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1.
J Clin Neuromuscul Dis ; 25(3): 122-131, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38441928

RESUMO

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.


Assuntos
Paralisia Bulbar Progressiva , Doenças Neuromusculares , Insuficiência Respiratória , Humanos , Feminino , Pessoa de Meia-Idade , Centros de Atenção Terciária , Estudos Retrospectivos , Doenças Neuromusculares/complicações , Doenças Neuromusculares/diagnóstico , Insuficiência Respiratória/diagnóstico , Insuficiência Respiratória/etiologia
2.
3.
Health Aff (Millwood) ; 41(8): 1078-1087, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35858118

RESUMO

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.


Assuntos
COVID-19 , Medicaid , Acessibilidade aos Serviços de Saúde , Humanos , Pandemias , Patient Protection and Affordable Care Act , Estados Unidos , Virginia
4.
J Neuroimmunol ; 367: 577862, 2022 06 15.
Artigo em Inglês | MEDLINE | ID: mdl-35439670

RESUMO

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.


Assuntos
Rituximab , Adulto , Estudos de Coortes , Humanos , Estudos Prospectivos , Estudos Retrospectivos , Resultado do Tratamento
5.
Health Policy ; 126(5): 427-437, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-34497031

RESUMO

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.


Assuntos
COVID-19 , Canadá/epidemiologia , Política de Saúde , Humanos , Irlanda/epidemiologia , Pandemias , Reino Unido/epidemiologia , Estados Unidos/epidemiologia
6.
Health Econ Policy Law ; 17(1): 62-75, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33663642

RESUMO

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.


Assuntos
COVID-19 , Pandemias , Teste para COVID-19 , Busca de Comunicante , Humanos , Pandemias/prevenção & controle , SARS-CoV-2 , Estados Unidos/epidemiologia
8.
Indian J Radiol Imaging ; 31(1): 57-64, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34316112

RESUMO

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.

9.
Neurol India ; 69(2): 369-375, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33904455

RESUMO

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.


Assuntos
Eletrodiagnóstico , Síndrome de Guillain-Barré , Axônios , Síndrome de Guillain-Barré/diagnóstico , Humanos , Condução Nervosa , Estudos Retrospectivos
10.
Ann Indian Acad Neurol ; 24(1): 32-39, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33911377

RESUMO

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.

12.
Can J Neurol Sci ; 48(5): 616-625, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-33397535

RESUMO

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.


Assuntos
Gamopatia Monoclonal de Significância Indeterminada , Paraproteinemias , Doenças do Sistema Nervoso Periférico , Plasmocitoma , Humanos , Masculino , Gamopatia Monoclonal de Significância Indeterminada/complicações , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Paraproteinemias/complicações , Paraproteinemias/diagnóstico , Estudos Retrospectivos
13.
J Cent Nerv Syst Dis ; 13: 11795735211057314, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-35173510

RESUMO

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.

14.
J Peripher Nerv Syst ; 26(1): 90-98, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33179828

RESUMO

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.


Assuntos
Potenciais de Ação/fisiologia , Eletrodiagnóstico/normas , Doenças Musculares/diagnóstico , Doenças Musculares/fisiopatologia , Condução Nervosa/fisiologia , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/fisiopatologia , Nervo Tibial/fisiologia , Adulto , Eletrodiagnóstico/métodos , Eletromiografia , Feminino , Seguimentos , Humanos , Síndrome de Isaacs/diagnóstico , Síndrome de Isaacs/fisiopatologia , Masculino , Pessoa de Meia-Idade , Mioquimia/diagnóstico , Mioquimia/fisiopatologia , Estudos Retrospectivos
15.
Ann Indian Acad Neurol ; 23(4): 528-535, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33223672

RESUMO

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.

16.
Ann Indian Acad Neurol ; 23(4): 522-527, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33223671

RESUMO

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.

17.
Case Rep Infect Dis ; 2020: 1315041, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32724683

RESUMO

West Nile virus (WNV) is currently a significant reemerging virus of the 21st century. It belongs to the family Flaviviridae and genus Flavivirus. Although it is primarily transmitted by the Culex spp of mosquitoes, other routes of transmission are also well defined. Of eight lineages described, Lineage 1a has been reported from many parts of South India and is known to cause neuroinvasive illness. Many tests and serological techniques have been described to diagnose WNV infection such as complement fixation, neutralization, heamagglutination inhibition, ELISA, and PCR for molecular confirmation. The latter far outweighs the limitations inherent in the other tests. WNV infection is being reported from Vellore for the first time after 1968. This paper aims to describe four cases of WNV infection causing central nervous system manifestations with its molecular characterization. West Nile virus infection was diagnosed with the available molecular techniques such as PCR and sequencing, which emphasizes the need for considering West Nile virus in the differential diagnosis of acute meningoencephalitis and the wider availability of molecular diagnostic tests.

18.
Ann Indian Acad Neurol ; 23(2): 201-205, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32189862

RESUMO

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.

19.
Ann Indian Acad Neurol ; 22(4): 464-471, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31736571

RESUMO

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.

20.
Int Neurourol J ; 23(3): 205-210, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31607099

RESUMO

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.

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