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1.
J Neurol ; 269(12): 6366-6376, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35869995

RESUMO

BACKGROUND: Optic neuritis (ON) is the most prevalent manifestation of pediatric multiple sclerosis (MSped) and myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGADped) in children > 6 years. In this study, we investigated retinal atrophy patterns and diagnostic accuracy of optical coherence tomography (OCT) in differentiating between both diseases after the first ON episode. METHODS: Patients were retrospectively identified in eight tertial referral centers. OCT, VEP and high/low-contrast visual acuity (HCVA/LCVA) have been investigated > 6 months after the first ON. Prevalence of pathological OCT findings was identified based on data of 144 age-matched healthy controls. RESULTS: Thirteen MOGADped (10.7 ± 4.2 years, F:M 8:5, 21 ON eyes) and 21 MSped (14.3 ± 2.4 years, F:M 19:2, 24 ON eyes) patients were recruited. We observed a significantly more profound atrophy of both peripapillary and macular retinal nerve fiber layer in MOGADped compared to MSped (pRNFL global: 68.2 ± 16.9 vs. 89.4 ± 12.3 µm, p < 0.001; mRNFL: 0.12 ± 0.01 vs. 0.14 ± 0.01 mm3, p < 0.001). Neither other macular layers nor P100 latency differed. MOGADped developed global atrophy affecting all peripapillary segments, while MSped displayed predominantly temporal thinning. Nasal pRNFL allowed differentiation between both diseases with the highest diagnostic accuracy (AUC = 0.902, cutoff < 62.5 µm, 90.5% sensitivity and 70.8% specificity for MOGADped). OCT was also substantially more sensitive compared to VEP in identification of ON eyes in MOGAD (pathological findings in 90% vs. 14%, p = 0.016). CONCLUSION: First MOGAD-ON results in a more severe global peripapillary atrophy compared to predominantly temporal thinning in MS-ON. Nasal pRNFL allows differentiation between both diseases with the highest accuracy, supporting the additional diagnostic value of OCT in children with ON.


Assuntos
Esclerose Múltipla , Neurite Óptica , Degeneração Retiniana , Humanos , Estudos Retrospectivos , Neurite Óptica/diagnóstico , Retina/diagnóstico por imagem , Retina/patologia , Tomografia de Coerência Óptica/métodos , Degeneração Retiniana/patologia , Esclerose Múltipla/complicações , Transtornos da Visão , Atrofia/patologia
3.
Eur J Neurol ; 27(12): 2595-2603, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32794258

RESUMO

BACKGROUND AND PURPOSE: Monitoring of the disease course of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) remains challenging because nerve conduction studies do not adequately correlate with functional disability. The prognostic value of pathological spontaneous activity (PSA) in needle electromyography (EMG) in different CIDP subgroups in a longitudinal context has, to date, not been analysed. We aimed to determine whether PSA was a prognostic marker or a marker of disease activity in a cohort of patients with CIDP. METHODS: A total of 127 patients with CIDP spectrum disorder were retrospectively analysed over 57 ± 47 months regarding the occurrence of PSA (fibrillations and positive sharp waves). The presence of PSA at diagnosis, newly occurring PSA, and continuously present PSA were longitudinally correlated with clinical disability using the Inflammatory Neuropathy Cause and Treatment Overall Disability Sum Score (INCAT-ODSS) and CIDP subtype. RESULTS: Pathological spontaneous activity occurred in 49.6% of all CIDP patients at first diagnosis. More frequent evidence of PSA was significantly associated with a higher INCAT-ODSS at the last follow-up. Continuous and new occurrence of PSA were associated with higher degree of disability at the last follow-up. The majority of patients with sustained evidence of PSA were characterized by an atypical phenotype, higher degree of disability, and the need for escalation of treatment. CONCLUSIONS: Pathological spontaneous activity was associated with a higher degree of disability and occurred more frequently in atypical CIDP variants according to the longitudinal data of a large cohort of patients with CIDP. Our results showed that EMG examination was an adequate marker for disease progression and should be evaluated during the disease course.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Progressão da Doença , Humanos , Condução Nervosa , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Prognóstico , Estudos Retrospectivos
4.
Colorectal Dis ; 22(11): 1677-1685, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32583513

RESUMO

AIM: The aim was to evaluate the influence of a half day, hands-on, workshop on the detection and repair of obstetric anal sphincter injuries (OASIs). METHOD: Starting in February 2011, hands-on workshops for the diagnosis and repair of OASIs were delivered by trained urogynaecologists in departments of tertiary medical centres in Israel. The structure of the hands-on workshop resembles the workshop organized at the International Urogynecological Association annual conferences. Participants included medical staff, midwives and surgical residents from each medical centre. We collected data regarding the rate of OASIs, 1 year before and 1 year following the workshop, in 11 medical centres. The study population was composed of parturients with the following inclusion criteria: singleton pregnancy, vertex presentation and vaginal delivery. Pre-viable preterm gestations (< 24 weeks), birth weight < 500 g, stillborn, and those with major congenital anomalies, multifoetal pregnancies, breech presentations and caesarean deliveries were excluded from the analysis. RESULTS: In the reviewed centres, 70 663 (49.3%) women delivered prior to the workshop (pre-workshop group) and 72 616 (50.7%) women delivered following the workshop (post-workshop group). Third- or fourth-degree perineal tears occurred in 248 women (0.35%) before the workshop, and in 328 (0.45%) following the workshop, a significant increase of 28.7% (P = 0.002). The increase in diagnosis was significant also in women with third-degree tears alone, 226 women (0.32%) before the workshop and 298 (0.41%) following the workshop, an increase of 28.3% (P = 0.005). CONCLUSION: The detection rate of OASIs has significantly increased following the hands-on workshop. The implementation of such programmes is crucial for increasing awareness and detection rates of OASI following vaginal deliveries.


Assuntos
Lacerações , Tocologia , Complicações do Trabalho de Parto , Canal Anal/lesões , Parto Obstétrico , Feminino , Humanos , Recém-Nascido , Israel/epidemiologia , Lacerações/diagnóstico , Lacerações/epidemiologia , Lacerações/terapia , Complicações do Trabalho de Parto/diagnóstico , Complicações do Trabalho de Parto/epidemiologia , Complicações do Trabalho de Parto/terapia , Gravidez , Estudos Retrospectivos , Fatores de Risco
6.
Eur J Neurol ; 27(4): 626-632, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31814240

RESUMO

BACKGROUND AND PURPOSE: Transorbital sonography (TOS) has emerged as promising imaging method for the diagnosis and follow-up of acute optic neuritis (ON). Available studies report an increase in the optic nerve diameter (OND) and the optic nerve sheath diameter (ONSD) in the case of a first episode of ON in the affected eye compared to either the contralateral unaffected eye or controls. However, the utility of TOS in the case of recurrent episodes of ON has never been assessed. METHODS: In our prospective cohort study, the diagnostic utility of TOS in patients with demyelinating diseases of the central nervous system was assessed, and the association between TOS, optical coherence tomography (OCT) and visual evoked potentials was examined further. RESULTS: Seventy-eight patients with a history of demyelinating disorders of the central nervous system (mean age 38.2 ± 14.2 years; 24% with acute ON) were included. No differences in the OND (3.2 ± 0.5 mm vs. 3.2 ± 0.4 mm) and ONSD (5.1 ± 0.8 mm vs. 5.1 ± 0.7 mm) measurements were found between patients with and without acute ON. Papillary swelling was more frequent in patients with acute ON (14.2% vs. 1.5%, P = 0.002). Patients with a history of previous ON were found to have lower OND (P < 0.001) and ONSD (P = 0.007) compared to patients without a history of previous ON. TOS measurements were inversely associated with disease duration and positively correlated with OCT findings. No association with visual evoked potential measurements was found. CONCLUSION: No evidence was found for TOS-sensitive differences in the OND and ONSD of patients with demyelinating diseases, according to the presence of acute ON. The association between TOS and OCT measurements deserves further investigation.


Assuntos
Nervo Óptico/diagnóstico por imagem , Neurite Óptica/diagnóstico por imagem , Ultrassonografia/métodos , Adulto , Atrofia/diagnóstico por imagem , Atrofia/patologia , Potenciais Evocados Visuais/fisiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Exame Neurológico , Nervo Óptico/patologia , Neurite Óptica/patologia , Estudos Prospectivos , Tomografia de Coerência Óptica , Adulto Jovem
7.
Transl Neurosci ; 10: 241-243, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31637048

RESUMO

Anti-N-methyl-D-aspartate receptor encephalitis (Anti-NMDARE) is a synaptic autoimmune encephalitis syndrome mainly affecting young females. An underlying tumor, most commonly ovarian teratomas in young females, may indicate a paraneoplastic syndrome. Prognostic factors of the clinical course of disease and outcome play a central role in view of early administration of second-line immunotherapy and intensive-care therapy. We report a case of severe Anti-NMDARE associated with unfavorable predictors including an extreme delta brush (EDB) electroencephalographic-pattern and high anti-NMDAR-antibody titers in the cerebral spinal fluid (CSF), which necessitated the admission to an intensive care unit. In spite of the poor prognosis, the patient completely recovered; we attribute this to an early escalation to second-line immunotherapy with rituximab and multidisciplinary intensive-care therapy. The present case underlines the relevance of multidisciplinary management for individuals with Anti-NMDARE.

8.
J Neurovirol ; 25(1): 133-136, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30414049

RESUMO

Long-term treatment of multiple sclerosis with natalizumab (NTZ) carries the risk of a devastating complication in the form of an encephalopathy caused by a reactivation of a latent John Cunningham virus infection (progressive multifocal leucoencephalopathy, PML). Early diagnosis is associated with considerably better prognosis. Quantitative EEG as an objective, rater-independent technique provides high sensitivity (88%) and specificity (82%) for the diagnosis of NTZ-PML. Combination of diagnostic modalities addressing static morphological (brain MRI) as well as functional (EEG) pathologic changes may improve risk management programmes.


Assuntos
Eletroencefalografia/métodos , Fatores Imunológicos/efeitos adversos , Vírus JC/efeitos dos fármacos , Leucoencefalopatia Multifocal Progressiva/diagnóstico , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Natalizumab/efeitos adversos , Adulto , Feminino , Humanos , Fatores Imunológicos/administração & dosagem , Vírus JC/crescimento & desenvolvimento , Vírus JC/patogenicidade , Leucoencefalopatia Multifocal Progressiva/induzido quimicamente , Leucoencefalopatia Multifocal Progressiva/patologia , Leucoencefalopatia Multifocal Progressiva/virologia , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla Recidivante-Remitente/diagnóstico , Esclerose Múltipla Recidivante-Remitente/patologia , Natalizumab/administração & dosagem , Prognóstico , Estudos Retrospectivos , Ativação Viral/efeitos dos fármacos
9.
Nervenarzt ; 90(2): 167-174, 2019 Feb.
Artigo em Alemão | MEDLINE | ID: mdl-30083879

RESUMO

BACKGROUND: Parkinson's disease is the most common neurodegenerative movement disorder and the fastest-growing neurological disease in the world. The diagnostic spectrum, demographic characteristics, comorbidities and case number developments of inpatient treatment in Germany with resulting implications for patient care have so far been insufficiently investigated. METHODS: Data from the diagnosis-related groups (DRG) statistics were analyzed in patients with a main and secondary diagnosis of primary Parkinson's syndrome (PS), secondary PS or other degenerative disease of the basal ganglia. For the reporting years 2010-2015, the dataset comprised 1,520,366 patient cases from 413 districts/independent cities throughout Germany. RESULTS: In 2015, mostly patients with moderate and severe primary PS were hospitalized (64.7%) often exhibiting motor fluctuations as well as marked medical and psychiatric comorbidities. Vascular parkinsonism was the most frequent secondary PS (36.6%) and progressive supranuclear palsy was the leading diagnosis in the other disorders of the basal ganglia (51.9%). Primary PS as a secondary diagnosis was found in many internal medicine hospitalizations. The inpatient case numbers for primary PS increased significantly from the years 2010 to 2015 and rural regions were particularly affected. CONCLUSION: The number of inpatient cases of Parkinson's disease is greatly increasing in Germany and mainly affects patients with severe motor complications and secondary parkinsonian syndromes. Particularly in rural areas, there is a risk of overburdening the treatment infrastructure, so that both outpatient and inpatient sectors must be strengthened. A limitation of the study is the analysis of only DRG coded data, whose quality could be improved in subsequent examinations by comparison with the current diagnostic criteria of the specialist societies.


Assuntos
Hospitalização , Doença de Parkinson , Alemanha/epidemiologia , Hospitalização/estatística & dados numéricos , Humanos , Doença de Parkinson/diagnóstico , Doença de Parkinson/epidemiologia , Doença de Parkinson/terapia
10.
J Neuroimaging ; 28(5): 506-514, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-29749662

RESUMO

BACKGROUND AND PURPOSE: Restless legs syndrome (RLS) is a multifactorial network disorder of a sensorimotor system extending from dopaminergic and glutamatergic cerebral structures to the spinal neurons and peripheral nerves. The role of peripheral nerve damage in the causality and severity progression for RLS patients remains unclear. METHODS: We performed a clinical and epidemiological study on a cohort of 34 RLS patients focusing on RLS risk factors and disease severity. We investigated the peripheral nerves with nerve conduction studies and with high-resolution nerve ultrasound (HRUS). RESULTS: In 18 of the 34 patients (mean age 67.4 ± 15 years old), a sensorimotor axonal neuropathy was diagnosed. These patients presented with late-onset RLS were treated with membrane stabilizing agents, whereas no neuropathy predisposing comorbidity could be identified for the majority of them. We could show an inverse correlation between the amplitudes of the tibial nerve for the patients with polyneuropathy and the RLS severity index. Neuropathy patients were characterized by an increase of the cross-sectional area (CSA) of the tibial nerve in the popliteal fossa and by increased intranerve and internerve variability values showing an asymmetry of CSA distribution. This pattern resembles previous studies on diabetic neuropathy. CONCLUSIONS: Early diagnosis, characterization, and treatment of neuropathy are increasingly relevant for RLS patients as it correlates with disease severity. HRUS revealed a pattern resembling diabetic neuropathy, which implies a similar pathophysiology with metabolic and ischemic origin of RLS-related axonal neuropathy.


Assuntos
Condução Nervosa/fisiologia , Nervos Periféricos/diagnóstico por imagem , Polineuropatias/diagnóstico por imagem , Síndrome das Pernas Inquietas/diagnóstico por imagem , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Nervos Periféricos/fisiopatologia , Polineuropatias/fisiopatologia , Síndrome das Pernas Inquietas/fisiopatologia , Fatores de Risco , Índice de Gravidade de Doença , Ultrassonografia
11.
Eur J Neurol ; 25(2): 215-237, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29352526

RESUMO

BACKGROUND AND PURPOSE: Multiple sclerosis (MS) is a complex disease of the central nervous system. As new drugs are becoming available, knowledge on diagnosis and treatment must continuously evolve. There is therefore a need for a reference tool compiling current data on benefit and safety, to aid professionals in treatment decisions and use of resources across Europe. The European Committee of Treatment and Research in Multiple Sclerosis (ECTRIMS) and the European Academy of Neurology (EAN) have joined forces to meet this need. The objective was to develop an evidence-based clinical practice guideline for the pharmacological treatment of people with MS to guide healthcare professionals in the decision-making process. METHODS: This guideline has been developed using the GRADE methodology and following the recently updated EAN recommendations for guideline development. Clinical questions were formulated in PICO format (patient, intervention, comparator, outcome) and outcomes were prioritized according to their relevance to clinical practice. An exhaustive literature search up to December 2016 was performed for each question and the evidence is presented narratively and, when possible, combined in a meta-analysis using a random-effects model. The quality of evidence for each outcome was rated into four categories - very high, high, low and very low - according to the risk of bias. GRADE evidence profiles were created using GRADEprofiler (GRADEpro) software (Version 3.6). The recommendations with assigned strength (strong, weak) were formulated based on the quality of evidence and the risk-benefit balance. Consensus between the panellists was reached by use of the modified nominal group technique. RESULTS: A total of 10 questions have been agreed, encompassing treatment efficacy, response criteria, strategies to address suboptimal response and safety concerns and treatment strategies in MS and pregnancy. The guideline takes into account all disease-modifying drugs approved by the European Medicine Agency at the time of publication. A total of 20 recommendations were agreed by the guideline working group members after three rounds of consensus.


Assuntos
Esclerose Múltipla/tratamento farmacológico , Neurologia/normas , Guias de Prática Clínica como Assunto/normas , Sociedades Médicas/normas , Europa (Continente) , Humanos
12.
Eur J Neurol ; 24(5): 726-733, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28328179

RESUMO

BACKGROUND AND PURPOSE: Significant effects on clinical/neuroradiological disease activity have been reported in patients with relapsing-remitting multiple sclerosis treated with delayed-release dimethyl fumarate (DMF) in phase III DEFINE/CONFIRM trials. We conducted a post hoc analysis of integrated data from DEFINE/CONFIRM to evaluate the effect of DMF on achieving no evidence of disease activity (NEDA) in patients with relapsing-remitting multiple sclerosis. METHODS: The analysis included patients randomized to DMF 240 mg twice daily, placebo or glatiramer acetate (CONFIRM only) for ≤2 years. A time-to-event method was used to estimate the percentage of patients achieving NEDA. Clinical NEDA (no relapses/no 12-week confirmed disability progression) was analysed in the intention-to-treat (ITT) population. Neuroradiological (no new/newly enlarging T2 hyperintense lesions/no gadolinium-enhancing lesions) and overall NEDA (clinical and neuroradiological NEDA) were analysed in the magnetic resonance imaging (MRI) cohort. RESULTS: The ITT and MRI populations comprised 1540 and 692 patients, respectively. The percentage of patients with clinical NEDA (ITT population) and neuroradiological NEDA (MRI cohort) was higher with DMF versus placebo over 2 years [clinical NEDA: 38.9% relative reduction; hazard ratio (HR), 0.61; 95% confidence interval (CI), 0.52-0.72; P < 0.0001; neuroradiological NEDA: 40.0% relative reduction; HR, 0.60; 95% CI, 0.49-0.73; P < 0.0001]. The percentage of patients achieving overall NEDA (MRI cohort) was also higher with DMF (26%) versus placebo (12%) over 2 years, with a relative risk reduction of 42.7% (HR, 0.57; 95% CI, 0.48-0.69; P < 0.0001). CONCLUSIONS: A significantly higher percentage of patients treated with DMF achieved NEDA status over 2 years compared with placebo.


Assuntos
Fumarato de Dimetilo/farmacologia , Progressão da Doença , Acetato de Glatiramer/farmacologia , Imunossupressores/farmacologia , Esclerose Múltipla Recidivante-Remitente/diagnóstico por imagem , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Adulto , Preparações de Ação Retardada , Fumarato de Dimetilo/administração & dosagem , Feminino , Acetato de Glatiramer/administração & dosagem , Humanos , Imunossupressores/administração & dosagem , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Recidiva , Resultado do Tratamento
13.
Mol Psychiatry ; 22(10): 1448-1454, 2017 10.
Artigo em Inglês | MEDLINE | ID: mdl-28138158

RESUMO

Systemic inflammation is accompanied by profound behavioral and mood changes that resemble symptoms of depression. Findings in animals suggest that pro-inflammatory cytokines released by activated immune cells in the periphery evoke these behavioral symptoms by driving inflammatory changes in the brain. However, experimental data in humans are lacking. Here we demonstrate in healthy male volunteers (10 endotoxin treated, 8 placebo treated) that intravenous administration of low-dose endotoxin (0.8 ng/kg body weight), a prototypical pathogen-associated molecular pattern that activates the innate immune system, not only induces a significant increase in peripheral blood cytokine concentrations (that is, tumor necrosis factor-α, interleukin (IL)-6, IL-10) but also results, with some latency, in a robust and selective increase of IL-6 in the cerebrospinal fluid (CSF). Moreover, we found a strong association between the endotoxin-induced increase of IL-6 in the CSF and the severity of mood impairment, with larger increases in CSF IL-6 concentration followed by a greater deterioration in mood. Taken together, these findings suggest that the appearance of depressive symptoms in inflammatory conditions might be primarily linked to an increase in central IL-6 concentration, identifying IL-6 as a potential therapeutic target in mood disorders.


Assuntos
Depressão/imunologia , Endotoxinas/administração & dosagem , Inflamação/imunologia , Interleucina-6/líquido cefalorraquidiano , Adulto , Citocinas/sangue , Depressão/sangue , Depressão/líquido cefalorraquidiano , Depressão/metabolismo , Humanos , Imunidade Inata , Inflamação/sangue , Inflamação/líquido cefalorraquidiano , Inflamação/psicologia , Interleucina-6/imunologia , Masculino , Adulto Jovem
14.
Matern Child Health J ; 21(3): 421-431, 2017 03.
Artigo em Inglês | MEDLINE | ID: mdl-28093689

RESUMO

Introduction Low-income populations have elevated exposure to early life risk factors for obesity, but are understudied in longitudinal research. Our objective was to assess the utility of a cohort derived from electronic health record data from safety net clinics for investigation of obesity emerging in early life. Methods We examined data from the PCORNet ADVANCE Clinical Data Research Network, a national network of Federally-Qualified Health Centers serving >1.7 million safety net patients across the US. This cohort includes patients who, in 2012-2014, had ≥1 valid body mass index measure when they were 0-5 years of age. We characterized the cohort with respect to factors required for early life obesity research in vulnerable subgroups: sociodemographic diversity, weight status based on World Health Organization (<2 years) or Centers for Disease Control (≥2 years) growth curves, and data longitudinality. Results The cohort includes 216,473 children and is racially/ethnically diverse (e.g., 17.9% Black, 45.4% Hispanic). A majority (56.9%) had family incomes below the Federal Poverty Level (FPL); 32% were <50% of FPL. Among children <2 years, 7.6 and 5.3% had high and low weight-for-length, respectively. Among children 2-5 years, 15.0, 12.7 and 2.4% were overweight, obese, and severely obese, respectively; 5.3% were underweight. In the study period, 79.2% of children had ≥2 BMI measures. Among 4-5 year olds, 21.9% had >1 BMI measure when they were <2 years. Discussion The ADVANCE Early Life cohort offers unique opportunities to investigate early life determinants of obesity in the understudied population of low income and minority children.


Assuntos
Bases de Dados como Assunto , Obesidade Infantil/epidemiologia , Pobreza/estatística & dados numéricos , Índice de Massa Corporal , Criança , Pré-Escolar , Estudos de Coortes , Registros Eletrônicos de Saúde/organização & administração , Etnicidade/estatística & dados numéricos , Feminino , Humanos , Lactente , Masculino , Obesidade Infantil/economia , Obesidade Infantil/etiologia , Fatores de Risco , Classe Social , Estados Unidos/epidemiologia
15.
Acta Neurol Scand ; 135(3): 324-331, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-27098675

RESUMO

OBJECTIVES: Cervical cord involvement is common in neuromyelitis optica (NMO) and multiple sclerosis (MS), but its impact on disability in NMO has rarely been studied. Recent publications on NMO examined the periventricular system, areas of high aquaporin-4 expression, but not yet by using ventricle volumetry. PURPOSE: To compare cervical cord atrophy, ventricular widening, and supra- and infratentorial brain measures between NMO and MS, and study their impact on clinical disability. METHODS: Magnet resonance imaging-based volumetry of upper cervical cord, third and fourth lateral ventricles, grey matter, white matter, brainstem, cerebellum and clinical status of 18 NMO and 20 MS patients, was compared between the groups and with 26 healthy controls. Patterns of ventricular widening relative to healthy controls were inspected by voxel-based morphometry of the cerebrospinal fluid. RESULTS: Cervical cord atrophy was similar in NMO and MS (75.2 ± 10.0 mm2 , respectively, 76.5 ± 9.5 mm2 vs 84.1 ± 8.6 mm2 in controls).Third ventricle increase in both groups, and specific fourth ventricle widening in MS were detected. Patient groups differed in third to fourth ventricle ratio (P = 0.002). In NMO, white matter correlated inversely with the affected cord segments (P = 0.001) and with cervical cord area (P = 0.043). The disability status was explained by cervical cord area and third ventricle volume (R2 =0.524) in NMO, and by grey matter and fourth ventricle volume (R2 =0.565) in MS. CONCLUSION: Cervical cord atrophy and third ventricular enlargement are both clinically relevant in NMO. Third and fourth ventricle volumetry shows differences between NMO and MS regarding the involvement of periventricular structures.


Assuntos
Medula Cervical/diagnóstico por imagem , Quarto Ventrículo/diagnóstico por imagem , Esclerose Múltipla/diagnóstico por imagem , Neuromielite Óptica/diagnóstico por imagem , Terceiro Ventrículo/diagnóstico por imagem , Adulto , Atrofia/diagnóstico por imagem , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade
16.
Fortschr Neurol Psychiatr ; 84(12): 733-738, 2016 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-27951604

RESUMO

An online survey of chief neurological consultants was conducted to find out how they judged the demand for and access to palliative and hospice care (PHC) structures for their neurological patients (NP) as well as their collaboration with PHC structures. 110 of 881 chief consultants who were contacted participated in the survey. About 10 % of their NP were considered suitable for PHC. They estimated that 9 % of the deceased had died from their underlying neurological disease. The integration of PHC structures into the management of various neurological diseases was considered worthwhile but 51.9 - 78.5 % indicated that there was no or little collaboration with PHC structures. 12 % of the participants were trained in palliative care (PC). To guarantee an adequate PHC also for NP, PHC knowledge should be integrated into the curriculum and training of neurologists and the PC community should further extend its services to NP, adapting their knowledge and structures to these patients' special needs.


Assuntos
Atitude do Pessoal de Saúde , Prestação Integrada de Cuidados de Saúde/organização & administração , Cuidados Paliativos na Terminalidade da Vida/organização & administração , Doenças do Sistema Nervoso/terapia , Cuidados Paliativos/organização & administração , Diretores Médicos , Encaminhamento e Consulta , Adulto , Idoso , Causas de Morte , Competência Clínica , Educação , Alemanha , Acessibilidade aos Serviços de Saúde/organização & administração , Necessidades e Demandas de Serviços de Saúde/organização & administração , Humanos , Comunicação Interdisciplinar , Colaboração Intersetorial , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/mortalidade , Doenças do Sistema Nervoso/psicologia , Equipe de Assistência ao Paciente/organização & administração , Sociedades Médicas
17.
J Geophys Res Space Phys ; 121(8): 7900-7913, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27867799

RESUMO

Energetic charged particle detectors characterize a portion of the plasma distribution function that plays critical roles in some physical processes, from carrying the currents in planetary ring currents to weathering the surfaces of planetary objects. For several low-resource missions in the past, the need was recognized for a low-resource but highly capable, mass-species-discriminating energetic particle sensor that could also obtain angular distributions without motors or mechanical articulation. This need led to the development of a compact Energetic Particle Detector (EPD), known as the "Puck" EPD (short for hockey puck), that is capable of determining the flux, angular distribution, and composition of incident ions between an energy range of ~10 keV to several MeV. This sensor makes simultaneous angular measurements of electron fluxes from the tens of keV to about 1 MeV. The same measurements can be extended down to approximately 1 keV/nucleon, with some composition ambiguity. These sensors have a proven flight heritage record that includes missions such as MErcury Surface, Space ENvironment, GEochemistry, and Ranging and New Horizons, with multiple sensors on each of Juno, Van Allen Probes, and Magnetospheric Multiscale. In this review paper we discuss the Puck EPD design, its heritage, unexpected results from these past missions and future advancements. We also discuss high-voltage anomalies that are thought to be associated with the use of curved foils, which is a new foil manufacturing processes utilized on recent Puck EPD designs. Finally, we discuss the important role Puck EPDs can potentially play in upcoming missions.

18.
Fortschr Neurol Psychiatr ; 84(6): 363-7, 2016 Jun.
Artigo em Alemão | MEDLINE | ID: mdl-27391986

RESUMO

Radiation-induced tissue damage is caused by ionizing radiation mainly affecting the skin, vascular, neuronal or muscle tissue. Early damages occur within weeks and months while late damages may occur months or even decades after radiation.Radiation-induced paresis of the spine or the trunk muscles with camptocormia or dropped-head syndrome are rare but have already been described as long-term sequelae after treatment of Hodgkin's lymphoma. The differential diagnosis includes limb-girdle muscular dystrophy, fascioscapulohumeral muscular dystrophy (FSHD) or lysosomal storage diseases (e. g. Acid Maltase Deficiency). We present the case of a patient with long lasting diagnostics over many months due to different inconclusive results.


Assuntos
Músculos do Dorso/inervação , Doença de Hodgkin/radioterapia , Atrofia Muscular Espinal/diagnóstico , Distrofias Musculares/diagnóstico , Músculos do Pescoço/inervação , Paresia/diagnóstico , Polirradiculopatia/diagnóstico , Lesões por Radiação/diagnóstico , Radiculopatia/diagnóstico , Curvaturas da Coluna Vertebral/diagnóstico , Raízes Nervosas Espinhais/efeitos da radiação , Adulto , Comorbidade , Diagnóstico Diferencial , Fracionamento da Dose de Radiação , Eletromiografia , Doença de Hodgkin/patologia , Humanos , Linfonodos/efeitos da radiação , Masculino , Estadiamento de Neoplasias , Exame Neurológico/efeitos da radiação , Aceleradores de Partículas , Fótons/efeitos adversos , Fótons/uso terapêutico , Dosagem Radioterapêutica , Baço/efeitos da radiação
19.
J Neurol ; 263(8): 1626-32, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27260297

RESUMO

Dimethyl fumarate (DMF) was recently approved for treating patients with relapsing-remitting multiple sclerosis (RRMS) based on two phase III clinical trials demonstrating its efficacy. This prompts the need for demonstrating the clinical efficacy and safety of DMF in the real world. By retrospective analysis of medical records at two German MS centers, 644 MS patients treated with DMF were identified. All were included in a safety analysis, and a subgroup of patients with available efficacy data during previous MS therapies (n = 352) was further analyzed for annualized relapse rate and disability progression assessed by the EDSS. In the overall DMF population studied, the annualized relapse rate decreased from 0.52 at baseline to 0.35, and the annualized disability progression from 0.15 to 0.10. Patients who were switched from interferons or glatiramer acetate to DMF revealed a greater benefit, whereas patients pretreated with more potent immunotherapies did not respond that well. Interestingly, patients with a lymphocyte count ≥2000/µl after 0.52 years (mean, SD 0.2) of DMF treatment did not benefit compared to those with lower lymphocyte counts. In total, 22.2 % of the patients withdrew from DMF due to side effects, with gastrointestinal discomfort (12.7 %) and lymphopenia (5.3 %) as most frequently reported reasons. Our study corroborates that DMF is an overall safe and effective drug that reduces relapse rate as well as disability progression in MS patients. Further prospective studies are warranted to establish the additional parameters predicting DMF response, especially in patients switching from other first-line immunotherapies.


Assuntos
Fumarato de Dimetilo/uso terapêutico , Imunossupressores/uso terapêutico , Esclerose Múltipla/tratamento farmacológico , Resultado do Tratamento , Adulto , Avaliação da Deficiência , Feminino , Seguimentos , Humanos , Contagem de Linfócitos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Estatísticas não Paramétricas
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