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1.
Artigo em Inglês | MEDLINE | ID: mdl-32178602

RESUMO

We have identified a previously unrecognised cluster of a newly recognised condition - acute flaccid myelitis (AFM) - among acute flaccid paralysis (AFP) cases identified by the Australian Paediatric Active Enhanced Disease Surveillance Network (PAEDS) 2007-2017. In the 12 months before and after detection of enterovirus D68 (EV-D68) from a single AFP case in April 2016, 24 of 97 notified cases of AFP were found to be clinically compatible with AFM; of these 24 cases, ten, clustered in early 2016, met magnetic resonance imaging (MRI) criteria for AFM. Detection of emerging enteroviruses requires collection of respiratory, cerebrospinal fluid and stool specimens, and should be routine practice for all AFP cases.


Assuntos
Viroses do Sistema Nervoso Central , Infecções por Enterovirus , Mielite , Doenças Neuromusculares , Austrália/epidemiologia , Viroses do Sistema Nervoso Central/diagnóstico , Viroses do Sistema Nervoso Central/epidemiologia , Criança , Surtos de Doenças , Enterovirus Humano D , Infecções por Enterovirus/diagnóstico , Infecções por Enterovirus/epidemiologia , Humanos , Imagem por Ressonância Magnética , Mielite/diagnóstico , Mielite/epidemiologia , Doenças Neuromusculares/diagnóstico , Doenças Neuromusculares/epidemiologia
2.
PLoS One ; 15(2): e0228671, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32053652

RESUMO

BACKGROUND: Acute flaccid myelitis (AFM) is a severe illness similar to paralytic poliomyelitis. It is unclear how frequently AFM occurred in U.S. children after poliovirus elimination. In 2014, an AFM cluster was identified in Colorado, prompting passive US surveillance that yielded 120 AFM cases of unconfirmed etiology. Subsequently, increased reports were received in 2016 and 2018. To help inform investigations on causality of the recent AFM outbreaks, our objective was to determine how frequently AFM had occurred before 2014, and if 2014 cases had different characteristics. METHODS: We conducted a retrospective study covering 2005-2014 at 5 pediatric centers in 3 U.S. regions. Possible AFM cases aged ≤18 years were identified by searching discharge ICD-9 codes and spinal cord MRI reports (>37,000). Neuroradiologists assessed MR images, and medical charts were reviewed; possible cases were classified as AFM, not AFM, or indeterminate. RESULTS: At 5 sites combined, 26 AFM cases were identified from 2005-2013 (average annual number, 3 [2.4 cases/100,000 pediatric hospitalizations]) and 18 from 2014 (12.6 cases/100,000 hospitalizations; Poisson exact p<0.0001). A cluster of 13 cases was identified in September-October 2014 (temporal scan p = 0.0001). No other temporal or seasonal trend was observed. Compared with cases from January 2005-July 2014 (n = 29), cases from August-December 2014 (n = 15) were younger (p = 0.002), more frequently had a preceding respiratory/febrile illness (p = 0.03), had only upper extremities involved (p = 0.008), and had upper extremity monoplegia (p = 0.03). The cases had higher WBC counts in cerebrospinal fluid (p = 0.013). CONCLUSION: Our data support emergence of AFM in 2014 in the United States, and those cases demonstrated distinctive features compared with preceding sporadic cases.


Assuntos
Viroses do Sistema Nervoso Central/diagnóstico , Viroses do Sistema Nervoso Central/epidemiologia , Surtos de Doenças , Mielite/diagnóstico , Mielite/epidemiologia , Doenças Neuromusculares/diagnóstico , Doenças Neuromusculares/epidemiologia , Adolescente , Fatores Etários , Viroses do Sistema Nervoso Central/líquido cefalorraquidiano , Viroses do Sistema Nervoso Central/terapia , Criança , Pré-Escolar , Enterovirus Humano D , Feminino , Hospitalização , Hospitais Pediátricos , Humanos , Lactente , Classificação Internacional de Doenças , Imagem por Ressonância Magnética , Masculino , Mielite/líquido cefalorraquidiano , Mielite/terapia , Doenças Neuromusculares/líquido cefalorraquidiano , Doenças Neuromusculares/terapia , Estudos Retrospectivos , Estações do Ano , Estados Unidos
3.
Urology ; 135: 165-170, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31626855

RESUMO

OBJECTIVE: To describe the urologic sequalae of several rare congenital neuromuscular diseases. METHODS: We retrospectively reviewed medical records at Gillette Specialty Healthcare (2014-2018) of patients presenting to urology clinic with lower urinary tract symptoms and select rare congenital diseases: muscular dystrophy, spinal muscular atrophy, and Rett syndrome. RESULTS: Muscular dystrophies (n = 19) are X-linked myogenic disorders characterized by progressive muscle wasting and weakness. Men present to the urologist at variable ages, typically with complaints of functional incontinence and normal cystometrograms; we manage them with oral anticholinergic medications, condom catheter, or suprapubic catheter. Spinal muscular atrophy (n = 6) is a rare autosomal recessive disease characterized by degeneration of the anterior horn cells in the spinal cord and motor nuclei in the lower brainstem leading to progressive muscle weakness and atrophy. Patients typically present with nephrolithiasis and urinary retention in late adolescence/early adulthood, but timing varies. Filling cystometrograms have been normal. We allow passive retention with intermittent catheterization and creation of catheterizable channels, when indicated. Rett syndrome (n = 5) is a rare, noninheritable genetic condition affecting females characterized by a brief period of normal development followed by loss of speech and purposeful hand use; there are characteristic behaviors. Patients present in early adulthood with complaints of urinary retention. We manage retention with permissive retention or sphincter chemodenervation. CONCLUSION: Several congenital neuromuscular conditions can cause lower urinary tract symptoms when these individuals become adults. We have discussed the clinical characteristics and management of select neurogenic and myogenic bladder conditions seen in adults with congenital conditions.


Assuntos
Cateterismo Uretral Intermitente , Sintomas do Trato Urinário Inferior/terapia , Doenças Neuromusculares/complicações , Doenças Raras/complicações , Agentes Urológicos/uso terapêutico , Adolescente , Adulto , Idoso , Criança , Fraldas para Adultos , Feminino , Humanos , Sintomas do Trato Urinário Inferior/diagnóstico , Sintomas do Trato Urinário Inferior/etiologia , Masculino , Pessoa de Meia-Idade , Doenças Neuromusculares/congênito , Doenças Neuromusculares/epidemiologia , Prevalência , Doenças Raras/congênito , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
4.
Rev. neurol. (Ed. impr.) ; 69(11): 442-452, 1 dic., 2019. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-187112

RESUMO

Introducción: Los recientes avances en el abordaje terapéutico de las enfermedades neuromusculares pediátricas han permitido un mejor pronóstico y, en consecuencia, surge la necesidad de medir la calidad de vida relacionada con la salud como parte de un abordaje integral. Es importante disponer de instrumentos válidos y específicos para su correcta valoración que contemplen la calidad de vida relacionada con la salud tanto autopercibida por los niños como por los padres. Objetivo: Evaluar la validez y la fiabilidad de la versión española del módulo neuromuscular de la Pediatric Quality of Life Inventory (PedsQL) para la medición de la calidad de vida autopercibida por niños de 5 a 7 años con enfermedades neuromusculares y la de sus padres. Sujetos y métodos: Con autorización de Mapi-Research-Trust, se procedió a la adaptación transcultural de la versión española de la escala. Posteriormente, se realizó un test-retest a 27 niños de 5 a 7 años y a 37 padres para evaluar la consistencia interna, la fiabilidad y la validez de constructo. Resultados: El coeficiente alfa de Cronbach mostró una consistencia interna buena para los niños y excelente para los padres. La correlación intraobservador indicó una excelente fiabilidad para ambos. La validez de constructo sugirió que una estructura de más dimensiones podría ser más adecuada y explicaría un mayor porcentaje de variabilidad. Conclusiones: La versión española del módulo neuromuscular de la PedsQL para evaluar calidad de vida de niños de 5 a 7 años con enfermedades neuromusculares y de sus padres tiene buena consistencia interna y fiabilidad


Introduction: The recent advances in the therapies for some neuromuscular disorders imply a better prognosis. As a consequence, health-related quality of life has emerged as a core outcome. It is particularly important to know both the self-perceived health-related quality of life by children, as soon as possible, as well as the parental perception. Therefore, it is essential to have valid and specific scales for proper assessment. Aim: To assess the validity and reliability of the Spanish version of Pediatric Quality of Life Inventory (PedsQL) Neuromuscular Module for self-perceived and parent perceived quality of life of children aged 5-7 with neuromuscular disorders. Subjects and methods: Cross-cultural validity of the Spanish version was carried out with the permission of the Mapi-Research-Trust. Subsequently, a test-retest was administered to 27 children aged 5-7 and 37 parents in order to evaluate internal consistency, intra-observer reliability and construct validity. Results: The Cronbach alpha coeffi cient showed good internal consistency for children and was rated as excellent by parents. Furthermore, the intra-observer correlation indicated an excellent reliability for both. Construct validity analysis suggested that a new scale structure with more dimensions might be more adequate. Moreover, said structure will also explain a greater percentage of variability. Conclusion: The Spanish version of PedsQL Neuromuscular Module for the self-perceived and parent-perceived quality of life of children aged 5-7 showed good internal consistency and reliability


Assuntos
Criança , Doenças Neuromusculares/epidemiologia , Qualidade de Vida , Reprodutibilidade dos Testes , Psicometria , Tradução , Análise Fatorial , Inquéritos e Questionários
5.
Pediatrics ; 144(5)2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31591135

RESUMO

BACKGROUND: Acute flaccid myelitis (AFM) is a neurologic condition characterized by flaccid limb weakness. After a large number of reports of AFM in 2014, the Centers for Disease Control and Prevention began standardized surveillance in the United States to characterize the disease burden and explore potential etiologies and epidemiologic associations. METHODS: Persons meeting the clinical case criteria of acute flaccid limb weakness from January 1, 2015, through December 31, 2017, were classified as confirmed (spinal cord gray matter lesions on MRI) or probable (white blood cell count >5 cells per mm3 in cerebrospinal fluid [CSF]). We describe clinical, radiologic, laboratory, and epidemiologic findings of pediatric patients (age ≤21 years) confirmed with AFM. RESULTS: Of 305 children reported from 43 states, 193 were confirmed and 25 were probable. Of confirmed patients, 61% were male, with a median age of 6 years (range: 3 months to 21 years; interquartile range: 3 to 10 years). An antecedent respiratory or febrile illness was reported in 79% with a median of 5 days (interquartile range: 2 to 7 days) before limb weakness. Among 153 sterile-site specimens (CSF and serum) submitted to the Centers for Disease Control and Prevention, coxsackievirus A16 was detected in CSF and serum of one case patient and enterovirus D68 was detected in serum of another. Of 167 nonsterile site (respiratory and stool) specimens, 28% tested positive for enterovirus or rhinovirus. CONCLUSIONS: AFM surveillance data suggest a viral etiology, including enteroviruses. Further study is ongoing to better characterize the etiology, pathogenesis, and risk factors of this rare condition.


Assuntos
Viroses do Sistema Nervoso Central/epidemiologia , Mielite/epidemiologia , Doenças Neuromusculares/epidemiologia , Adolescente , Distribuição por Idade , Viroses do Sistema Nervoso Central/virologia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Mielite/virologia , Doenças Neuromusculares/virologia , Estados Unidos/epidemiologia , Adulto Jovem
6.
Nat Med ; 25(11): 1748-1752, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31636453

RESUMO

Since 2012, the United States of America has experienced a biennial spike in pediatric acute flaccid myelitis (AFM)1-6. Epidemiologic evidence suggests non-polio enteroviruses (EVs) are a potential etiology, yet EV RNA is rarely detected in cerebrospinal fluid (CSF)2. CSF from children with AFM (n = 42) and other pediatric neurologic disease controls (n = 58) were investigated for intrathecal antiviral antibodies, using a phage display library expressing 481,966 overlapping peptides derived from all known vertebrate and arboviruses (VirScan). Metagenomic next-generation sequencing (mNGS) of AFM CSF RNA (n = 20 cases) was also performed, both unbiased sequencing and with targeted enrichment for EVs. Using VirScan, the viral family significantly enriched by the CSF of AFM cases relative to controls was Picornaviridae, with the most enriched Picornaviridae peptides belonging to the genus Enterovirus (n = 29/42 cases versus 4/58 controls). EV VP1 ELISA confirmed this finding (n = 22/26 cases versus 7/50 controls). mNGS did not detect additional EV RNA. Despite rare detection of EV RNA, pan-viral serology frequently identified high levels of CSF EV-specific antibodies in AFM compared with controls, providing further evidence for a causal role of non-polio EVs in AFM.


Assuntos
Viroses do Sistema Nervoso Central/genética , Infecções por Enterovirus/genética , Enterovirus/genética , Mielite/genética , Doenças Neuromusculares/genética , Estudos Soroepidemiológicos , Anticorpos Antivirais/líquido cefalorraquidiano , Anticorpos Antivirais/imunologia , Antígenos Virais/genética , Antígenos Virais/imunologia , Viroses do Sistema Nervoso Central/líquido cefalorraquidiano , Viroses do Sistema Nervoso Central/epidemiologia , Viroses do Sistema Nervoso Central/virologia , Pré-Escolar , Enterovirus/patogenicidade , Infecções por Enterovirus/líquido cefalorraquidiano , Infecções por Enterovirus/epidemiologia , Infecções por Enterovirus/virologia , Feminino , Humanos , Lactente , Masculino , Mielite/líquido cefalorraquidiano , Mielite/epidemiologia , Mielite/virologia , Doenças Neuromusculares/líquido cefalorraquidiano , Doenças Neuromusculares/epidemiologia , Doenças Neuromusculares/virologia , Estados Unidos
7.
Emerg Infect Dis ; 25(9): 1676-1682, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31407660

RESUMO

Acute flaccid myelitis (AFM) is a polio-like disease that results in paralysis in previously healthy persons. Although the definitive cause of AFM remains unconfirmed, enterovirus D68 (EV-D68) is suspected based on 2014 data demonstrating an increase in AFM cases concomitant with an EV-D68 outbreak. We examined the prevalence in children and the molecular evolution of EV-D68 for 2009-2018 in Philadelphia, Pennsylvania, USA. We detected widespread EV-D68 circulation in 2009, rare detections in 2010 and 2011, and then biennial circulation, only in even years, during 2012-2018. Prevalence of EV-D68 significantly correlated with AFM cases during this period. Finally, whole-genome sequencing revealed early detection of the B1 clade in 2009 and continued evolution of the B3 clade from 2016 to 2018. These data reinforce the need to improve surveillance programs for nonpolio enterovirus to identify possible AFM triggers and predict disease prevalence to better prepare for future outbreaks.


Assuntos
Viroses do Sistema Nervoso Central/epidemiologia , Surtos de Doenças , Enterovirus Humano D/isolamento & purificação , Infecções por Enterovirus/epidemiologia , Mielite/epidemiologia , Doenças Neuromusculares/epidemiologia , Viroses do Sistema Nervoso Central/virologia , Criança , Infecções por Enterovirus/virologia , Feminino , Humanos , Estudos Longitudinais , Masculino , Mielite/virologia , Doenças Neuromusculares/virologia , Philadelphia/epidemiologia , Estudos Retrospectivos
8.
MMWR Morb Mortal Wkly Rep ; 68(27): 608-614, 2019 Jul 12.
Artigo em Inglês | MEDLINE | ID: mdl-31295232

RESUMO

BACKGROUND: Acute flaccid myelitis (AFM), a serious paralytic illness, was first recognized as a distinct condition in 2014, when cases were reported concurrent with a large U.S. outbreak of severe respiratory illness caused by enterovirus D-68 (EV-D68). Since 2014, nationwide outbreaks of AFM have occurred every 2 years in the United States; the cause for the recent change in the epidemiology of AFM in the United States, including the occurrence of outbreaks and a biennial periodicity since 2014, is under investigation. This report updates clinical, laboratory, and outcome data for cases reported to CDC during 2018. METHODS: Clinical data and specimens from persons in the United States who met the clinical criterion for AFM (acute onset of flaccid limb weakness) with onset in 2018 were submitted to CDC for classification of the illnesses as confirmed, probable, or non-AFM cases. Enterovirus/rhinovirus (EV/RV) testing was performed on available specimens from persons meeting the clinical criterion. Descriptive analyses, laboratory results, and indicators of early recognition and reporting are summarized. RESULTS: From January through December 2018, among 374 reported cases of AFM, 233 (62%) (from 41 states) were classified as confirmed, 26 (7%) as probable, and 115 (31%) as non-AFM cases. Median ages of patients with confirmed, probable, and non-AFM cases were 5.3, 2.9, and 8.8 years, respectively. Laboratory testing identified multiple EV/RV types, primarily in respiratory and stool specimens, in 44% of confirmed cases. Among confirmed cases, the interval from onset of limb weakness until specimen collection ranged from 2 to 7 days, depending on specimen type. Interval from onset of limb weakness until reporting to CDC during 2018 ranged from 18 to 36 days, with confirmed and probable cases reported earlier than non-AFM cases. CONCLUSION: Identification of risk factors leading to outbreaks of AFM remains a public health priority. Prompt recognition of signs and symptoms, early specimen collection, and complete and rapid reporting will expedite public health investigations and research studies to elucidate the recent epidemiology of AFM and subsequently inform treatment and prevention recommendations.


Assuntos
Viroses do Sistema Nervoso Central/epidemiologia , Surtos de Doenças/estatística & dados numéricos , Mielite/epidemiologia , Doenças Neuromusculares/epidemiologia , Vigilância da População , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Estações do Ano , Estados Unidos/epidemiologia , Adulto Jovem
9.
Eur J Pediatr ; 178(9): 1305-1315, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31338675

RESUMO

Acute flaccid myelitis is characterized by the combination of acute flaccid paralysis and a spinal cord lesion largely restricted to the gray matter on magnetic resonance imaging. The term acute flaccid myelitis was introduced in 2014 after the upsurge of pediatric cases in the USA with enterovirus D68 infection. Since then, an increasing number of cases have been reported worldwide. Whereas the terminology is new, the clinical syndrome has been recognized in the past in association with several other neurotropic viruses such as poliovirus.Conclusion: This review presents the current knowledge on acute flaccid myelitis with respect to the clinical presentation and its differential diagnosis with Guillain-Barré syndrome and acute transverse myelitis. We also discuss the association with enterovirus D68 and the presumed pathophysiological mechanism of this infection causing anterior horn cell damage. Sharing clinical knowledge and insights from basic research is needed to make progress in diagnosis, treatment, and prevention of this new polio-like disease. What is Known: • Acute flaccid myelitis (AFM) is a polio-like condition characterized by rapid progressive asymmetric weakness, together with specific findings on MRI • AFM has been related to different viral agents, but recent outbreaks are predominantly associated with enterovirus D68. What is New: • Improving knowledge on AFM must increase early recognition and adequate diagnostic procedures by clinicians. • The increasing incidence of AFM urges cooperation between pediatricians, neurologists, and microbiologists for the development of treatment and preventive options.


Assuntos
Viroses do Sistema Nervoso Central/diagnóstico , Enterovirus Humano D , Infecções por Enterovirus/diagnóstico , Mielite/diagnóstico , Doenças Neuromusculares/diagnóstico , Viroses do Sistema Nervoso Central/epidemiologia , Viroses do Sistema Nervoso Central/terapia , Viroses do Sistema Nervoso Central/virologia , Diagnóstico Diferencial , Infecções por Enterovirus/complicações , Infecções por Enterovirus/epidemiologia , Infecções por Enterovirus/terapia , Saúde Global , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/virologia , Humanos , Mielite/epidemiologia , Mielite/terapia , Mielite/virologia , Mielite Transversa/diagnóstico , Mielite Transversa/virologia , Doenças Neuromusculares/epidemiologia , Doenças Neuromusculares/terapia , Doenças Neuromusculares/virologia , Prognóstico
10.
Rev Chil Pediatr ; 90(3): 309-315, 2019 Jun.
Artigo em Espanhol | MEDLINE | ID: mdl-31344191

RESUMO

INTRODUCTION: The early diagnosis of Sleep Disordered Breathing (SDB) may allow proper intervention. Currently, polygraphy (PG) is a reliable and accessible alternative. OBJECTIVE: To describe and analyze the PG of children > 1 year old with suspicion of SDB. PATIENTS AND METHOD: PG of children > 1 year old and adolescents from Concepcion, Chile, with suspected SDB were included, from December 2011 to Au gust 2017. Demographic, clinical and polygraphic variables were collected. It was used descriptive sta tistics, expressing results in median and range. The association between apnea-hypopnea index (AHI) and oxygen saturation was determined by Spearman's Rho, considering significance of p < 0.05. Re sults: 190 studies were analyzed. Age 7.9 years old (1.0-20.6), 61% males. DIAGNOSIS: neuromuscular disease (NMD) (24.2%), chronic lung damage (21.1%), upper airway obstruction (UAO) (19.5%), neurological damage (11%), Down syndrome (8.9%), upper airway malformations (7.4%), central hypoventilation (3.7%), obesity (2.6%), and others (1.6%). 55.3% were altered PG, with 53.3% of mild Obstructive Sleep Apnea Hypopnea Syndrome (OSAHS), 30.5% moderate, and 16.2% severe. There were no significant differences in AHI between groups of pathologies (p = 0.032), highlighting a higher AHI in obese patients 9 (0.41-51), and those with NMD 23.9 (0.4-36.6). It was found asso ciation between AHI and oxygen saturation parameters: mean saturation (rho = -0.425; p = 0.001), minimum (rho = -0.654; p = 0.001), and oxygen saturation below 90% (rho = 0.323; p = 0.001) in the whole sample. DISCUSSION: There was a high percentage of OSAHS in at-risk pediatric patients, especially in those with NMD and obesity. PG is an accessible and implementable tool in a public hospital, a situation that can potentially be extrapolated to other healthcare centers.


Assuntos
Polissonografia/métodos , Síndromes da Apneia do Sono/diagnóstico , Apneia Obstrutiva do Sono/diagnóstico , Adolescente , Criança , Pré-Escolar , Chile/epidemiologia , Feminino , Humanos , Lactente , Masculino , Doenças Neuromusculares/epidemiologia , Oxigênio/metabolismo , Obesidade Pediátrica/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Síndromes da Apneia do Sono/epidemiologia , Apneia Obstrutiva do Sono/epidemiologia
11.
Neurology ; 92(24): e2822-e2831, 2019 06 11.
Artigo em Inglês | MEDLINE | ID: mdl-31127074

RESUMO

OBJECTIVE: To characterize the most common neurologic diagnoses leading to hospitalization for homeless compared to housed individuals and to assess whether homelessness is an independent risk factor for 30-day readmission after an admission for a neurologic illness. METHODS: We performed a retrospective serial cross-sectional study using data from the Healthcare Cost and Utilization Project California State Inpatient Database from 2006 to 2011. Adult patients with a primary neurologic discharge diagnosis were included. The primary outcome was 30-day readmission. We used multilevel logistic regression to examine the association between homelessness and readmission after adjustment for patient factors. RESULTS: We identified 1,082,347 patients with a neurologic primary diagnosis. The rate of homelessness was 0.37%. The most common indications for hospitalization among homeless patients were seizure and traumatic brain injury, both of which were more common in the homeless compared to housed population (19.3% vs 8.1% and 31.9% vs 9.2%, respectively, p < 0.001). A multilevel mixed-effects model controlling for patient age, sex, race, insurance type, comorbid conditions, and clustering on the hospital level found that homelessness was associated with increased 30-day readmission (odds ratio 1.5, 95% confidence interval 1.4-1.6, p < 0.001). This association persisted after this analysis was repeated within specific diagnoses (patients with epilepsy, trauma, encephalopathy, and neuromuscular disease). CONCLUSION: The most common neurologic reasons for admission among homeless patients are seizure and traumatic brain injury; these patients are at high risk for readmission. Future interventions should target the drivers of readmissions in this vulnerable population.


Assuntos
Pessoas em Situação de Rua/estatística & dados numéricos , Doenças do Sistema Nervoso/epidemiologia , Readmissão do Paciente/estatística & dados numéricos , Adulto , Idoso , Idoso de 80 Anos ou mais , Encefalopatias/epidemiologia , Lesões Encefálicas Traumáticas/epidemiologia , California/epidemiologia , Estudos Transversais , Epilepsia/epidemiologia , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Neuromusculares/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Convulsões/epidemiologia
12.
Medicine (Baltimore) ; 98(18): e15321, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31045770

RESUMO

Patients with myotonic muscular dystrophy type 1 (DM1) tend to exhibit earlier respiratory insufficiency than patients with other neuromuscular diseases at similar or higher forced vital capacity (FVC). This study aimed to analyze several pulmonary function parameters to determine which factor contributes the most to early hypercapnia in patients with DM1.We analyzed ventilation status monitoring, pulmonary function tests (including FVC, maximal voluntary ventilation [MVV], and maximal inspiratory and expiratory pressure), and polysomnography in subjects with DM1 who were admitted to a single university hospital. The correlation of each parameter with hypercapnia was determined. Subgroup analysis was also performed by dividing the subjects into 2 subgroups according to usage of mechanical ventilation.Final analysis included 50 patients with a mean age of 42.9 years (standard deviation = 11.1), 46.0% of whom were male. The hypercapnia was negatively correlated with MVV, FVC, forced expiratory volume in 1 second (FEV1), and their ratios to predicted values in subjects with myotonic muscular dystrophy type 1. At the same partial pressure of carbon dioxide, the ratio to the predicted value was lowest for MVV, then FEV1, followed by FVC. Moreover, the P values for differences in MVV and its ratio to the predicted value between ventilator users and nonusers were the lowest.When screening ventilation failure in patients with DM1, MVV should be considered alongside other routinely measured parameters.


Assuntos
Hipercapnia/fisiopatologia , Pulmão/fisiopatologia , Ventilação Voluntária Máxima/fisiologia , Distrofia Miotônica/complicações , Adulto , Dióxido de Carbono/análise , Feminino , Volume Expiratório Forçado/fisiologia , Humanos , Masculino , Pressões Respiratórias Máximas/métodos , Pessoa de Meia-Idade , Distrofia Miotônica/classificação , Distrofia Miotônica/fisiopatologia , Doenças Neuromusculares/epidemiologia , Doenças Neuromusculares/fisiopatologia , Polissonografia/métodos , Valor Preditivo dos Testes , Pressão , Estudos Prospectivos , Testes de Função Respiratória/métodos , Estudos Retrospectivos , Capacidade Vital/fisiologia
13.
mBio ; 10(2)2019 04 02.
Artigo em Inglês | MEDLINE | ID: mdl-30940708

RESUMO

Since 2014, acute flaccid myelitis (AFM), a long-recognized condition associated with polioviruses, nonpolio enteroviruses, and various other viral and nonviral causes, has been reemerging globally in epidemic form. This unanticipated reemergence is ironic, given that polioviruses, once the major causes of AFM, are now at the very threshold of global eradication and cannot therefore explain any aspect of AFM reemergence. Instead, the new AFM epidemic has been temporally associated with reemergences of nonpolio enteroviruses such as EV-D68, until recently thought to be an obscure virus of extremely low endemicity. This perspective reviews the enigmatic epidemiologic, virologic, and diagnostic aspects of epidemic AFM reemergence; examines current options for clinical management; discusses future research needs; and suggests that the AFM epidemic offers important clues to mechanisms of viral disease emergence.


Assuntos
Viroses do Sistema Nervoso Central/epidemiologia , Doenças Transmissíveis Emergentes/epidemiologia , Gerenciamento Clínico , Mielite/epidemiologia , Doenças Neuromusculares/epidemiologia , Viroses do Sistema Nervoso Central/diagnóstico , Viroses do Sistema Nervoso Central/etiologia , Viroses do Sistema Nervoso Central/terapia , Doenças Transmissíveis Emergentes/diagnóstico , Doenças Transmissíveis Emergentes/etiologia , Doenças Transmissíveis Emergentes/terapia , Saúde Global , Humanos , Mielite/diagnóstico , Mielite/etiologia , Mielite/terapia , Doenças Neuromusculares/diagnóstico , Doenças Neuromusculares/etiologia , Doenças Neuromusculares/terapia
16.
PLoS One ; 14(3): e0210574, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30913206

RESUMO

BACKGROUND: Population trends of disease prevalence and incidence over time measure burden of disease and inform healthcare planning. Neuromuscular disorders (NMD) affect muscle and nerve function with varying degrees of severity and disease progression. OBJECTIVE: Using health administrative databases we described trends in incidence, prevalence, and mortality of adults and children with NMD. We also explored place of death and use of palliative care. METHODS: Population-based (Ontario, Canada) cohort study (2003 to 2014) of adults and children with NMD identified using International Classification of Disease and health insurance billing codes within administrative health databases. RESULTS: Adult disease prevalence increased on average per year by 8% (95% confidence interval (CI) 6% to 10%, P <.001), with the largest increase in adults18-39 years. Childhood disease prevalence increased by 10% (95% CI 8% to 11%, P <.0001) per year, with the largest increase in children 0 to 5 years. Prevalence increased across all diagnoses except amyotrophic lateral sclerosis and spinal muscular atrophy for adults and all diagnoses for children. Adult incidence decreased by 3% (95% CI -4% to -2%, P <.0001) but incidence remained stable in children. Death occurred in 34,336 (18.5%) adults; 21,236 (61.8%) of whom received palliative care. Death occurred in 1,009 (5.6%) children; 507 (50.2%) of whom received palliative care. Mortality decreased over time in adults (odds ratio (OR) 0.86, 95% CI 0.86-0.87, P <.0001) and children (OR 0.79, 95% CI 0.76-0.82, P <.0001). Use of palliative care over time increased for adults (OR 1.18, 95% CI 1.09 to 1.28, P <.0001) and children (OR 1.22, 95% CI 1.20 to 1.23, P <.0001). CONCLUSIONS: In both adults and children, NMD prevalence is rising and mortality rates are declining. In adults incidence is decreasing while in children it remains stable. This confirms on a population-based level the increased survival of children and adults with NMD.


Assuntos
Doenças Neuromusculares/classificação , Doenças Neuromusculares/epidemiologia , Adolescente , Adulto , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Mortalidade , Doenças Neuromusculares/mortalidade , Razão de Chances , Ontário/epidemiologia , Admissão do Paciente/estatística & dados numéricos , Prevalência , Estudos Retrospectivos , Adulto Jovem
18.
PLoS One ; 14(3): e0213680, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30870495

RESUMO

OBJECTIVE: We investigated the presence of non-neuromuscular phenotypes in patients affected by Spinal Muscular Atrophy (SMA), a disorder caused by a mutation in the Survival of Motor Neuron (SMN) gene, and whether these phenotypes may be clinically detectable prior to clinical signs of neuromuscular degeneration and therefore independent of muscle weakness. METHODS: We utilized a de-identified database of insurance claims to explore the health of 1,038 SMA patients compared to controls. Two analyses were performed: (1) claims from the entire insurance coverage window; and (2) for SMA patients, claims prior to diagnosis of any neuromuscular disease or evidence of major neuromuscular degeneration to increase the chance that phenotypes could be attributed directly to reduced SMN levels. Logistic regression was used to determine whether phenotypes were diagnosed at significantly different rates between SMA patients and controls and to obtain covariate-adjusted odds ratios. RESULTS: Results from the entire coverage window revealed a broad spectrum of phenotypes that are differentially diagnosed in SMA subjects compared to controls. Moreover, data from SMA patients prior to their first clinical signs of neuromuscular degeneration revealed numerous non-neuromuscular phenotypes including defects within the cardiovascular, gastrointestinal, metabolic, reproductive, and skeletal systems. Furthermore, our data provide evidence of a potential ordering of disease progression beginning with these non-neuromuscular phenotypes. CONCLUSIONS: Our data point to a direct relationship between early, detectable non-neuromuscular symptoms and SMN deficiency. Our findings are particularly important for evaluating the efficacy of SMN-increasing therapies for SMA, comparing the effectiveness of local versus systemically delivered therapeutics, and determining the optimal therapeutic treatment window prior to irreversible neuromuscular damage.


Assuntos
Bases de Dados Factuais , Seguro Saúde/estatística & dados numéricos , Atrofia Muscular Espinal/diagnóstico , Atrofia Muscular Espinal/epidemiologia , Doenças Neuromusculares/diagnóstico , Adolescente , Adulto , Fatores Etários , Idoso , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Atrofia Muscular Espinal/fisiopatologia , Mutação , Doenças Neuromusculares/epidemiologia , Doenças Neuromusculares/fisiopatologia , Razão de Chances , Fenótipo , Análise de Regressão , Proteína 1 de Sobrevivência do Neurônio Motor/genética , Fatores de Tempo , Adulto Jovem
20.
Orv Hetil ; 160(Suppl 1): 13-21, 2019 Feb.
Artigo em Húngaro | MEDLINE | ID: mdl-30724604

RESUMO

INTRODUCTION: With the increasing number of the incidence of neuromusculoskeletal and brain circulation disorders, there is a higher demand for neuromusculoskeletal rehabilitation care. AIMS: The aim of our study is to analyse the performance indicators of neuromusculoskeletal rehabilitation care in Hungary financed by the statutory public health insurance system. METHODS: Data were derived from the financial database of the National Health Insurance Fund of Hungary. We analysed the period between 2014 and 2017. We investigated the distribution of neuromusculoskeletal rehabilitation hospital beds, the patient turnover and patients' pathways. We analysed the regional inequalities in the access to (hospital beds) and utilization (number of patients) of rehabilitation care. RESULTS: In 2017, there were 6798 publicly financed neuromusculoskeletal rehabilitation hospital beds in Hungary (6.94 beds/10 000 population). We observed the lowest number of hospital bed in Komárom-Esztergom (1.5 beds/10 000 population), Somogy (2.0) and Pest (2.7) counties. We found the highest number of hospital beds in Zala (12.6), Gyor-Moson-Sopron (12.2) and Baranya (11.5) counties. The more than 2-fold difference in the utilization (Komárom-Esztergom: 52.3 patients/10 000 population; Gyor-Moson-Sopron: 136 patients/10 000 population) confirms regional inequalities. Between 2014 and 2017, the annual number of patients showed an increasing tendency, while the average length of stay varied between 21.8 and 22.4 days/patient. The correlation coefficient between hospitals beds and the number of patients was very high (0.798). CONCLUSION: We found significant regional inequalities in the access to and utilization of neuromusculoskeletal rehabilitation. Orv Hetil. 2019; 160(Suppl 1): 13-21.


Assuntos
Política de Saúde , Acesso aos Serviços de Saúde , Doenças Musculoesqueléticas/reabilitação , Programas Nacionais de Saúde/economia , Doenças Neuromusculares/reabilitação , Acesso aos Serviços de Saúde/economia , Humanos , Hungria/epidemiologia , Incidência , Doenças Musculoesqueléticas/economia , Doenças Musculoesqueléticas/epidemiologia , Doenças Neuromusculares/economia , Doenças Neuromusculares/epidemiologia , Fatores Socioeconômicos
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