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1.
Semin Ultrasound CT MR ; 44(5): 424-435, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37555684

RESUMO

Myelitis is an extensive group of pathologies, including inflammatory, demyelinating, and infectious disorders, sometimes mimicking tumors. This article will discuss infectious myelitis, mainly the patterns of spinal cord involvement caused by each infectious agent and the contribution of magnetic resonance imaging as a major tool to establish the specific diagnosis.


Assuntos
Mielite Transversa , Mielite , Humanos , Mielite/diagnóstico por imagem , Mielite/etiologia , Diagnóstico Diferencial , Imageamento por Ressonância Magnética/métodos , Mielite Transversa/complicações , Mielite Transversa/diagnóstico , Mielite Transversa/patologia , Medula Espinal/diagnóstico por imagem
2.
Rofo ; 195(12): 1081-1096, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37479218

RESUMO

BACKGROUND: Differential diagnosis of non-compressive cervical myelopathy encompasses a broad spectrum of inflammatory, infectious, vascular, neoplastic, neurodegenerative, and metabolic etiologies. Although the speed of symptom onset and clinical course seem to be specific for certain neurological diseases, lesion pattern on MR imaging is a key player to confirm diagnostic considerations. METHODS: The differentiation between acute complete transverse myelitis and acute partial transverse myelitis makes it possible to distinguish between certain entities, with the latter often being the onset of multiple sclerosis. Typical medullary MRI lesion patterns include a) longitudinal extensive transverse myelitis, b) short-range ovoid and peripheral lesions, c) polio-like appearance with involvement of the anterior horns, and d) granulomatous nodular enhancement prototypes. RESULTS AND CONCLUSION: Cerebrospinal fluid analysis, blood culture tests, and autoimmune antibody testing are crucial for the correct interpretation of imaging findings. The combination of neuroradiological features and neurological and laboratory findings including cerebrospinal fluid analysis improves diagnostic accuracy. KEY POINTS: · The differentiation of medullary lesion patterns, i. e., longitudinal extensive transverse, short ovoid and peripheral, polio-like, and granulomatous nodular, facilitates the diagnosis of myelitis.. · Discrimination of acute complete and acute partial transverse myelitis makes it possible to categorize different entities, with the latter frequently being the overture of multiple sclerosis (MS).. · Neuromyelitis optica spectrum disorders (NMOSD) may start as short transverse myelitis and should not be mistaken for MS.. · The combination of imaging features and neurological and laboratory findings including cerebrospinal fluid analysis improves diagnostic accuracy.. · Additional brain imaging is mandatory in suspected demyelinating, systemic autoimmune, infectious, paraneoplastic, and metabolic diseases..


Assuntos
Esclerose Múltipla , Mielite Transversa , Poliomielite , Animais , Humanos , Mielite Transversa/diagnóstico por imagem , Mielite Transversa/patologia , Diagnóstico Diferencial , Esclerose Múltipla/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos
3.
Mult Scler Relat Disord ; 67: 104068, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35933757

RESUMO

BACKGROUND: Transverse myelitis (TM) occurs isolated or within other acquired demyelinating syndromes (ADS) such as neuromyelitis optica spectrum disorders (NMOSD), multiple sclerosis (MS) or myelin oligodendrocyte glycoprotein antibody associated disorders (MOGAD). OBJECTIVE: To describe and compare clinical and MRI features of children with ADS presenting with TM grouped according to antibody status and diagnosis of MS and NMOSD. PATIENTS AND METHODS: Children with TM, radiological involvement of the myelon, MOG and aquaporin-4 antibody status were elegible. RESULTS: 100 children were identified and divided into MOGAD (n=33), NMOSD (n=7), double seronegative TM (n=34), and MS (n=26). MOGAD children had mainly acute disseminated encephalomyelitis + TM/ longitudinally extensive TM (LETM) (42%) or isolated LETM (30%). In MOGAD, LETM was present in more than half of all children (55%) with predominant involvement of only the grey matter (73%). Leptomeningeal enhancement was highly predictive of MOGAD (16/30; p=0.003). In MS patients spinal MRI showed single (50%) or multiple short lesions (46%) with involvement of grey and white matter (68%). Double seronegative children presented with LETM (74%) and brain lesions were less frequent compared to the other groups (30%). CONCLUSION: Children with ADS presenting with TM reveal important radiological differences such as LETM with predominant involvement of spinal grey matter and leptomeningeal enhancement in MOGAD.


Assuntos
Esclerose Múltipla , Mielite Transversa , Neuromielite Óptica , Humanos , Mielite Transversa/patologia , Glicoproteína Mielina-Oligodendrócito , Aquaporina 4 , Síndrome , Imageamento por Ressonância Magnética , Autoanticorpos
4.
J Korean Med Sci ; 37(7): e52, 2022 Feb 21.
Artigo em Inglês | MEDLINE | ID: mdl-35191229

RESUMO

Acute transverse myelitis (ATM) has been reported as rare complication of vaccination. Herein, we report 2 cases of ATM after the administration of an mRNA vaccine for coronavirus disease 2019 (COVID-19). The first one is an 81-year-old man who received the BNT162b2 vaccine. He presented with bilateral hand weakness. Spine magnetic resonance imaging (MRI) showed high signal intensity from the C1 to C3 vertebrae. The second is a 23-year-old woman who received the BNT162b2 vaccine and experienced tingling in her legs. Spine MRI showed a high signal intensity lesion at the conus medullaris. These patients were treated with intravenous methylprednisolone and their symptoms improved slightly. Careful follow-up is needed to identify adverse events after the administration of mRNA vaccines for COVID-19.


Assuntos
Vacina BNT162/efeitos adversos , Mãos/fisiopatologia , Perna (Membro)/fisiopatologia , Mielite Transversa/patologia , Medula Espinal/fisiopatologia , Vacinação/efeitos adversos , Idoso de 80 Anos ou mais , Vacina BNT162/imunologia , COVID-19/imunologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Metilprednisolona/uso terapêutico , Mielite Transversa/diagnóstico , Mielite Transversa/tratamento farmacológico , SARS-CoV-2/imunologia , Coluna Vertebral/diagnóstico por imagem , Adulto Jovem
5.
J Neurol ; 269(6): 3175-3179, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-34999955

RESUMO

INTRODUCTION: Isolated spinal cord angiitis (ISCA) is very rare disease. But, it is frequently encountered in the differential diagnosis of atypical spinal cord syndromes. CASE PRESENTATION AND REVIEW OF THE LITERATURE: We present a 31-year-old male who presented with progressive paraparesis, and diagnosed with pathologically confirmed ISCA. Longitudinal cystic transverse myelitis was documented in spinal MRI. He responded well to cyclophosphamide and steroid combination, and no relapse was noted during the 4-year follow-up. A standard systematic analysis of the germane literature disclosed 15 more ISCA cases. In total 16 cases (mean age: 46.5, 10 males), ISCA was diagnosed with pathological evaluation in all (Biopsy in 11, Autopsy in 5). MRI lesion is characterized by usually multisegmental longitudinal and sometimes cystic expansile lesions. In seven cases, it was described as "(pseudo)tumoral" by the authors. Albeit absence of elevation of CSF protein/WBC or "compatible" spinal MRI lesion may aid to exclude ISCA to some extent, pathological confirmation is currently necessary for the diagnosis. In 11 cases, ISCA was treated similar to primary supratentorial vasculitis. Mortality rate is 31%. DISCUSSION: ISCA diagnosis, a typical example of which we have presented here, can only be established by tissue examination. However, noninvasive diagnostic criteria are critically needed. Our data suggest that this can only be possible with multinational multicenter prospective registry.


Assuntos
Mielite Transversa , Doenças da Medula Espinal , Vasculite do Sistema Nervoso Central , Adulto , Humanos , Imageamento por Ressonância Magnética , Masculino , Estudos Multicêntricos como Assunto , Mielite Transversa/patologia , Recidiva Local de Neoplasia/patologia , Medula Espinal/diagnóstico por imagem , Medula Espinal/patologia , Doenças da Medula Espinal/diagnóstico por imagem , Doenças da Medula Espinal/patologia , Vasculite do Sistema Nervoso Central/diagnóstico por imagem , Vasculite do Sistema Nervoso Central/tratamento farmacológico
6.
Mult Scler ; 28(11): 1663-1666, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-33635151

RESUMO

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory demyelinating disorder of the central nervous system (CNS). Aquaporin-4 (AQP4) antibodies in the serum are highly specific for the diagnosis of NMOSD, but the sensitivity remains under 90% allowing for diagnosis of AQP4 IgG seronegative disease. It remains of crucial importance to identify seronegative NMOSD myelitis as early as the first attack to initiate long-term treatment that will reduce future relapses and disability and to avoid potentially harmful treatments such as those of multiple sclerosis (MS). Over the years, many spinal imaging features have been reported to favour the diagnosis of NMOSD, but only longitudinally extensive transverse myelitis (LETM) was specific enough to make the diagnostic criteria in the AQP4 IgG seronegative cases. Bright spotty lesions (BSLs), which are defined as hyperintense lesions on axial T2-weighted images and sometimes associated with T1 low signal, are now reported to have a higher specificity and sensitivity compared to LETM in predicting a diagnosis of NMOSD against other causes of myelitis. In the review, we aim to highlight the position of BSLs in diagnosing NMOSD as well as its possible role as a prognostic factor for the clinical outcome.


Assuntos
Mielite Transversa , Neuromielite Óptica , Aquaporina 4 , Autoanticorpos , Biomarcadores , Humanos , Imunoglobulina G , Mielite Transversa/patologia , Recidiva Local de Neoplasia/complicações
7.
J Neuroimmunol ; 361: 577739, 2021 12 15.
Artigo em Inglês | MEDLINE | ID: mdl-34628134

RESUMO

We report the case of a patient with symptoms of myelopathy following acute SARS-CoV-2 infection. MRI documented a longitudinally extensive transverse myelitis and further investigation was unremarkable with the exception of positivity for MOG-IgG in serum. This report extends the spectrum of post-COVID-19 neurological syndromes, and documents a very significant improvement to long-term oral corticosteroid therapy in this setting. Further prospective studies are needed to establish the risk of recurrence in this subset of patients.


Assuntos
Autoanticorpos/imunologia , COVID-19/complicações , Glicoproteína Mielina-Oligodendrócito/imunologia , Mielite Transversa/virologia , Adulto , Autoantígenos/imunologia , COVID-19/imunologia , Humanos , Masculino , Mielite Transversa/imunologia , Mielite Transversa/patologia , SARS-CoV-2
8.
Front Immunol ; 12: 653786, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33981305

RESUMO

Introduction: Although acute transverse myelitis (ATM) is a rare neurological condition (1.34-4.6 cases per million/year) COVID-19-associated ATM cases have occurred during the pandemic. Case-finding methods: We report a patient from Panama with SARS-CoV-2 infection complicated by ATM and present a comprehensive clinical review of 43 patients with COVID-19-associated ATM from 21 countries published from March 2020 to January 2021. In addition, 3 cases of ATM were reported as serious adverse events during the clinical trials of the COVID-19 vaccine ChAdOx1 nCoV-19 (AZD1222). Results: All patients had typical features of ATM with acute onset of paralysis, sensory level and sphincter deficits due to spinal cord lesions demonstrated by imaging. There were 23 males (53%) and 20 females (47%) ranging from ages 21- to 73- years-old (mean age, 49 years), with two peaks at 29 and 58 years, excluding 3 pediatric cases. The main clinical manifestations were quadriplegia (58%) and paraplegia (42%). MRI reports were available in 40 patients; localized ATM lesions affected ≤3 cord segments (12 cases, 30%) at cervical (5 cases) and thoracic cord levels (7 cases); 28 cases (70%) had longitudinally-extensive ATM (LEATM) involving ≥4 spinal cord segments (cervicothoracic in 18 cases and thoracolumbar-sacral in 10 patients). Acute disseminated encephalomyelitis (ADEM) occurred in 8 patients, mainly women (67%) ranging from 27- to 64-years-old. Three ATM patients also had blindness from myeloneuritis optica (MNO) and two more also had acute motor axonal neuropathy (AMAN). Conclusions: We found ATM to be an unexpectedly frequent neurological complication of COVID-19. Most cases (68%) had a latency of 10 days to 6 weeks that may indicate post-infectious neurological complications mediated by the host's response to the virus. In 32% a brief latency (15 hours to 5 days) suggested a direct neurotropic effect of SARS-CoV-2. The occurrence of 3 reported ATM adverse effects among 11,636 participants in the AZD1222 vaccine trials is extremely high considering a worldwide incidence of 0.5/million COVID-19-associated ATM cases found in this report. The pathogenesis of ATM remains unknown, but it is conceivable that SARS-CoV-2 antigens -perhaps also present in the AZD1222 COVID-19 vaccine or its chimpanzee adenovirus adjuvant- may induce immune mechanisms leading to the myelitis.


Assuntos
Vacinas contra COVID-19/efeitos adversos , COVID-19/complicações , Mielite Transversa/complicações , SARS-CoV-2/patogenicidade , Adolescente , Adulto , Idoso , ChAdOx1 nCoV-19 , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mielite Transversa/diagnóstico , Mielite Transversa/patologia , Mielite Transversa/fisiopatologia , Doenças do Sistema Nervoso/complicações , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/patologia , Doenças do Sistema Nervoso/fisiopatologia , SARS-CoV-2/imunologia , SARS-CoV-2/fisiologia , Medula Espinal/diagnóstico por imagem , Medula Espinal/patologia , Medula Espinal/fisiopatologia , Tropismo Viral , Adulto Jovem
9.
J Neuroimmunol ; 353: 577523, 2021 04 15.
Artigo em Inglês | MEDLINE | ID: mdl-33640717

RESUMO

OBJECTIVE: To report a unique case and literature review of post COVID-19 associated transverse myelitis and dysautonomia with abnormal MRI and CSF findings. BACKGROUND: Coronavirus disease have been reported to be associated with several neurological manifestations such as stroke, Guillain-Barré syndrome, meningoencephalitis amongst others. There are only few reported cases of transverse myelitis with the novel coronavirus (n-CoV-2) and only one reported case identifying dysautonomia in COVID-19 patient. Here, we identify a COVID-19 patient diagnosed with acute transverse myelitis in addition to dysautonomia following with complete resolution of symptoms. METHOD: A retrospective chart review of a patient diagnosed with post SARS-CoV-2 infection acute transverse myelitis and dysautonomia, and a review of literature of all the reported cases of transverse myelitis and COVID-19, from December 1st, 2019 till December 25th, 2020, was performed. CONCLUSION: To our knowledge, this is the first reported case of transverse myelitis and dysautonomia in a patient with SARS-CoV-2 infection, who responded to intravenous methyl prednisone and bromocriptine. Follow-up imaging of the spine showed complete resolution of the lesion. Further studies would be recommended to identify the underlying correlation between COVID-19 and transverse myelitis.


Assuntos
COVID-19/complicações , Mielite Transversa/virologia , Disautonomias Primárias/virologia , Medula Espinal/patologia , Adulto , Anti-Inflamatórios/uso terapêutico , Bromocriptina/uso terapêutico , COVID-19/patologia , Agonistas de Dopamina/uso terapêutico , Humanos , Masculino , Metilprednisolona/uso terapêutico , Mielite Transversa/tratamento farmacológico , Mielite Transversa/patologia , SARS-CoV-2
10.
Brain Dev ; 43(5): 626-636, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33509615

RESUMO

OBJECTIVE: It may be difficult for clinicians to estimate the prognosis of pediatric acute transverse myelitis (ATM). The aim of this study was to define prognostic factors for relapsing disease and poor outcome in pediatric ATM. METHODS: This prospective cohort study included 49 children, 18 boys and 31 girls (median age 13.1 years, IQR 6.5-16.2) with a first episode of ATM. Factors associated with relapsing disease and poor outcome (Expanded Disability Status Scale (EDSS) ≥ 4) were assessed during a median follow-up of 37 months (IQR 18-75). RESULTS: In total, 14 patients (29%) experienced ≥ 1 relapse(s) and nine patients (18%) had a poor outcome. Factors at onset associated with relapsing disease included higher age (16.1 vs. 11.6 years, p = 0.002), longer time to maximum severity of symptoms (5.5 vs. 3 days, p = 0.01), lower maximum EDSS score (4.0 vs. 6.5, p = 0.003), short lesion on spinal MRI (64 vs. 21%, p = 0.006), abnormalities on brain MRI (93 vs. 44%, p = 0.002) and presence of oligoclonal bands in cerebrospinal fluid (67 vs. 14%, p = 0.004). The only factor associated with poor outcome was presence of a spinal cord lesion on MRI without cervical involvement (56 vs. 14%, p = 0.02). CONCLUSION: Pediatric ATM patients presenting with clinical, radiological and laboratory features associated with multiple sclerosis (MS) are at risk for relapsing disease. In absence of these known MS risk factors at onset of disease these patients are at low risk for relapses. Only a minority of pediatric ATM patients in this cohort have a poor outcome.


Assuntos
Esclerose Múltipla/diagnóstico , Mielite Transversa/diagnóstico , Doença Aguda , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Esclerose Múltipla/metabolismo , Esclerose Múltipla/patologia , Esclerose Múltipla/fisiopatologia , Mielite Transversa/metabolismo , Mielite Transversa/patologia , Mielite Transversa/fisiopatologia , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/metabolismo , Neuromielite Óptica/patologia , Neuromielite Óptica/fisiopatologia , Avaliação de Resultados em Cuidados de Saúde , Prognóstico , Recidiva
11.
Trop Doct ; 51(1): 117-119, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32933380

RESUMO

Transverse myelitis typically extends two or less spinal segments, whereas longitudinal extensive transverse myelitis (LETM) extends three or more spinal segments in length and may occasionally span all the segments of the spinal cord. We present a case of spinal tuberculosis presenting with LETM with true lower motor neuron-type flaccid paraplegia.


Assuntos
Mielite Transversa/etiologia , Paraplegia/etiologia , Tuberculose da Coluna Vertebral/complicações , Adulto , Humanos , Imageamento por Ressonância Magnética , Masculino , Neurônios Motores/patologia , Mielite Transversa/diagnóstico por imagem , Mielite Transversa/patologia , Mielite Transversa/fisiopatologia , Paraplegia/diagnóstico por imagem , Paraplegia/patologia , Paraplegia/fisiopatologia , Tuberculose da Coluna Vertebral/diagnóstico por imagem , Tuberculose da Coluna Vertebral/patologia , Tuberculose da Coluna Vertebral/fisiopatologia
12.
Ideggyogy Sz ; 73(7-08): 275-285, 2020 Jul 30.
Artigo em Inglês | MEDLINE | ID: mdl-32750245

RESUMO

Background and aims - Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports - Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion - Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.


Assuntos
Encéfalo/patologia , Linfoma/patologia , Mielite Transversa/patologia , Biópsia , Evolução Fatal , Feminino , Cefaleia/etiologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Paraparesia/etiologia
13.
Neurol Res ; 42(7): 612-617, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32497470

RESUMO

BACKGROUND/AIMS: Both of neuromyelitis optica spectrum disease (NMOSDs) and idiopathic transverse myelitis (ITM) could present as acute transverse myelitis. However, long-term immunological treatment and prognosis are different for high recurrence of NMOSDs. In this study, we summarized clinical differences between acute attack myelitis of NMOSDs and ITM, we further screened serum auto-antibodies to help understand the two distinct clinical entities. METHODS: This is a retrospective study on 48 NMOSD patients and 49 ITM patients in neurological department of Nanjing Drum Tower Hospital from 2013 to 2019. Clinical, CSF and MRI profiles on the acute episode were also compared between NMOSD patients and ITM patients. Serum AQP4 and auto-antibodies were tested. Clinical parameters were further compared between NMOSD patients with and without auto-antibodies. RESULTS: Compared with ITM patients, NMOSD patients manifested with longer vertebral segments (5.42 ± 3.17 segments vs. 2.31 ± 2.36 segments, p < 0.001), higher female/male ratio (13:3 vs. 20:29, p < 0.001), higher IgG index (30.30% vs. 9.09%, p < 0.05). Positive rates of anti-Ro-52 (47.92% vs. 14.29%, p < 0.001), anti-ANAs (50.00% vs.10.20%, p < 0.001) and anti-SSA (35.42% vs. 6.12%, p = 0.001) were significantly higher in the NMOSD patients than the ITM patients. Seropositive Ro-52 and SSA were associated with longer injured spinal cord segments. However, Ro-52 antibody may not be associated with NMOSD relapsing during our follow up. CONCLUSIONS: NMOSD patients manifested with longer vertebral segments, higher female/male ratio, IgG index, anti-ANAs, anti-Ro-52 and anti-SSA seroprevalence than ITM patients. These features may help clinicians better distinguish NMOSD from ITM and provide long-term immunotherapy reasonably.


Assuntos
Mielite Transversa/diagnóstico , Mielite Transversa/patologia , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/patologia , Adulto , Idoso , Autoanticorpos/sangue , Autoanticorpos/imunologia , Autoantígenos/imunologia , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mielite Transversa/imunologia , Neuromielite Óptica/imunologia , Estudos Retrospectivos
14.
J Neuroinflammation ; 17(1): 128, 2020 Apr 23.
Artigo em Inglês | MEDLINE | ID: mdl-32326965

RESUMO

BACKGROUND: Longitudinally extensive transverse myelitis (LETM) is classically related to aquaporin (AQP4)-antibodies (Ab) neuromyelitis optica spectrum disorders (NMOSD) or more recently to myelin oligodendrocyte glycoprotein (MOG)-Ab associated disease. However, some patients remain negative for any diagnosis, despite a large work-up including AQP4-Ab and MOG-Ab. Data about natural history, disability outcome, and treatment are limited in this group of patients. We aimed to (1) describe clinical, biological, and radiological features of double seronegative LETM patients; (2) assess the clinical course and identify prognostic factors; and (3) assess the risk of recurrence, according to maintenance immunosuppressive therapy. METHODS: Retrospective evaluation of patients with a first episode of LETM, tested negative for AQP-Ab and MOG-Ab, from the French nationwide observatory study NOMADMUS. RESULTS: Fifty-three patients (median age 38 years (range 16-80)) with double seronegative LETM were included. Median nadir EDSS at onset was 6.0 (1-8.5), associated to a median EDSS at last follow-up of 4.0 (0-8). Recurrence was observed in 24.5% of patients in the 18 following months, with a median time to first relapse of 5.7 months. The risk of recurrence was lower in the group of patients treated early with an immunosuppressive drug (2/22, 9%), in comparison with untreated patients (10/31, 32%). CONCLUSIONS: A first episode of a double seronegative LETM is associated to a severe outcome and a high rate of relapse in the following 18 months, suggesting that an early immunosuppressive treatment may be beneficial in that condition.


Assuntos
Mielite Transversa/imunologia , Mielite Transversa/patologia , Recuperação de Função Fisiológica , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Aquaporina 4/imunologia , Autoanticorpos/sangue , Autoanticorpos/imunologia , Estudos de Coortes , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Glicoproteína Mielina-Oligodendrócito/imunologia , Mielite Transversa/terapia , Plasmaferese/métodos , Prognóstico , Recidiva , Fatores de Risco , Adulto Jovem
15.
Mult Scler Relat Disord ; 41: 102030, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-32145694

RESUMO

We describe a case of an unusually early and severe manifestation of Aquaporin-4 (AQP-4) positive Neuromyelitis Optica Spectrum Disorder (NMOSD) in a two-year-old girl. We discuss learning points from her clinical presentation and highlight differences between pediatric and adult presentations of the disease. We argue that AQP-4 NMOSD should always be considered in the differential diagnosis for any child presenting with an acute neuroimmunological process given the morbidity associated with the condition and the importance of early diagnosis and treatment.


Assuntos
Aquaporina 4/imunologia , Mielite Transversa/diagnóstico , Pré-Escolar , Feminino , Humanos , Imageamento por Ressonância Magnética , Mielite Transversa/imunologia , Mielite Transversa/patologia , Mielite Transversa/fisiopatologia , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/imunologia , Neuromielite Óptica/patologia , Neuromielite Óptica/fisiopatologia
16.
Mult Scler Relat Disord ; 37: 101432, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32172999

RESUMO

Neuromyelitis optica (NMO) is a disease characterised by severe relapses of optic neuritis and longitudinally extensive transverse myelitis and it has a strong female predilection. Pain is one of the most typical symptom in NMO. However, few studies have been conducted to examine the neuropathic pain mechanism of NMO patients or gender-specific effects using magnetic resonance imaging technique. A total of 38 female patients with NMO, 28 with pain (NMOWP) and 10 without pain (NMOWoP), were classified using the Brief Pain Inventory (BPI); 22 healthy females were also recruited. We used the FSL Image Registration and Segmentation Toolbox (FIRST) for subcortical region volumes quantifications, and voxel-based morphometry analysis for cortical gray matter (GM) volume, to examine the brain morphology in NMOWP patients. In addition, correlation test between structural measurements of NMO patients and clinical indexes was also performed. The results showed: 1) no significant differences in cortical GM density between the NMOWP and NMOWoP groups; 2) significantly smaller hippocampus and pallidum volumes in the NMOWP group compared with the NMOWoP group; 3) significant negative correlation between the average BPI and volumes of the accumbens nucleus and thalamus in NMO patients. These results revealed that structural abnormality exists in NMO female patients who have pain, with significant implications for our understanding of the brain morphology in NMO patients with pain.


Assuntos
Mielite Transversa/patologia , Neuralgia/etiologia , Neuromielite Óptica/patologia , Adulto , Atrofia/complicações , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Neuromielite Óptica/complicações
17.
BMC Neurol ; 20(1): 13, 2020 Jan 10.
Artigo em Inglês | MEDLINE | ID: mdl-31924167

RESUMO

BACKGROUND: Transverse myelitis (TM) is due to inflammatory spinal cord injury with bilateral neurologic involvement, which is sensory, motor, or autonomic in nature. It may be associated with autoimmune disease, vaccination, intoxication and infections. The most common infection cause of TM is Coxsackie virus and Mycoplasma pneumoniae. The cryptococcosis is rare. We present the case of disseminated cryptococcosis revealed by transverse myelitis in an immunocompetent 55-year-old male patient. The literature review is also stated. CASE PRESENTATION: The 55-year-old man suffered from gradual numbness, weakness in both lower limbs and finally paralyzed in the bed. The thoracic spine Computed tomography (CT) was normal, but multiple nodules in the lung were accidentally discovered. Thoracic Magnetic Resonance Imaging (MRI) showed diffused thoracic spinal cord thickening and extensively intramedullary T2 hyper intensity areas. Gadolinium contrast enhanced T1WI showed an intramedullary circle-enhanced nodule at 9th thoracic level. Diagnosis was made by histological examination of the bilateral lung biopsy. The patient was treated successfully with systemic amphotericin B liposome and fluconazole and intrathecal dexamethasone and amphotericin B liposome. CONCLUSIONS: This is a patient with disseminated cryptococcosis involving the lung, spinal cord and adrenal glands, which is rare in the absence of immunodeficiency.


Assuntos
Criptococose/complicações , Mielite Transversa/microbiologia , Antifúngicos/uso terapêutico , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Mielite Transversa/tratamento farmacológico , Mielite Transversa/patologia , Tomografia Computadorizada por Raios X
18.
Mult Scler Relat Disord ; 37: 101487, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31707235

RESUMO

BACKGROUND: Determining the frequency of longitudinally-extensive transverse myelitis (LETM: T2-lesion ≥3 vertebral segments) in multiple sclerosis (MS) is essential to assess its utility in differentiating from aquaporin-4-IgG (AQP4-IgG) positive neuromyelitis optica spectrum disorder (NMOSD) and myelin-oligodendrocyte-glycoprotein-IgG (MOG-IgG) myelitis. We sought to determine the frequency of LETM in MS during a myelitis attack. METHODS: We identified Olmsted County (MN, USA) residents on 12/31/2011 with inflammatory demyelinating disease. Inclusion criteria were: 1) Clinical myelitis episode accompanied by a new spinal magnetic resonance imaging (MRI) lesion (≤6 weeks from onset); 2) MS diagnosis by 2010 McDonald criteria; 3) Seronegative for AQP4-IgG and MOG-IgG. MRI characteristics were determined. RESULTS: Sixty-seven patients (median age at myelitis: 41 years [range, 16-65]; 76% females) with 92 myelitis attacks accompanied by a new MRI spinal cord lesion were identified. The frequency of LETM was 0%. The median T2-hyperintense lesion length in vertebral segments was 1.0 (range, 0.5-2.5) and 82/92 (89%) were peripheral in location on axial sequences; 58% had associated gadolinium enhancement. Two patients (2% of attacks) had multiple short lesions resembling LETM on sagittal images but axial sequences confirmed multiple non-contiguous short lesions. CONCLUSION: LETM is rare in adult MS myelitis and its presence should prompt evaluation for AQP4-IgG, MOG-IgG or other etiologies. Careful scrutiny of axial images is important as coalescence of multiple short lesions may lead to the artifactual appearance of an LETM.


Assuntos
Esclerose Múltipla , Mielite Transversa , Adolescente , Adulto , Idoso , Aquaporina 4/imunologia , Bancos de Espécimes Biológicos , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Minnesota/epidemiologia , Esclerose Múltipla/diagnóstico por imagem , Esclerose Múltipla/epidemiologia , Esclerose Múltipla/imunologia , Esclerose Múltipla/patologia , Glicoproteína Mielina-Oligodendrócito/imunologia , Mielite Transversa/diagnóstico por imagem , Mielite Transversa/epidemiologia , Mielite Transversa/imunologia , Mielite Transversa/patologia , Adulto Jovem
19.
Mult Scler ; 26(8): 997-1000, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-31621483

RESUMO

BACKGROUND: Longitudinally extensive transverse myelitis (LETM) accompanying systemic lupus erythematosus (SLE) is often due to coexisting aquaporin-4-IgG seropositive neuromyelitis optica spectrum disorder but has not been associated with myelin oligodendrocyte glycoprotein-IgG (MOG-IgG). OBJECTIVE AND METHODS: Case report at an academic medical center. RESULTS: A 32-year-old woman developed severe transverse myelitis (paraplegia) shortly after SLE onset in the post-partum period. Magnetic resonance imaging (MRI) revealed an LETM, cerebrospinal fluid showed marked inflammation, and testing for infections was negative. Serum live-cell-based assay for MOG-IgG was positive but aquaporin-4-IgG was negative. CONCLUSION: In patients with SLE and LETM, MOG-IgG testing should be considered, in addition to AQP4-IgG.


Assuntos
Autoanticorpos/sangue , Lúpus Eritematoso Sistêmico/diagnóstico , Glicoproteína Mielina-Oligodendrócito/imunologia , Mielite Transversa/diagnóstico , Transtornos Puerperais/diagnóstico , Adulto , Aquaporina 4/imunologia , Feminino , Humanos , Imunoglobulina G , Imageamento por Ressonância Magnética , Mielite Transversa/sangue , Mielite Transversa/imunologia , Mielite Transversa/patologia , Transtornos Puerperais/sangue , Transtornos Puerperais/imunologia , Transtornos Puerperais/patologia
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