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1.
J Public Health Manag Pract ; 30(4): E161-E164, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38870384

RESUMO

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.


Assuntos
Escolha da Profissão , Médicos , Humanos , Feminino , Médicos/estatística & dados numéricos , Médicos/psicologia , Masculino , Estados Unidos
2.
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
3.
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
4.
Radiographics ; 39(3): 795-819, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30978149

RESUMO

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.


Assuntos
Seio Cavernoso/diagnóstico por imagem , Neoplasias de Cabeça e Pescoço/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Adulto , Idoso , Algoritmos , Angiografia Digital/métodos , Neoplasias Encefálicas/diagnóstico por imagem , Seio Cavernoso/patologia , Trombose do Corpo Cavernoso/diagnóstico por imagem , Artérias Cerebrais/diagnóstico por imagem , Veias Cerebrais/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Crânio/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Adulto Jovem
5.
Rheumatol Int ; 38(2): 203-209, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-28550399

RESUMO

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.


Assuntos
Doenças Autoimunes/tratamento farmacológico , Glucocorticoides/administração & dosagem , Hospitais de Ensino , Imunoglobulina G/imunologia , Imunossupressores/administração & dosagem , Inflamação/tratamento farmacológico , Ácido Micofenólico/administração & dosagem , Centros de Atenção Terciária , Adulto , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/imunologia , Autoimunidade/efeitos dos fármacos , Biomarcadores/sangue , Quimioterapia Combinada , Registros Eletrônicos de Saúde , Feminino , Fibrose , Glucocorticoides/efeitos adversos , Humanos , Imunoglobulina G/sangue , Imunossupressores/efeitos adversos , Índia , Inflamação/diagnóstico , Inflamação/imunologia , Masculino , Pessoa de Meia-Idade , Ácido Micofenólico/efeitos adversos , Indução de Remissão , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
6.
Neurol India ; 66(5): 1377-1380, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30233007

RESUMO

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.


Assuntos
Exame Neurológico/métodos , Tratos Piramidais/lesões , Traumatismos da Medula Espinal/diagnóstico , Adulto , Feminino , Dedos , Humanos , Masculino , Pessoa de Meia-Idade , Reflexo de Babinski , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Traumatismos da Medula Espinal/fisiopatologia , Adulto Jovem
7.
Br J Neurosurg ; 31(2): 199-204, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-27416074

RESUMO

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.


Assuntos
Doenças do Sistema Nervoso Autônomo/fisiopatologia , Descompressão Cirúrgica/métodos , Procedimentos Neurocirúrgicos/métodos , Pele/fisiopatologia , Espondilose/fisiopatologia , Espondilose/cirurgia , Sistema Nervoso Simpático/fisiopatologia , Adulto , Doenças do Sistema Nervoso Autônomo/etiologia , Feminino , Seguimentos , Humanos , Hipotensão Ortostática/etiologia , Hipotensão Ortostática/fisiopatologia , Extremidade Inferior/fisiopatologia , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Pele/inervação , Resultado do Tratamento , Bexiga Urinaria Neurogênica/etiologia , Bexiga Urinaria Neurogênica/fisiopatologia
8.
Neurol India ; 65(1): 52-57, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28084238

RESUMO

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.


Assuntos
Isquemia Encefálica/terapia , Fibrinolíticos/administração & dosagem , Trombólise Mecânica/métodos , Avaliação de Processos e Resultados em Cuidados de Saúde , Acidente Vascular Cerebral/terapia , Ativador de Plasminogênio Tecidual/administração & dosagem , Idoso , Isquemia Encefálica/tratamento farmacológico , Terapia Combinada , Feminino , Hospitais de Ensino , Humanos , Masculino , Pessoa de Meia-Idade , Acidente Vascular Cerebral/tratamento farmacológico , Ondas Ultrassônicas , Ultrassonografia Doppler Transcraniana
9.
Neurol India ; 63(1): 72-6, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25751473

RESUMO

Necrotizing myopathy with pipestem capillaries is a form of chronic inflammatory myopathy, with histopathology showing necrotizing myopathy, minimal cellular infiltration, and microangiopathy. A 30-year-old female presented with progressive limb weakness of 6 months, with skin pigmentation and Raynaud's phenomenon. Serum creatine phosphokinase was 3990 u/L. Muscle biopsy showed necrotic fibers, focal sparse perivascular inflammation/perifascicular atrophy, endomysial/epimysial vessel wall thickening with luminal narrowing. The features were of inflammatory necrotizing myopathy and neuropathy with pipestem capillaries/microangiopathy. She was pulsed with intravenous immunoglobulin, methylprednisolone, and cyclophosphamide and showed a good improvement. In the absence of widespread inflammatory response and classical histopathology findings, it is important to diagnose this condition as it shows a good response to aggressive and prolonged immunotherapy.


Assuntos
Capilares/patologia , Dermatomiosite/diagnóstico , Nervos Periféricos/patologia , Adulto , Creatina Quinase/sangue , Ciclofosfamida/uso terapêutico , Dermatomiosite/terapia , Feminino , Humanos , Imunoglobulinas/uso terapêutico , Inflamação/diagnóstico , Inflamação/terapia , Metilprednisolona/uso terapêutico , Músculo Esquelético/irrigação sanguínea , Músculo Esquelético/patologia , Necrose/diagnóstico , Necrose/terapia , Doença de Raynaud/diagnóstico , Doença de Raynaud/terapia
10.
Neurol India ; 63(4): 542-7, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26238889

RESUMO

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.

11.
Neurol India ; 63(6): 866-73, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26588619

RESUMO

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.

12.
BMC Med Genet ; 15: 114, 2014 Oct 25.
Artigo em Inglês | MEDLINE | ID: mdl-25344417

RESUMO

BACKGROUND: Spinocerebellar ataxia type 1 (SCA1) is a late onset autosomal dominant cerebellar ataxia, caused by CAG triplet repeat expansion in the ATXN1 gene. The frequency of SCA1 occurrence is more in Southern India than in other regions as observed from hospital-based studies. However there are no reports on variability of CAG repeat expansion, phenotype-genotype association and founder mutations in a homogenous population from India. METHODS: Genomic DNA isolated from buccal mouthwash of the individuals in the cohort was used for PCR-based diagnosis of SCA1. Subsequently SNP's found within the ATXN1 loci were identified by Taqman allelic discrimination assays. Significance testing of the genotype-phenotype associations was calculated by Kruskal-Wallis ANOVA test with post-hoc Dunnett's test and Pearson's correlation coefficient. RESULTS: By genetic analysis of an affected population in Southern India we identified 21 pre-symptomatic individuals including four that were well past the average age of disease onset of 44 years, 16 symptomatic and 63 normal individuals. All pre-symptomatic cases harbor "pure" expansions of greater than 40 CAGs. Genotyping to test for the presence of two previously identified SNPs showed a founder effect of the same repeat carrying allele as in the general Indian population. We show that SCA1 disease onset is significantly delayed when transmission of the disease is maternal. CONCLUSIONS: Our finding of early disease onset in individuals with a paternally inherited allele could serve as valuable information for clinicians towards early detection of SCA1 in patients with affected fathers. Identification of older pre-symptomatic individuals (n = 4) in our cohort among individuals with a shared genetic and environmental background, suggests that second site genetic or epigenetic modifiers might significantly affect SCA1 disease progression. Moreover, such undetected SCA1 cases could underscore the true prevalence of SCA1 in India.


Assuntos
Proteínas do Tecido Nervoso/genética , Proteínas Nucleares/genética , Ataxias Espinocerebelares/genética , População Branca/genética , Adulto , Idade de Início , Ataxina-1 , Ataxinas , Estudos de Coortes , Feminino , Efeito Fundador , Genótipo , Humanos , Índia , Masculino , Pessoa de Meia-Idade , Linhagem , Polimorfismo de Nucleotídeo Único , Expansão das Repetições de Trinucleotídeos , Adulto Jovem
13.
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
14.
J Neurol Neurosurg Psychiatry ; 84(9): 995-1000, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-23591554

RESUMO

BACKGROUND: Cerebral venous thrombosis (CVT) is an important cause for stroke in the young where the role for decompressive craniectomy is not well established. OBJECTIVE: To analyse the outcome of CVT patients treated with decompressive craniectomy. METHODS: Clinical and imaging features, preoperative findings and long-term outcome of patients with CVT who underwent decompressive craniectomy were analysed. RESULTS: Over 10 years (2002-2011), 44/587 (7.4%) patients with CVT underwent decompressive craniectomy. Diagnosis of CVT was based on magnetic resonance venography (MRV)/inferior vena cava (IVC). Decision for surgery was taken at admission in 19/44 (43%), within 12 h in 5/44 (11%), within first 48 h in 15/44 (34%) and beyond 48 h in 10/44 (22%). Presence of midline shift of ≥ 10 mm (p<0.0009) and large infarct volume (mean 146.63 ml; SD 52.459, p<0.001) on the baseline scan influenced the decision for immediate surgery. Hemicraniectomy was done in 38/44 (86%) and bifrontal craniectomy in 6/44 (13.6%). Mortality was 9/44 (20%). On multivariate analysis (5% level of significance) age <40 years and surgery within 12 h significantly increased survival. Mean follow-up was 25.5 months (range 3-66 months), 26/35 (74%) had 1 year follow-up. Modified Rankin Scale (mRs) continued to improve even after 6 months with 27/35 (77%) of survivors achieving mRs of ≤ 2. CONCLUSIONS: This is the largest series on decompressive craniectomy for CVT in literature to date. Decompressive craniotomy should be considered as a treatment option in large venous infarcts. Very good outcomes can be expected especially if done early and in those below 40 years.


Assuntos
Craniectomia Descompressiva/métodos , Trombose Intracraniana/cirurgia , Trombose Venosa/cirurgia , Adulto , Infarto Cerebral/patologia , Craniectomia Descompressiva/mortalidade , Feminino , Seguimentos , Lateralidade Funcional/fisiologia , Escala de Coma de Glasgow , Humanos , Processamento de Imagem Assistida por Computador , Trombose Intracraniana/diagnóstico por imagem , Trombose Intracraniana/mortalidade , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Cuidados Pós-Operatórios , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/terapia , Radiografia , Estudos Retrospectivos , Acidente Vascular Cerebral/cirurgia , Retalhos Cirúrgicos , Análise de Sobrevida , Resultado do Tratamento , Trombose Venosa/diagnóstico por imagem , Trombose Venosa/mortalidade , Adulto Jovem
15.
Neurochem Res ; 38(10): 2009-18, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23846623

RESUMO

The neuromuscular disorder, calpainopathy (LGMD 2A), is a major muscular dystrophy classified under limb girdle muscular dystrophies. Genetic mutations of the enzyme calpain 3 cause LGMD 2A. Calpainopathy is phenotypically observed as progressive muscle wasting and weakness. Pathomechanisms of muscle wasting of calpainopathy remain poorly understood. Oxidative stress, NF-κB and the ubiquitin proteasomal pathway underlie the pathology of several muscle wasting conditions but their role in calpainopathic dystrophy is not known. Oxidative and nitrosative stress, the source of reactive oxygen species, NF-κB signaling and protein ubiquitinylation were studied in 15 calpainopathic and 8 healthy control human muscle biopsies. Oxidative stress and NF-κB/IKK ß signaling were increased in calpainopathic muscle and may contribute to increased protein ubiquitinylation and muscle protein loss. Preventing oxidative stress or inhibition of NF-κB signaling could be considered for treatment of LGMD 2A.


Assuntos
Distrofia Muscular do Cíngulo dos Membros/patologia , NF-kappa B/fisiologia , Estresse Oxidativo , Complexo de Endopeptidases do Proteassoma/fisiologia , Ubiquitina/metabolismo , Adolescente , Calpaína/genética , Criança , Humanos , Quinase I-kappa B/fisiologia , Músculos/metabolismo , Distrofia Muscular do Cíngulo dos Membros/fisiopatologia , Nitritos/metabolismo , Transdução de Sinais , Adulto Jovem
16.
Muscle Nerve ; 48(3): 415-22, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-23861190

RESUMO

INTRODUCTION: Surface electromyography (SEMG) allows objective assessment and guides selection of appropriate treatment in focal hand dystonia (FHD). METHODS: Sixteen-channel SEMG obtained during different phases of a writing task was used to study timing, activation patterns, and spread of muscle contractions in FHD compared with normal controls. Customized software was developed to acquire and analyze EMG signals. RESULTS: SEMG of FHD subjects (20) showed "early onset" during motor imagery, rapid proximal muscle recruitment, agonist-antagonist co-contraction involving proximal muscle groups, "delayed offset" after stopping writing, higher rectified mean amplitudes, and mirror activity in contralateral limb compared with controls (16). Muscle activation latencies were heterogenous in FHD. CONCLUSIONS: Anticipation, delayed relaxation, and mirror EMG activation were noted in FHD. A clear pattern of muscle activation cannot be ascertained. Multi-channel SEMG can aid in objective assessment of temporal-spatial distribution of activity and can refine targeted therapies like chemodenervation and biofeedback.


Assuntos
Distúrbios Distônicos/patologia , Distúrbios Distônicos/reabilitação , Mãos/fisiopatologia , Imagens, Psicoterapia/métodos , Músculo Esquelético/fisiopatologia , Adulto , Biorretroalimentação Psicológica , Estudos de Casos e Controles , Avaliação da Deficiência , Estimulação Elétrica , Eletromiografia , Feminino , Lateralidade Funcional , Humanos , Masculino , Pessoa de Meia-Idade , Contração Muscular , Observação , Fatores de Tempo , Redação , Adulto Jovem
17.
18.
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
19.
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
20.
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
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