomia ( P < 0.002), sepsis ( P < 0.008), neuropathy ( P < 0.002), and phrenic dysfunction ( P < 0.016) were associated with prolonged ventilation. Majority of the patients (42, 67.7%) had a favorable outcome (disease status) as defined by remission/minimal manifestations at the time of last follow-up (median 36 months, IQR 15-66). Persistent bulbar weakness ( P < 0.001), neuropsychiatric illness ( P < 0.001), and comorbidities ( P < 0.017) were associated with refractory MG. Eighteen patients (29%) had recurrent crisis. Eleven patients succumbed in the cohort. The main predictors of mortality were tumor progression ( P < 0.001) and cardiac illness ( P < 0.004)., Discussion: A comprehensive treatment approach in MC will translate to good short- and long-term outcomes. The main cornerstones of therapy will include (1) Identification of refractory MG with the implementation of phenotype-based therapy; (2) Addressing comorbidities including cardiac autonomic neuropathy, bulbar weakness, phrenic dysfunction; and (3) Meticulous tumor surveillance., Competing Interests: There are no conflicts of interest., (Copyright: © 2006 - 2019 Annals of Indian Academy of Neurology.)

Purpose: The brainstem plays an important role in the control of micturition, and brainstem strokes are known to present with micturition dysfunction. Micturition dysfunction in cases of lateral medullary infarction (LMI) is uncommon, but often manifests as urinary retention. In this study, we investigated the neuro-anatomical correlates of urinary retention in patients with LMI., Methods: This was a hospital-based retrospective study conducted in the neurology unit of a quaternary-level teaching hospital. Inpatient records from January 2008 to May 2018 were searched using a computerized database. Cases of isolated LMI were identified and those with micturition dysfunction were reviewed. MRI brain images of all patients were viewed, and individual lesions were mapped onto the Montreal Neurological Institute (MNI) space manually using MRIcron. Nonparametric mapping toolbox software was used for voxel-based lesion-symptom analysis. The Liebermeister test was used for statistical analysis, and the resultant statistical map was displayed on the MNI template using MRIcron., Results: During the study period, 31 patients with isolated LMI were identified. Their mean age was 48 years and 28 (90%) were male. Six of these patients (19%) developed micturition dysfunction. All 6 patients had urinary retention and 1 patient each had urge incontinence and overflow incontinence. In patients with LMI, the lateral tegmentum of the medulla showed a significant association with urinary retention., Conclusion: In patients with isolated LMI, we postulate that disruption of the descending pathway from the pontine micturition centre to the sacral spinal cord at the level of the lateral tegmentum results in urinary retention.

Several extrinsic factors affect precision grip task variability. Presence of moisture in glabrous skin can regulate the impact of extrinsic factors. Consequently, wetness may influence the precision grip task variability per se by regulating its force, rate and time parameters. This study aims to examine the influence of age, coefficient of skin friction (CF) and object weight (extrinsic factors) on precision grip task variability in dry skin condition (DSC) and very wet skin conditions (VWSC). Eighty healthy subjects performed precision grip task with four different weights (1.3N, 1.4N, 1.5N, and 1.7N) sequentially in DSC and VWSC. Simple and multiple linear regression analysis were performed to estimate the independent and combined effect of extrinsic factors on precision grip parameters. Our results show that the extent of variability caused by the extrinsic factors on precision grip task significantly reduced when objects were held with VWSC than DSC. Wetting of the skin also decreased standard deviation and coefficient of variation of friction. The frictional range of individuals was widespread in DSC (0.62-3.42) while VWSC brought it to a closer range (0.77-1.64). Our findings suggest that wetness of skin reduces precision grip task variability, and further knowledge on this may help in designing precision grip as a quantitative screening tool for patients with hand dysfunction., (Copyright © 2019 Elsevier B.V. All rights reserved.)

In conjunction with the third regional Southeast Asian (SEA) therapeutic plasma exchange (TPE) conference in Kuala Lumpur, Malaysia, 25 clinicians and researchers from SEA and South Asian countries attended the inaugural strategy meeting for the establishment of a regional TPE consortium for neurological disorders. The primary objective was to establish regional collaboration to improve delivery of TPE services in SEA. A pre-meeting survey was conducted to gather insights on disease spectrum, contextual practice challenges, and the need for a regional TPE consensus. Challenges identified include limited healthcare funding in support of diagnostic workup, TPE therapy, as well as development of clinical infrastructure and expertise capacity building. There was favorable interest in developing a working plan contextualized to this region. Strategies to overcome challenges were discussed. This included the need for a comprehensive referral system and network of regional TPE centers suited to local needs, supported by innovative TPE delivery programs., (© 2019 International Society for Apheresis, Japanese Society for Apheresis, and Japanese Society for Dialysis Therapy.)

Cavernous sinuses are paired interconnected venous plexuses situated in the floor of the middle cranial fossa on either side of the sella turcica and sphenoid sinus. They are lined by dura mater and consist of multiple venous channels within. The cavernous sinuses are intimately related to the internal carotid artery and its associated sympathetic plexus, the oculomotor nerve, the trochlear nerve, the abducens nerve, and the ophthalmic nerve. Cavernous sinuses are connected to the orbit, the pterygopalatine fossa, the infratemporal fossa, the nasopharynx, and the posterior cranial fossa by various foramina, fissures, and canals in the skull base. A multitude of structures in close relation to the cavernous sinus give rise to a myriad of possible pathologic conditions that can be broadly classified into (a) neoplastic, (b) vascular, (c) infective or inflammatory, or (d) miscellaneous lesions. These pathologic conditions can have overlapping clinical manifestations. Hence, imaging plays a crucial role in identifying the disease, assessing its extent, providing a pertinent differential diagnosis to guide further management, and suggesting a site or route for biopsy. MRI is the modality of choice to depict the cavernous sinuses, with CT and digital subtraction angiography playing supplementary roles in certain situations. In this article, the cavernous sinus lesions encountered in our institution during a 10-year period are reviewed. The purpose of the article is to (a) describe the anatomy of the cavernous sinus; (b) demonstrate the multimodality imaging spectrum of a wide variety of pathologic conditions involving the cavernous sinus, correlating with the histopathologic findings; (c) highlight important imaging clues for differential diagnosis; and (d) help the reader overcome potential pitfalls in interpretation. Online supplemental material is available for this article. © RSNA, 2019.

Introduction: Distal sensory polyneuropathy (DSP) is one of the most common neurological disorders. Although several studies have studied the role of the neurological examination in DSP, there are only limited studies on the utility of timed vibration sense (VBS) and joint position sense (JPS) testing in the diagnosis of DSP., Objectives: The objective is to study the utility of timed VBS testing and JPS testing at the great toe in clinical detection of DSP., Methods: This study was prospectively conducted in the neurology department of a tertiary care hospital in India. Patients with DSP referred to the electrophysiology laboratory from August 2017 to December 2017 were screened. Patients with symptomatic DSP which was confirmed by electrophysiological studies were taken as cases and normal participants with no symptoms or electrophysiological findings suggestive of DSP served as controls., Results: We studied 127 patients and 194 controls. The mean age of the patients was 48.7 (14.5) years in the patient group and 39.7 (14.5) years in the control group. The male: female ratio was 77/50 in the patient group and 112/82 in the control group. Abnormal clinical examination was found in 95% of the patients with DSP. The most common abnormal examination components were impaired ankle reflexes (70%), vibration (85%), and JPS (39.6%) sensation. Using the receiver operating characteristic curve for the diagnosis of DSP, a vibratory response lasting <8 s at the great toe had a sensitivity of 85% and specificity of 42.8%. For JPS testing at the great toe, obtaining two or more incorrect responses had a sensitivity of 33% and specificity of 87.6%., Conclusion: VBS testing was more sensitive and JPS testing was more specific in making a clinical diagnosis of DSP. For timed VBS, duration of >8 s at the great toe was a useful test to rule out DSP, and for JPS testing at the great toe, obtaining two or more incorrect responses was a useful test in ruling in the diagnosis of DSP., Competing Interests: There are no conflicts of interest.

Objectives: To investigate the mutational spectrum and genotype-phenotype correlation in Indian patients with congenital myasthenic syndrome (CMS), using next-generation sequencing of 5 genes., Methods: CHRNE, COLQ, DOK7, RAPSN, and GFPT1 were sequenced in 25 affected patients., Results: We found clinically significant variants in 18 patients, of which variants in CHRNE were the most common, and 9 were novel. A common pathogenic COLQ variant was also detected in 4 patients with isolated limb-girdle congenital myasthenia., Conclusions: Targeted screening of 5 genes is an effective alternate test for CMS, and an affordable one even in a developing country such as India. In addition, we recommend that patients with isolated limb-girdle congenital myasthenia be screened initially for the common COLQ pathogenic variant. This study throws the first light on the genetic landscape of CMSs in India.

Introduction: The Babinski sign is one of the most important clinical signs for detecting corticospinal tract (CST) lesions. However, due to variations in testing and interpretation, it has been associated with low interobserver agreement rates. In this study, the diagnostic value of finger and foot tapping in detecting CST lesions was compared to that of the Babinski sign., Materials and Methods: Three groups of participants were recruited: Group 1 - individuals having CST lesions diagnosed on the basis of clinical examination as well as neuroimaging; group 2 - individuals having a non-CST neurological illness; group 3 - normal individuals who were relatives of the patients recruited. The sensitivity and specificity of finger tapping, foot tapping, and Babinski sign were calculated., Results: 375 patients, 125 in each group, were included. The overall sensitivity for Babinski sign was 49.6% and specificity was 85.8%. The overall sensitivity for finger and foot tapping was 79.5% and specificity was 88.4%. The interobserver agreement between the medical students and the neurologist was greater for finger and foot tapping (Kappa = 0.83) when compared to Babinski sign (Kappa = 0.45)., Conclusion: Finger and foot tapping is a valid and reliable test in the clinical diagnosis of corticospinal lesions. The reliability and validity of Babinski sign is variable and thus its ability to diagnose the manifestations of corticospinal lesions is less when compared to the finger and foot tapping test., Competing Interests: There are no conflicts of interest

Aim: To determine the accuracy of transcranial color-coded Doppler sonography (TCCS) in the evaluation of cerebral arterial system in patients with ischemic stroke attending a tertiary care hospital in South India., Objectives: (1) To describe the topographical distribution of atherosclerotic lesions in the cerebral circulation in patients presenting with ischemic stroke from the Indian subcontinent and (2) to determine the accuracy of TCCS for detection and quantification of intracranial stenoses in various segments of the intracerebral arterial system in comparison with magnetic resonance angiography (MRA)., Materials and Methods: The demographic profile and risk factors of consecutive patients who presented to neurology outpatient department with cerebral ischemia and scheduled for MRA were determined. These patients had undergone neck Doppler, TCCS, and MRA. The agreement between the MRA and TCCS was assessed using kappa statistics. The sensitivity, specificity, and positive and negative predictive values of TCCS as compared to MRA were calculated., Results: Ninety patients were included in the final analysis. Intracranial atherosclerosis was found in 35.6% of cases. The agreement between TCCS and MRA in detecting lesions for the different arterial segments in the intracranial circulation was 0.83 for anterior cerebral artery (ACA), 0.66 for M1 segment of middle cerebral artery (MCA), 0.45 for M2 segment of MCA, 0.86 for terminal internal carotid artery (TICA), 0.46 for posterior cerebral artery (PCA), and 0.81 for vertebral artery (VA). The sensitivity for the detection of hemodynamically significant arterial lesions in different vascular segments was 100%, 70%, 33.3%, 90.9%, 33.3%, and 72.7% for ACA, M1, M2, TICA, PCA, and VA, respectively., Conclusion: Intracranial atherosclerosis was found to be the predominant distribution of cerebral atherosclerosis. TCCS is a safe method for evaluation of proximal basal cerebral arteries in the intracranial circulation with relatively better sensitivity in the anterior circulation., Competing Interests: There are no conflicts of interest.

IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease. This disease may be associated with elevated serum and tissue IgG4 levels. Early treatment prevents fibrosis and organ damage. We retrospectively studied the clinicopathologic correlation and outcome of treatment in IgG4-RD. This single-center retrospective study was done using electronic records of patients subjected to assay of serum IgG4 levels in our laboratory by nephelometry. There were 473 patients with suspected IgG4-RD. Of them, 41 patients fulfilled comprehensive diagnostic criteria for IgG4-RD and 432 had diseases other than IgG4-RD. Clinical and histopathological data including tissue IgG4/IgG ratio, other relevant laboratory findings as well as management data of 41 patients with IgG4-RD were analyzed. There were 29 males and 12 females with mean age of 44.1 ± 2.19 years. Thirteen patients had definite, 19 had probable and 9 had possible IgG4-RD. Male predominance, multiple organ involvement and IgG4 responder Index were significantly higher in definite IgG4-RD as compared to probable and possible IgG4-RD. Serum IgG4 level was elevated in 37 patients (90.2%). Glucocorticoids were used in 35 patients (85.4%) and second-line immunosuppressive agent in 23 patients (65.7%). Of the 21 patients on follow-up, 19 (90.7%) had clinical improvement at the first follow-up visit. Nine (90%) out of the ten patients who were assessed by IgG4 responder index, also had shown improved score with treatment. Patients with IgG4-RD in our series showed favorable responses to treatment with glucocorticoids and addition of steroid sparing immunosuppressive agents (mainly mycophenolate mofetil) helped successful tapering of steroids, while maintaining the improvement.

Background: Autoimmune autonomic dysfunction is described in Myasthenia Gravis. In myasthenic crisis, the spectrum of autonomic dysfunction is hitherto uncharacterized., Objective: The objective of this study is to describe the spectrum of autonomic dysfunction in myasthenic crises using the composite autonomic symptom scale 31 (COMPASS 31) autonomic symptom questionnaire and power spectral analysis of heart rate variability (HRV), which is a simple way of estimating general autonomic dysfunction., Methods: Adult patients with myasthenic crisis from January 1, 2014 to March 15, 2015, were prospectively included in this study. The COMPASS 31 questionnaire for symptoms of autonomic dysfunction and power spectral analysis of HRV were assessed. These were compared with the patient's demographic and clinical parameters and with previous literature. IRB approval was obtained., Results: Sixteen patients were included (M:F 3:1). 15/16 patents (93%) had autonomic dysfunction on COMPASS 31 questionnaire. The domains of involvement were gastrointestinal (80%), orthostatic (67.7%), pupillomotor (67.7%); sudomotor (33.3%), and vasomotor (13.3%). Parasympathetic dysfunction predominance was suggested by the symptom profile. HRV analysis showed a low frequency (LF) spectral shift suggesting slowed parasympathetic responsiveness (LF normalized unit (nu): high frequency [HF] nu mean 8.35, standard deviation ± 5.4, 95% confidence interval 2.2-12.5), which significantly exceeded the mean LF nu: HF nu ratios of the majority of previously reported noncrises myasthenic populations., Conclusions: Myasthenic crisis has autonomic dysfunction involving multiple organ systems. Increased latency of parasympathetic reflexes is suggested. A comprehensive management protocol addressing different autonomic domains is required for holistic patient care., Competing Interests: There are no conflicts of interest.

Objective: Motor impairments related to hand function are common symptoms in patients with movement disorders, such as Parkinson's disease (PD) and focal hand dystonia (FHD). However, hand dysfunction has not been quantitatively assessed as a clinical tool for screening patient groups from healthy controls (HCs). The aim of our study was 1) to quantitatively assess hand dysfunction in patients with PD and FHD and its usefulness as a screening tool 2) to grade disease severity in PD and FHD based on hand dysfunction., Methods: The current case-control study included HCs ( n = 50) and patients with known history of PD ( n = 25) or FHD ( n = 16). Hand function was assessed by a precision grip task while participants lifted objects of 1.3 N and 1.7 N under dry skin conditions, followed by very wet skin conditions (VWSCs). Receiver operating characteristic and summative scoring analyses were performed., Results: In PD, the combination of loading phase duration and lifting phase duration at quantitative cutoffs of 0.36 and 0.74 seconds identified 21/25 patients as diseased and 49/50 subjects as HCs with 1.7 N under VWSCs. In PD, 5/21 was graded as "mild" and 16/21 as "moderate cases." In FHD, slip force at a cutoff of 1.2 N identified 13/16 patients as diseased and 41/50 subjects as HC with 1.7 N under VWSCs, but disease severity could not be graded., Conclusion: Our results demonstrate the use of precision grip task as an important clinical tool in assessment of hand dysfunction in movement disorder patients. Use of quantitative cutoffs may improve diagnostic accuracy and serve as a valuable adjunct to existing clinical assessment methods.

Objectives: There are limited data regarding long-term follow-up and therapeutic outcomes in Sjogren's syndrome (SS)-associated peripheral neuropathy. In this study, we aim to study the clinical, electrophysiological spectrum and therapeutic responses among the different subtypes of SS-associated neuropathy. The predictors of suboptimal treatment response will be identified., Methods: The study included a retrospective cohort of patients with SS-associated neuropathy between January 2012 and November 2015. Baseline clinical, laboratory, electrophysiological data and details of treatment were noted. Therapeutic outcomes were assessed at follow-up and compared among the different subtypes. Prognostic predictors were determined using logistic regression analysis., Results: Fifty-four patients were included in the study. Sensory ataxic neuropathy (17, including 9 with sensory ganglionopathy) and radiculoneuropathy (11) were the main subtypes. Notable atypical presentations included acute neuropathies, pure motor neuropathies, and hypertrophic neuropathy. Concomitant autoimmune disorders were present in 24 (44.4%) patients. Most presentations were subacute-chronic (51, 94.4%). Minor salivary gland biopsy had a higher yield compared to serological markers (81.5 vs. 44.4%). Sensory ataxic neuropathy was associated with greater severity and autonomic dysfunction. Improvement was noted in 33 (61%) patients. Cranial neuropathy and radiculoneuropathy subtypes were associated with the best treatment responses. Chronicity, orthostatic hypotension, baseline severity, and marked axonopathy (nerve biopsy) were predictive of a suboptimal therapeutic response., Conclusions: The study highlights the heterogeneous spectrum, atypical presentations, and differential therapeutic responses. SS-associated neuropathy remains underdiagnosed. Early diagnosis and prompt initiation of immunotherapy before worsening axonal degeneration is paramount. SS-associated neuropathy need not necessarily be associated with a poor prognosis., Competing Interests: There are no conflicts of interest.

Introduction: In chronic inflammatory demyelinating polyneuropathy (CIDP), emphasis has been on motor disabilities, and autonomic dysfunction in these patients has not been addressed systematically., Materials and Methods: Autonomic function was prospectively analyzed in 38 patients with CIDP. Quantitative autonomic function testing was done using Finometer ® PRO and severity of adrenergic and cardiovagal dysfunction graded according to composite autonomic severity score and sudomotor dysfunction assessed using sympathetic skin response., Results: Thirty-four (89%) patients had features of autonomic dysfunction. Thirty-three (86%) patients had cardiovagal dysfunction, 21 (55%) had adrenergic dysfunction, and 24 (63%) had sudomotor dysfunction. Autonomic dysfunction was mild to moderate in the majority (86%)., Conclusions: Autonomic dysfunction in CIDP is underreported and potentially amenable to therapy. Our cohort had a high proportion of adrenergic dysfunction compared to previous studies., Competing Interests: There are no conflicts of interest.

Visual impairment can complicate cerebral venous thrombosis (CVT). Here, we describe the various pathophysiological mechanisms and treatments available. A retrospective chart review of all patients treated for CVT in a large quaternary teaching hospital was done, and cases with visual impairment due to CVT were identified. The various mechanisms causing visual impairment in CVT were (1) raised intracranial pressure (ICP) caused by venous thrombosis without venous infarcts resulting in a benign intracranial hypertension-like presentation of CVT, (2) venous infarcts involving the occipital cortex, (3) raised ICP following the development of a secondary dural arteriovenous (AV) fistula, and (4) arterial occipital infarcts due to posterior cerebral artery compression secondary to herniation in large venous infarcts. Apart from using systemic anticoagulants to attempt recanalization and drugs with carbonic anhydrase inhibitor activity to reduce the ICPs, treatment modalities employed to save vision were (1) recanalization by local thrombolysis, stenting, or mechanical devices; (2) cerebrospinal fluid diversion procedures such as theco-periotoneal shunting; (3) optic nerve sheath fenestration; and (4) specific treatment for conditions such as dural AV fistula occurring as a late complication. CVT can cause visual impairment through different pathophysiological mechanisms. Depending on the mechanism, treatment strategies need to be tailored. Furthermore, very close monitoring is needed both in the acute and in the follow-up period, as new pathophysiological mechanisms can arise, compromising the vision. This may require a different treatment approach. Literature on this aspect of CVT is lacking., Competing Interests: There are no conflicts of interest.

Background: There are sparse data on the recovery of sympathetic skin response (SSR) following decompressive surgery in patients with cervical spondylotic myelopathy (CSM). We designed a study to assess SSR in patients with moderate and severe (Nurick grades 3, 4 and 5) CSM, and its recovery following central corpectomy (CC)., Method: We conducted a prospective study on 19 patients with moderate and severe CSM who underwent CC from June 2008 to December 2010. Autonomic dysfunction was defined as the presence of 'bladder dysfunction' or 'orthostatic hypotension'. All patients underwent SSR test preoperatively and at follow-up. Functional evaluation was done using Nurick grade and modified Japanese Orthopedic Association (mJOA) score preoperatively and at follow-up., Findings: In the preoperative assessment, 14 of 19 (73.7%) patients had bladder dysfunction and orthostatic hypotension. SSR was absent in 13 (68.4%) patients preoperatively. At a mean follow-up of 14.5 months after CC, SSR was present in 12 of the 14 patients available for follow-up. SSR returned postoperatively in 9 of the 11 patients in whom it was absent preoperatively. Recovery of SSR postoperatively had significant correlation with improvement in Nurick grade (p =0.02), improvement in lower limb component of mJOA score (p =0.001) and Nurick grade recovery rate (p = 0.008)., Conclusions: Dysfunction of the autonomic pathways as determined by the SSR is seen in nearly 70% of patients with moderate and severe CSM but did not correlate with other autonomic functions, suggesting possibly different pathways for different autonomic functions. Following uninstrumented CC, SSR returned in almost 80% of patients in whom it was absent preoperatively and this correlated significantly with improvement in functional grade. Decompressive surgery can reverse autonomic dysfunction in most of these patients.

Objectives: Carbamazepine (CBZ) is a commonly used anti-epileptic in rural hospitals in India. These hospitals lack the facilities to measure CBZ concentration; however, in larger hospitals this is performed using high performance liquid chromatography (HPLC). Dried blood spot (DBS) represents a feasible matrix for safe transportation by post/courier. This study was to determine whether the concentration of CBZ in serum can be predicted from that measured in DBS using an inexpensive HPLC method and inexpensive standard filter paper., Methods: CBZ in serum and DBS from 80 epileptic patients were measured using a validated HPLC assay. The data was then randomly divided into two groups; simple Deming regression was performed with the first group and validation was performed using the second., Results: There was a good correlation between the serum and DBS concentrations (r = 0.932) in the first group. The regression equation obtained was: predicted serum concentration = DBS concentration x 0.83 + 1.09. In the validation group, the correlation between the predicted and actual serum concentrations was also good (r = 0.958), and the mean difference between them was only 0.28 μg/ml (p = 0.8062). The imprecision and bias in both the groups were acceptable., Conclusion: Using inexpensive materials, serum CBZ concentrations can be accurately predicted from DBS specimens. This method can be recommended for the therapeutic drug monitoring of CBZ in resource-limited settings.

Objectives: To determine the proportion of refractory status epilepticus (RSE) and super-RSE (SRSE) among patients with status epilepticus (SE) and to analyze RSE and non-RSE (NRSE) in terms of etiology and predictors for RSE., Materials and Methods: Patients were identified from discharge summaries database with keywords of SE and records of the portable electroencephalogram (EEG) machine from January 2011 to March 2016., Results: Two hundred and eighteen events were included in the study with 114 (52.3%) males, bimodal age preponderance age <5 years 30%, and second peak in age 15-65 years 52.8%, preexisting seizures were present in 34.4% ( n = 75). Nearly 77.1% had NRSE ( n = 168) and 22.9% had RSE ( n = 50). This included 17 patients with SRSE ( n = 17, 7.8% of all SE). Central nervous system (CNS) infection was a single largest etiological group in SE (69/218, 31.7%). In RSE, autoimmune encephalitis (17/50) and CNS infection (13/50) were the largest groups. De novo seizures ( P = 0.007), low sensorium at admission ( P = 0.001), low albumin at admission ( P = 0.002), and first EEG being abnormal ( P = 0.001) were risk factors on bivariate analysis. An unfavorable status epilepticus severity score (STESS) was predictive for RSE ( P = 0.001). On multivariate analysis, de novo seizures ( P = 0.009) and abnormal EEG at admission ( P = 0.03) were predictive for RSE., Conclusions: Fifty patients had RSE (22.9%), of which 17 went on to become SRSE (7.8%). Unfavorable STESS score was predictive for RSE on bivariate analysis. On multivariate analysis, de novo seizures and abnormal initial EEG were predictors of RSE., Competing Interests: There are no conflicts of interest.

Background: In dural venous sinus thrombosis (DVST), the mortality ranges 5-30%. Deep venous system involvement and septic dural sinus thrombosis have a higher mortality rate. In acute occlusion, collateral flow may not be established, which may result in significant edema and mass effect. Endovascular interventions may be considered as a treatment option in appropriate high-risk patients with DVST., Materials and Methods: Eight patients with magnetic resonance imaging (MRI)-confirmed dural sinus thrombosis, who did not respond to the conventional standard medical treatment, were subsequently treated with mechanical thrombectomy using the Penumbra System ® . In all cases, medical treatment including anticoagulants were continued following the procedure for a minimum period of 1 year., Results: Recanalization of the dural sinus thrombosis was achieved in all 8 cases. There were no immediate or late endovascular-related complications. One death occurred due to an unrelated medical event. At 6 months, there was notable improvement in the modified Rankin Score (mRS), with 5/8 (62%) patients achieving mRS of 2 or less. The follow-up ranged between 3 months and 26 months (mean: 14.5 months), and there were no new neurological events during the follow-up period., Conclusion: Cerebral venous sinus thrombosis is a rare but life-threatening condition that demands timely diagnosis and therapy. In cases of rapidly declining neurological status despite standard therapy with systemic anticoagulation and anti-edema measures, mechanical thrombectomy could be a lifesaving and effective option. In this study, good outcomes were observed in the majority of patients at long-term follow up., Competing Interests: There are no conflicts of interest.

Background: Intravenous (IV) tissue plasminogen activator (tPA) infusion combined with transcranial low-frequency ultrasound waves targeted on the occluded arterial segment (sonothrombolysis) can increase recanalization in large artery-acute ischemic stroke (LA-AIS)., Aims: To evaluate the benefits of sonothrombolysis in LA-AIS., Settings and Designs: An open-labeled observational study done in a quaternary care teaching hospital., Methodology: Patients with LA-AIS within the window period (<4.5 h) with no contraindications for IV-recombinant tPA were sonothrombolysed. Recanalization was monitored and graded using the transcranial Doppler thrombolysis in brain ischemia (TIBI) flow criteria and also by time of flight magnetic resonance angiography using a modified thrombolysis in myocardial infarction score. Parenchymal changes were assessed using computed tomography (CT) or diffusion-weighted imaging-Alberta Stroke Programme Early CT Score. National Institutes of Health Stroke Scale (NIHSS) and modified Rankin Scale (mRS) were used to assess the outcome., Results: Eighteen patients underwent sonothrombolysis and the mean onset to needle time was 138 min (range 65-256). TIBI residual flow grade of ≥2 was seen in 15 of 18 patients (83%). Immediate dramatic improvement (NIHSS score ≤3 points or improvement by ≥10 points) was seen in 6 of 18 patients (30%) and in 9 of 18 patients (50%) within the next 24 h. Two patients (one with TIBI 0, another with re-occlusion) underwent mechanical thrombectomy post-sonothrombolysis. Symptomatic hemorrhage occurred in 5.5% of the patients. At 6 months, 2 of 18 patients (11%) died and 10 of 16 patients (63%) achieved mRS ≤2., Conclusions: Sonothrombolysis appears to be a safe way to augment the effect of tPA without increasing the door to needle time with the added advantage of observing flow through the occluded artery in real time.

Context: Multiple sclerosis (MS) has a spectrum of heterogeneity, as seen in western and eastern hemispheres, in the clinical features, topography of involvement and differences in natural history., Aim: To study the clinical spectrum, imaging, and electrophysiological as well as cerebrospinal fluid (CSF) characteristics and correlate them with outcome., Settings and Design: Retrospective analysis of MS patients during a period of 20 years., Subjects and Methods: Cases were selected according to recent McDonald's criteria (2010), They were managed in the Department of Neurology, Christian Medical College, Vellore., Statistical Analysis Used: Chi-square and Fisher's exact tests were used for categorical variables. Multiple binary logistic regressions were done to assess significance. Kaplan-Meier curves were drawn to estimate the time to irreversible disability., Results: A total of 157 patients with female preponderance (55%) were included. The inter quartile range duration of follow-up was 9.1 (8.2, 11) years for 114 patients, who were included for final outcome analysis. Relapsing remitting MS (RRMS) (54.1%) was the most common type of MS seen. RRMS had a significantly better outcome (odds ratio: 0.12, 95% confidence interval: 0.02-0.57, P = 0.008) compared to progressive form of MS (primary progressive, secondary progressive). The Expanded Disability Status Scale score of patients at presentation and at final follow-up was 4.4 ± 1.31 and 4.1 ± 2.31, respectively. During the first presentation, polysymptomatic manifestations like motor and sphincteric involvement, incomplete recovery from the first attack; and, during the disease course, bowel, bladder, cerebellar and pyramidal affliction, predicted a worse outcome., Conclusion: A high incidence of optico-spinal presentation, predominance of RRMS and a low yield on cerebrospinal fluid (CSF) studies are the major findings of our study. A notable feature was the analysis of prognostic markers of disability.

Background: Strokes caused by normal variants of the cerebral circulation can be difficult to diagnose, hence a high index of suspicion is needed. This case series discusses the clinical and radiological aspects of one such stroke caused by occlusion of the artery of Percheron (AOP)., Materials and Methods: Computerized discharge summaries, outpatient records and imaging from picture archiving and communication system (PACS, GE), of patients with AOP infarction over a period of 12-years (2002-2014) were identified and their clinical and radiological features analyzed., Results: Of 3589 strokes (both ischemic and hemorrhagic), 17 (0.47%) were due to AOP infarction. Their mean age was 50 years (range: 31-72 years). Disorders of consciousness (94%) were the most common presenting symptoms followed by gaze (53%) and memory impairment (24%). At follow-up, 2/17 (12%) patients developed extrapyramidal features. All patients had bilateral paramedian thalamic infarcts on magnetic resonance imaging (MRI). Associated anterior thalamic (5/17; 30%) and mid brain (10/17; 59%) infarcts were also seen. CT scan done in 11/17 patients prior to the MRI picked up only 6/11 (55%) of these infarcts. The most common etiological factors detected using the Trial of Org 10172 in Acute Stroke Treatment (TOAST) criteria were cardio embolic (8/17; 47%) followed by small vessel occlusion (7/17; 41%). Mortality occurred in 2/17 (12%) patients. At 6 months, a modified Rankin score of 2 or less was seen in 8/17 (47%) patients., Conclusions: Artery of Percheron infarcts should be considered in the differential diagnosis of patients presenting with sudden alterations in consciousness. MRI should be the investigation of choice. An embolic etiology should be actively looked for.

Hypomyelination, hypodontia, hypogonadotropic hypogonadism (4H) syndrome is a rare hypomyelination disorder with around 40 cases reported worldwide. Children with hypomyelination, hypodontia, hypogonadotropic hypogonadism syndrome present with varying degrees of developmental delay with a spastic ataxic syndrome with delayed eruption of teeth along with disruption in the eruption sequence, hypogonadotropic hypogonadism, and a fluctuating clinical course with intercurrent infections and varying periods of stability. The disorder is caused by mutations in POL3A and POL3B genes and is collectively termed as pol III-related leukodystrophies. Here we describe 2 children with hypomyelination, hypodontia, hypogonadotropic, hypogonadism syndrome and the association of multiple vertebral fusion anomalies in one of them, which has not been previously described in the literature. We conclude that the spectrum of the disorder is not limited to brain parenchyma alone and involves all the structures arising from neural ectoderm, and this needs further research., (© The Author(s) 2014.)