RESUMO
Natalizumab is a well-established disease-modifying therapy used in active multiple sclerosis (MS). The most serious adverse event is progressive multifocal leukoencephalopathy. For safety reasons, hospital implementation is mandatory. The SARS-CoV-2 pandemic has deeply affected hospital practices leading French authorities to temporarily authorize to administer the treatment at home. The safety of natalizumab home administration should be assessed to allow ongoing home infusion. The aim of the study is to describe the procedure and assess the safety in a home infusion natalizumab model. Patients presenting relapsing-remitting MS treated by natalizumab for over two years, non-exposed to John Cunningham Virus (JCV) and living in the Lille area (France) were included from July 2020 to February 2021 to receive natalizumab infusion at home every four weeks for 12 months. Teleconsultation occurrence, infusion occurrence, infusion cancelling, JCV risk management, annual MRI completion were analyzed. The number of teleconsultations allowing infusion was 365 (37 patients included in the analysis), all home infusions were preceded by a teleconsultation. Nine patients did not complete the one-year home infusion follow-up. Two teleconsultations canceled infusions. Two teleconsultations led to a hospital visit to assess a potential relapse. No severe adverse event was reported. All 28 patients who have completed the follow-up benefited from biannual hospital examination and JCV serologies and annual MRI. Our results suggested that the established home natalizumab procedure was safe using the university hospital home-care department. However, the procedure should be evaluated using home-based services outside the university hospital.
Assuntos
COVID-19 , Vírus JC , Leucoencefalopatia Multifocal Progressiva , Esclerose Múltipla Recidivante-Remitente , Esclerose Múltipla , Humanos , Natalizumab/efeitos adversos , Fatores Imunológicos/efeitos adversos , SARS-CoV-2 , Esclerose Múltipla/tratamento farmacológico , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Esclerose Múltipla Recidivante-Remitente/epidemiologia , Gestão de RiscosRESUMO
BACKGROUND: Cognitive impairment is frequent and disabling in multiple sclerosis (MS). The Brief International Cognitive Assessment in MS (BICAMS) is a recent short battery usable in clinical practice for cognitive evaluation of MS patients. OBJECTIVE: To find cortical areas or brain volumes on magnetic resonance imaging (MRI) structural sequences associated with BICAMS scores in MS. METHODS: In this cross-sectional single-center study (NCT03656055, September 4, 2018), 96 relapsing remitting-MS patients under natalizumab and without recent clinical or radiological inflammation were included. Patients underwent brain MRI and the three BICAMS tests, evaluating information processing speed (SDMT), visuo-spatial memory (BVMT-R), and verbal memory (FVLT). RESULTS: Cortical thickness in the left frontal superior and the right precentral gyri was associated with BVMT-R scores whereas cortical thickness in the left Broca's area and the right superior temporal gyrus was associated with FVLT scores. We observed associations between white matter inflammatory lesions connected to these cortical regions and BICAMS subscores. CONCLUSIONS: BICAMS scores are associated with specific cortical areas, the cognitive domain matching the known functions of the cortical area. Specific cognitive impairments in MS may be associated with specific cortical regions, themselves influenced by white matter inflammatory lesions and demographical parameters (age, sex, education level).
Assuntos
Disfunção Cognitiva , Esclerose Múltipla Recidivante-Remitente , Esclerose Múltipla , Cognição , Disfunção Cognitiva/complicações , Estudos Transversais , Humanos , Esclerose Múltipla/complicações , Esclerose Múltipla Recidivante-Remitente/complicações , Esclerose Múltipla Recidivante-Remitente/diagnóstico por imagem , Testes NeuropsicológicosRESUMO
BACKGROUND: In multiple sclerosis (MS), the prevalence of alexithymia, defined as an inability to identify and describe emotions, is close to 50% but the prevalence of this symptom in clinically isolated syndrome (CIS) is unknown. Characterizing alexithymia at an early stage of the disease can help to clarify psychobehavioural disturbances in CIS patients. METHODS: Forty CIS patients, who fulfilled the MRI criteria for dissemination in space, were matched with 40 healthy subjects. They completed self-assessment scales for alexithymia, depression, anxiety, apathy and empathy. Cognitive functions were assessed using a battery of neuropsychological tests. RESULTS: The mean delay (± standard deviation) between the occurrence of CIS and inclusion in the study was 3.9 (2.8) months. The frequency of alexithymia was higher in CIS patients than in controls, with a prevalence of 42% (P<0.0001). Alexithymia correlated with anxiety and depression but not with cognition. Alexithymia was dependent only on depression (P=0.003). CONCLUSION: Alexithymia, characterized by difficulty identifying feelings, is present in patients in the early stage of MS, and seems to be strongly associated with depression. Difficulty in social interaction could be a risk of future affective disorders.
Assuntos
Sintomas Afetivos , Transtornos de Ansiedade , Sintomas Afetivos/epidemiologia , Ansiedade , Cognição , Emoções , HumanosRESUMO
Coronavirus disease 2019 (COVID-19) is a viral infection caused by the Severe Acute Respiratory Syndrome coronavirus 2 (SARS-CoV-2), which spreads rapidly from person to person and manifests in most symptomatic patients as a respiratory illness, similar to prior SARS viruses. Neurologic manifestations of COVID-19 are uncommon; those so far reported include encephalopathy, stroke from large-vessel occlusion, and polyneuropathy. We report a unique neurologic complication of COVID-19 in a patient who had extensive cerebral small-vessel ischemic lesions resembling cerebral vasculitis in a characteristic combined imaging pattern of ischemia, hemorrhage, and punctuate postcontrast enhancement. Also, a characteristic lower extremity skin rash was present in our patient. Our observation lends support to the increasingly suspected mechanism of "endotheliitis" associated with this novel coronavirus.
Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Pneumonia Viral/complicações , Vasculite do Sistema Nervoso Central/diagnóstico por imagem , Idoso , COVID-19 , Humanos , Imageamento por Ressonância Magnética , Masculino , Imagem Multimodal , Pandemias , SARS-CoV-2 , Tomografia Computadorizada por Raios X , Vasculite do Sistema Nervoso Central/etiologiaRESUMO
BACKGROUND AND PURPOSE: Follow-up MR imaging of brain AVMs currently relies on contrast-enhanced sequences. Noncontrast techniques, including arterial spin-labeling and TOF, may have value in detecting a residual nidus after radiosurgery. The aim of this study was to compare noncontrast with contrast-enhanced MR imaging for the differentiation of residual-versus-obliterated brain AVMs in radiosurgically treated patients. MATERIALS AND METHODS: Twenty-eight consecutive patients with small brain AVMs (<20 mm) treated by radiosurgery were followed with the same MR imaging protocol. Three neuroradiologists, blinded to the results, independently reviewed the following: 1) postcontrast images alone (4D contrast-enhanced MRA and postcontrast 3D T1 gradient recalled-echo), 2) arterial spin-labeling and TOF images alone, and 3) all MR images combined. The primary end point was the detection of residual brain AVMs using a 5-point scale, with DSA as the reference standard. RESULTS: The highest interobserver agreement was for arterial spin-labeling/TOF (κ = 0.81; 95% confidence interval, 0.66-0.93). Regarding brain AVM detection, arterial spin-labeling/TOF had higher sensitivity (sensitivity, 85%; specificity, 100%; 95% CI, 62-97) than contrast-enhanced MR imaging (sensitivity, 55%; specificity, 100%; 95% CI, 27-73) and all MR images combined (sensitivity, 75%; specificity, 100%; 95% CI, 51-91) (P = .008). All nidus obliterations on DSA were detected on MR imaging. In 6 patients, a residual brain AVM present on DSA was only detected with arterial spin-labeling/TOF, including 3 based solely on arterial spin-labeling images. CONCLUSIONS: In this study of radiosurgically treated patients with small brain AVMs, arterial spin-labeling/TOF was found to be superior to gadolinium-enhanced MR imaging in detecting residual AVMs.
Assuntos
Malformações Arteriovenosas Intracranianas/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Neuroimagem/métodos , Radiocirurgia/métodos , Adolescente , Adulto , Idoso , Angiografia Digital/métodos , Feminino , Seguimentos , Gadolínio , Humanos , Malformações Arteriovenosas Intracranianas/radioterapia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Marcadores de SpinRESUMO
BACKGROUND AND PURPOSE: Myelitis is common, related to multiple aetiologies and constitute in some cases a differential diagnosis for spinal cord tumors. Our objective was to review the clinical and paraclinical aspects of the main aetiologies of myelitis. METHODS: These aetiologies will be reviewed based on data not only from the scientific literature but also from our personal experience reported in different cohorts of patients. RESULTS: Multiple sclerosis is the main cause of partial myelitis in young adults. Neuromyelitis optica is now a well-known specific entity frequently revealed by a transverse myelitis. The diagnosis is based on specific criteria, including the presence of anti-NMO antibodies. In our cohorts, approximately 12 % of the patients admitted for an acute or subacute myelitis were related to infections, mainly of a viral origin. Patients with myelitis must be screened for systemic diseases. As for neuromyelitis optica, patients with myelitis related to a systemic disease should be treated in emergency. Acute myelitis is sometimes the first symptom of a systemic lupus or of a sarcoidosis. Sjögren syndrome can mimic myelitis related to primary progressive multiple sclerosis. Spinal cord imaging contributes greatly to defining the myelitis. CONCLUSION: In most cases, a routine clinical and paraclinical examination and the follow-up of the patients can contribute to establishing the aetiology of a myelitis.
Assuntos
Mielite/diagnóstico , Mielite/etiologia , Neoplasias da Medula Espinal/diagnóstico , Diagnóstico Diferencial , Humanos , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/etiologia , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/etiologiaRESUMO
Antibodies against conformation-dependent epitopes of myelin-oligodendrocyte-glycoprotein (MOG-abs) are present in subgroups of neuromyelitis optica spectrum disorder (NMOSD), recurrent optic neuritis (rON), multiple sclerosis (MS), and anti-NMDAR encephalitis. Using optical coherence tomography (OCT) we assessed whether MOG-abs might serve as potential marker of retinal axonal degeneration. We investigated a clinically heterogeneous cohort of 13 MOG-abs-positive patients (4 MOG-abs-positive rON, 4 MOG-abs-positive adult MS, 3 MOG-abs-positive relapsing encephalomyelitis, 2 MOG-abs-positive aquaporin-4-abs-negative NMOSD). As controls, we studied 13 age, sex and ON episode(s)-matched MOG-abs and aquaporin-4-abs-negative (AQP4-abs-negative) MS patients and 13 healthy controls (HC). In addition, we investigated 19 unmatched AQP4-abs-positive MOG-abs-negative NMOSD subjects. Considering all eyes, global pRNFL [in µm, mean (SD)] was significantly reduced in MOG-abs-positive patients [72.56 (22.71)] compared to MOG-abs-negative MS [80.81 (13.55), p = 0.0128], HCs [103.54 (8.529), p = 0.0014] and NMOSD [88.32 (18.43), p = 0.0353]. Non ON eyes from MOG-abs-positive subjects showed significant subclinical atrophy of temporal pRNFL quadrants. Microcystic macular edema (MME) was observed only in eyes of MOG-abs-positive (24%) and AQP4-abs-positive NMOSD (5.6%), but not in MOG-abs-negative MS or HC (p < 0.01). MOG-abs may serve as potential marker of retinal degeneration. Specifically, MOG-abs-related OCT features predominate in temporal pRNFL quadrants (resembling the MS retinal pattern), might be more severe than AQP4-abs-positive NMOSD, indicate subclinical pathology, and may be associated with MME.
Assuntos
Autoanticorpos/sangue , Glicoproteína Mielina-Oligodendrócito/imunologia , Neurite Óptica/diagnóstico por imagem , Neurite Óptica/imunologia , Retina/diagnóstico por imagem , Adulto , Encefalite Antirreceptor de N-Metil-D-Aspartato/diagnóstico por imagem , Encefalite Antirreceptor de N-Metil-D-Aspartato/imunologia , Aquaporina 4/imunologia , Biomarcadores/metabolismo , Encefalomielite/diagnóstico por imagem , Encefalomielite/imunologia , Feminino , Seguimentos , Humanos , Edema Macular/diagnóstico por imagem , Edema Macular/imunologia , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla Recidivante-Remitente/diagnóstico por imagem , Esclerose Múltipla Recidivante-Remitente/imunologia , Neuromielite Óptica/diagnóstico por imagem , Neuromielite Óptica/imunologia , Nervo Óptico/diagnóstico por imagem , Tomografia de Coerência ÓpticaRESUMO
OBJECTIVES: To assess the association between optic nerve double inversion recovery (DIR) hypersignal length and retinal axonal loss in neuroinflammatory diseases affecting optic nerves. METHODS: We recruited patients previously affected (> 6 months) by a clinical episode of optic neuritis (ON). We had 25 multiple sclerosis (MS) patients, eight neuromyelitis optica spectrum disorder (NMOSD) patients and two patients suffering from idiopathic caused ON undergo brain magnetic resonance imaging (MRI); including a 3-dimensional (3D) DIR sequence, optical coherence tomography (OCT) examination and visual disability evaluation. Evaluation criteria were retinal thickness/volume, optic nerve DIR hypersignal length and high/low contrast vision acuity. RESULTS: In the whole cohort, we found good associations (< 0.0001) between optic nerve DIR hypersignal length, peripapillary retinal nerve fiber layer thickness, inner macular layers volumes, and visual disability. We found subclinical radiological optic nerve involvement in 38.5% of non-ON MS eyes. CONCLUSIONS: Optic nerve DIR hypersignal length may be a biomarker for retinal axonal loss, easily applicable in routine and research on new anti-inflammatory or neuroprotective drug evaluation. Detection of subclinical ON with 3D-DIR in a non-negligible proportion of MS patients argues in favor of optic nerve imaging in future OCT MS studies, in order to achieve a better understanding of retinal axonal loss in non-ON eyes.
Assuntos
Esclerose Múltipla/patologia , Fibras Nervosas/patologia , Neuromielite Óptica/patologia , Nervo Óptico/patologia , Neurite Óptica/patologia , Retina/patologia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neurite Óptica/diagnóstico , Células Ganglionares da Retina/patologia , Tomografia de Coerência Óptica/métodosRESUMO
We investigated the brain magnetic susceptibility changes induced by natalizumab-associated progressive multifocal leukoencephalopathy. We retrospectively included 12 patients with natalizumab-progressive multifocal leukoencephalopathy, 5 with progressive multifocal leukoencephalopathy from other causes, and 55 patients with MS without progressive multifocal leukoencephalopathy for comparison. MR imaging examinations included T2* or SWI sequences in patients with progressive multifocal leukoencephalopathy (86 examinations) and SWI in all patients with MS without progressive multifocal leukoencephalopathy. Signal abnormalities on T2* and SWI were defined as low signal intensity within the cortex and/or U-fibers and the basal ganglia. We observed T2* or SWI signal abnormalities at the chronic stage in all patients with progressive multifocal leukoencephalopathy, whereas no area of low SWI signal intensity was detected in patients without progressive multifocal leukoencephalopathy. Among the 8 patients with asymptomatic natalizumab-progressive multifocal leukoencephalopathy, susceptibility changes were observed in 6 (75%). The basal ganglia adjacent to progressive multifocal leukoencephalopathy lesions systematically appeared hypointense by using T2* and/or SWI. Brain magnetic susceptibility changes may be explained by the increased iron deposition and constitute a useful tool for the diagnosis of progressive multifocal leukoencephalopathy.
Assuntos
Fatores Imunológicos/efeitos adversos , Leucoencefalopatia Multifocal Progressiva/induzido quimicamente , Leucoencefalopatia Multifocal Progressiva/diagnóstico , Natalizumab/efeitos adversos , Idoso , Anticorpos Monoclonais Humanizados/efeitos adversos , Encéfalo/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética/efeitos adversos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosRESUMO
BACKGROUND: Retinal optical coherence tomography (OCT) permits quantification of retinal layer atrophy relevant to assessment of neurodegeneration in multiple sclerosis (MS). Measurement artefacts may limit the use of OCT to MS research. OBJECTIVE: An expert task force convened with the aim to provide guidance on the use of validated quality control (QC) criteria for the use of OCT in MS research and clinical trials. METHODS: A prospective multi-centre (n = 13) study. Peripapillary ring scan QC rating of an OCT training set (n = 50) was followed by a test set (n = 50). Inter-rater agreement was calculated using kappa statistics. Results were discussed at a round table after the assessment had taken place. RESULTS: The inter-rater QC agreement was substantial (kappa = 0.7). Disagreement was found highest for judging signal strength (kappa = 0.40). Future steps to resolve these issues were discussed. CONCLUSION: Substantial agreement for QC assessment was achieved with aid of the OSCAR-IB criteria. The task force has developed a website for free online training and QC certification. The criteria may prove useful for future research and trials in MS using OCT as a secondary outcome measure in a multi-centre setting.
Assuntos
Esclerose Múltipla/patologia , Retina/patologia , Tomografia de Coerência Óptica/normas , Atrofia/patologia , Humanos , Estudos Prospectivos , Controle de QualidadeRESUMO
INTRODUCTION: Cerebrotendinous xanthomatosis, a metabolic leukodystrophy with an autosomal recessive inheritance, is secondary to deficiency of sterol 27-hydroxylase, an enzyme involved in cholesterol catabolism. Classical symptoms include clinical or infraclinical xanthomas affecting the skin and tendons, early cataracts, neurological signs and diarrhea. Brain imaging reveals involvement of the dentate nuclei and periventricular white matter hyperintensities. The diagnosis is based on an increased cholestanol level in serum, confirmed by the presence of a mutation in the CYP27A1 gene. Treatment is based on chenodeoxycholic acid. METHOD: We report a retrospective multicentric study of 15 cases of cerebrotendinous xanthomatosis diagnosed in French adults. Clinical, molecular and MRI findings were recorded in all patients. RESULTS: The average age at diagnosis was 39years (range 27-65). Disease onset occurred in childhood in 73% of patients and in adulthood in 27%. All patients with a pediatric onset were diagnosed during adulthood (age range 28-65years). Clinical symptoms variably associated cerebellar syndrome, pyramidal syndrome, cognitive decline, epilepsy, neuropathy (sought in 10 of our patients, present in forms in 8), psychiatric disorders, cataract and xanthomas. One patient had an atypical presentation: monoparesis associated with xanthomas. Brain MRI was abnormal in all: findings consisted in T2-weighted hyperintensity of the dentate nuclei (47%), periventricular leuoencephalopathy (73%) which preferentially involved the posterior cerebral part (60%), leucoencephalopathy with a vascular pattern (7%), hyperintensity of the cortico-spinal tracts (53%), globi pallidi, corpus callosum and cerebral atrophy (33%). Serum cholestanol was elevated in 93% of patients. The most frequent mutation was 1183C>T (n=5/15). Under treatment with chenodeoxycholic acid, eight patients improved initially, followed by stabilization in five of them, and worsening in the others. Four patients died. CONCLUSION: Patients with the xanthoma-neurological disorder association should be tested for cerebrotendinous xanthomatosis. The disease often begins in childhood with a diagnostic delay but also in adulthood. Involvement of the dentate nuclei is specific but not sensitive and the supratentorial leucoencephalopathy is not specific but with an antero-posterior gradient. A vascular distribution and involvement of the corpus callosum are possible. Serum cholestanol assay is very reliable: an elevated level provides the diagnosis, which must nevertheless be confirmed by molecular biology.
Assuntos
Xantomatose Cerebrotendinosa , Adulto , Idade de Início , Idoso , Substituição de Aminoácidos , Encéfalo/patologia , Ácido Quenodesoxicólico/uso terapêutico , Colestanotriol 26-Mono-Oxigenase/deficiência , Colestanotriol 26-Mono-Oxigenase/genética , Feminino , Genes Recessivos , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Mutação de Sentido Incorreto , Estudos Retrospectivos , Avaliação de Sintomas , Xantomatose Cerebrotendinosa/tratamento farmacológico , Xantomatose Cerebrotendinosa/epidemiologia , Xantomatose Cerebrotendinosa/patologiaRESUMO
BACKGROUND AND PURPOSE: BIONAT is a French multicentric phase IV study of natalizumab (NTZ)-treated relapsing-remitting multiple sclerosis (MS) patients. The purpose of this study was to collect clinical, radiological and biological data on 1204 patients starting NTZ, and to evaluate the clinical/radiological response to NTZ after 2 years of treatment. METHODS: Patients starting NTZ at 18 French MS centres since June 2007 were included. Good response to NTZ was defined by the absence of clinical and radiological activity. Data analysed in this first report on the BIONAT study focus on patients who started NTZ at least 2 years ago (n = 793; BIONAT2Y ). RESULTS: NTZ was discontinued in 17.78% of BIONAT2Y. The proportion of patients without combined disease activity was 45.59% during the first two successive years of treatment. Systematic dosage of anti-NTZantibodies (Abs) detected only two supplementary patients with anti-NTZ Abs compared with strict application of recommendations. A significant decrease of IgG,M concentrations at 2 years of treatment was found. CONCLUSIONS: The efficacy of NTZ therapy on relapsing-remitting MS in a real life setting is confirmed in the BIONAT cohort. The next step will be the identification of biomarkers predicting response to NTZ therapy and adverse events.
Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Vigilância de Produtos Comercializados , Adulto , Estudos de Coortes , Feminino , Humanos , Masculino , Natalizumab , Estudos ProspectivosRESUMO
BACKGROUND: Current treatment options for first-line immunotherapy in relapsing-remitting multiple sclerosis (MS) are recombinant interferon-ß and glatiramer acetate. However, these therapies are only partially effective and certain patients may fail to respond. For this reason, it is important to elaborate alternative treatment strategies. Induction therapy represents a more aggressive approach in which powerful drugs are used right from the beginning to tackle the disease process hard and early. Natalizumab is a powerful monoclonal antibody approved for the treatment of relapsing-remitting MS and is known to silence disease activity. METHODS: We describe here the early outcome at 1 month and at 6 months of three patients treated with natalizumab for relapsing-remitting MS. RESULTS: All three patients had a high disease activity before the initiation of natalizumab, with 4, 8 and 5 gadolinium-enhancing lesions on brain MRI respectively. On the MRI scans made at 1 month after the first infusion, and at 6 months, there was no more gadolinium-enhancement and no new T2-lesion. Clinically, they did not experience any relapse. DISCUSSION: In these three cases, natalizumab showed a dramatic efficacy: the patients became "disease activity free" right from the first infusion. To our knowledge, natalizumab is not classically used as an induction therapy, unlike mitoxantrone. However, this treatment has potential hematological and cardiac toxicity and its use can be limited. Thus, in JC virus negative patients, natalizumab could be an interesting alternative treatment. CONCLUSION: Our report suggests that induction strategy with natalizumab may be applicable in patients with aggressive multiple sclerosis. A study of more similar cases may be interesting to confirm these preliminary results.
Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Quimioterapia de Indução/métodos , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Adolescente , Adulto , Feminino , Humanos , Imageamento por Ressonância Magnética , Esclerose Múltipla Recidivante-Remitente/diagnóstico , Natalizumab , Adulto JovemRESUMO
BACKGROUND AND PURPOSE: Therapeutic strategies for patients with MS partly rely on contrast-enhanced MR imaging. Our aim was to assess the diagnostic performance of 3D turbo spin-echo MR imaging with variable refocusing flip angles at 3T for the detection of enhanced inflammatory lesions in patients with multiple sclerosis. MATERIALS AND METHODS: Fifty-six patients with MS were prospectively investigated by using postcontrast T1-weighted axial 2D spin-echo and 3D TSE MR images. The order in which both sequences were performed was randomized. Axial reformats from 3D T1 TSE were generated to match the 2D spin-echo images. The reference standard was defined by using clinical data and all MR images available. Three separate sets of MR images (2D spin-echo images, axial reformats, and multiplanar images from 3D TSE sequences) were examined in a blinded fashion by 2 neuroradiologists separately for the detection of enhanced MS lesions. Image artifacts and contrast were evaluated. RESULTS: No artifacts related to vascular pulsation were observed on 3D TSE images, whereas image artifacts were demonstrated on 2D spin-echo images in 41 patients. One hundred twelve enhanced MS lesions were identified in 19 patients. Sixty-four lesions were correctly diagnosed by using 2D spin-echo images; 90, by using 3D TSE axial reformatted views; and 106, by using multiplanar analysis of the 3D TSE sequence. Multiplanar analysis was 94.7% sensitive and 100% specific for the diagnosis of patients with at least 1 enhanced lesion. Contrast of enhanced MS lesions was significantly improved by using the 3D TSE sequence (P < .011). CONCLUSIONS: The 3D TSE sequence with multiplanar analysis is a useful tool for the detection of enhanced MS lesions.
Assuntos
Encefalite/diagnóstico , Imageamento Tridimensional , Imageamento por Ressonância Magnética , Esclerose Múltipla/diagnóstico , Neuroimagem/métodos , Adulto , Precisão da Medição Dimensional , Encefalite/etiologia , Feminino , Humanos , Aumento da Imagem , Imageamento por Ressonância Magnética/métodos , Masculino , Esclerose Múltipla/complicações , Estudos ProspectivosRESUMO
INTRODUCTION: Anti-Hu antibody syndrome is a paraneoplastic syndrome usually associated with small cell lung carcinoma which induces various symptoms, particularly neurological ones. CASE REPORT: We describe the case of a 49-year old woman with a small cell lung carcinoma who initially experiences a spontaneous regression but then developed neurological symptoms associated with severe autonomic dysfunction manifesting as chronic intestinal pseudo-obstruction and leading finally to hemodynamic failure. CONCLUSION: Anti-Hu antibody syndrome remains a rare entity. Its diagnosis must be considered in the face of neurologic symptoms associated with small cell lung cancer.
Assuntos
Proteínas ELAV/imunologia , Síndromes Paraneoplásicas/diagnóstico , Síndromes Paraneoplásicas/terapia , Anticorpos Antinucleares/fisiologia , Diagnóstico Diferencial , Evolução Fatal , Feminino , Humanos , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/imunologia , Pessoa de Meia-Idade , Síndromes Paraneoplásicas/etiologia , Carcinoma de Pequenas Células do Pulmão/complicações , Carcinoma de Pequenas Células do Pulmão/imunologia , Fumar/efeitos adversosRESUMO
BACKGROUND: Neuromyelitis optica (NMO) is an inflammatory disease associated with optic neuritis and myelitis. Recently, several studies showed that optical coherence tomography (OCT) could be an interesting method for the evaluation of disease severity; however, to date there are no studies with a longitudinal follow-up of visual function in NMO. The aim of this study was to assess the ability of OCT to evaluate the progression of visual dysfunction in NMO. PATIENTS AND METHODS: A group of 30 NMO patients (thus, 60 eyes), comprised of 20 women and 10 men with a mean age of 43.7 +/- 12.3 years, were prospectively evaluated clinically and by a whole neuro-ophthalmological work-up, including: visual acuity (VA), fundoscopy, visual evoked potential (VEP), visual field (VF) and optical coherence tomography (OCT). All patients were tested at baseline (after a mean disease duration of 6.1 years) and after a mean time of follow-up of 18 months (range: 12-36 months). RESULTS: Mean VA was similar at the two evaluation times (0.77 +/- 0.36 versus 0.77 +/- 0.35). The mean VF defect decreased slightly, but the difference was not significant (-5.9 +/- 1.3 dB versus -5.3 +/- 1.3 dB). In contrast, the mean retinal thickness seen on OCT decreased from 87.4 +/- 23.3 µm to 79.7 +/- 22.4 µm (p = 0.006). These modifications were only observed in eyes with a past or a recent history of optic neuritis (-15.1 µm; p < 0.001) and not in eyes without any history of optic neuritis (-2.4 µm; not significant). Also, they occurred independently of the occurrence of relapses (n = 13) and especially optic neuritis episodes; however, the number of optic neuritis episodes was low (n = 5). CONCLUSION: OCT seems to be a more sensitive test than VA or VF for monitoring ophthalmological function in NMO and it seems to be helpful for the detection of infra-clinical episodes in patients with a past history of optic neuritis. Our results suggest that this easily performed technique should be used in the follow-up of NMO, but complementary studies are warranted to confirm its interest at an individual level.
Assuntos
Neuromielite Óptica/complicações , Tomografia de Coerência Óptica , Transtornos da Visão/diagnóstico , Acuidade Visual/fisiologia , Adulto , Estudos de Coortes , Potenciais Evocados Visuais/fisiologia , Feminino , Seguimentos , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Transtornos da Visão/etiologia , Campos Visuais/fisiologiaRESUMO
BACKGROUND AND PURPOSE: DTI is a promising technique for imaging of the spinal cord, but the technique has susceptibility-induced artifacts. We evaluated a pulse-triggered DTI sequence with an rFOV technique and coronal acquisition for the assessment of the cervical spinal cord in patients with myelitis at 3T. MATERIALS AND METHODS: A rFOV acquisition was established by a noncoplanar application of the excitation and the refocusing pulse in conjunction with outer volume suppression. The DTI sequence was performed in the coronal plane in 12 healthy volunteers and 40 consecutive patients with myelitis. Probabilistic tractography of the posterior and lateral funiculi was performed from the C1 to C7 levels. FA, MD, aD, rD, and ratios of aD and rD were measured. RESULTS: In healthy volunteers, mean DTI indices within the whole-fiber pathways were the following: FA = 0.61, MD = 1.17 × 10(-3) mm(2)/s, aD = 1.96 × 10(-3) mm(2)/s, rD = 0.77 × 10(-3) mm(2)/s, and ratios of aD and rD = 2.5. Comparison of healthy controls and patients with myelitis identified statistically significant differences for all DTI parameters. Different patterns of myelitis, including spinal cord atrophy and active inflammatory lesions, were recognized. There was a significant correlation between clinical severity and DTI parameters. CONCLUSIONS: The present work introduces a new approach for DTI of the cervical spinal cord at 3T, enabling a quantitative follow-up of patients with myelitis.
Assuntos
Algoritmos , Vértebras Cervicais/patologia , Imagem de Tensor de Difusão/métodos , Aumento da Imagem/métodos , Interpretação de Imagem Assistida por Computador/métodos , Mielite/patologia , Adulto , Feminino , Humanos , Masculino , Reprodutibilidade dos Testes , Sensibilidade e EspecificidadeRESUMO
OBJECTIVE: The purpose of our study was to assess the influence of pregnancy on the course of neuromyelitis optica (NMO) and the impact of epidural analgesia and breastfeeding on its activity in the postpartum period. METHODS: We performed a retrospective study of patients with NMO diagnosed according to Wingerchuk criteria. We noted the number of relapses during the year before pregnancy (BP), during pregnancy (first trimester, second trimester, third trimester), and the year after (Y + 1: first trimester, second trimester [PP2], and third and fourth trimesters postpartum). Epidural analgesia and breastfeeding were recorded. Disability was evaluated with the Kurtzke Expanded Disability Status Scale (EDSS). The annualized relapse rate (ARR) was calculated. RESULTS: We identified 124 patients (85 female) in the French NOMADMUS cohort on November 1, 2010. A total of 20 women (including 25 pregnancies) were informative with complete files. Comparisons between the ARR of each period and BP (1.0 ± 0.09) only showed an increased tendency for PP2 (0.8 ± 0.06, p = 0.07). Epidural analgesia and breastfeeding had no influence on the course of NMO. The EDSS score increased from 1.5 ± 1.7 BP to 2.6 ± 1.9 Y + 1 (p = 0.027). CONCLUSION: This study shows that pregnancy influences the activity of NMO, a finding that justifies close medical monitoring. We found no evidence to suggest that either epidural analgesia or breastfeeding has an aggravating effect on NMO.