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Mielite transversa aguda idiopática
ORPHA:139423CID-10 · G37.3CID-11 · 8A41.0DOENÇA RARA

Mielite transversa aguda idiopática (ATM) é uma doença inflamatória desmielinizante mediada pelo sistema imunitário da espinal medula com envolvimento motor, sensorial e autónomo.

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Introdução

O que você precisa saber de cara

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Mielite transversa aguda idiopática (ATM) é uma doença inflamatória desmielinizante mediada pelo sistema imunitário da medula espinhal com envolvimento motor, sensorial e autônomo.

Publicações científicas
27 artigos
Último publicado: 2024 Jul 1

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
All ages
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: G37.3
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (2)
0202010694
Sequenciamento completo do exoma (WES)genetic_test
0301070040
Atendimento em reabilitação — doenças rarasrehabilitation
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa2desde 2024
Total histórico27PubMed
Últimos 10 anos6publicações
Pico20151 papers
Linha do tempo
2024Hoje · 2026
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Nenhum gene associado encontrado

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Diagnóstico

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Tratamento e manejo

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·Pré-clínico4
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 4 ensaios
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Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Mielite transversa aguda idiopática

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Ensaios clínicos abertos e novidades científicas recentes

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Distribuição por fase
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Publicações mais relevantes

Timeline de publicações
6 papers (10 anos)
#1

Comparison between MRI-negative and positive results and the predictors for a poor prognosis in patients with idiopathic acute transverse myelitis.

BMC neurology2024 Jul 01

Idiopathic acute transverse myelitis (IATM) is a focal inflammatory disorder of the spinal cord that results in motor, sensory, and autonomic dysfunction. However, the comparative analysis of MRI-negative and MRI-positive in IATM patients were rarely reported. The purpose of this study was to compare MRI-negative with MRI-positive groups in IATM patients, analyze the predictors for a poor prognosis, thus explore the relationship between MRI-negative and prognosis. We selected 132 patients with first-attack IATM at the First Affiliated Hospital of Nanchang University from May 2018 to May 2022. Patients were divided into MRI-positive and MRI-negative group according to whether there were responsible spinal MRI lesions, and good prognosis and poor prognosis based on whether the EDSS score ≥ 4 at follow-up. The predictive factors of poor prognosis in IATM patients was analyzed by logistic regression models. Of the 132 patients, 107 first-attack patients who fulfilled the criteria for IATM were included in the study. We showed that 43 (40%) patients had a negative spinal cord MRI, while 27 (25%) patients were identified as having a poor prognosis (EDSS score at follow-up ≥ 4). Compared with MRI-negative patients, the MRI-positive group was more likely to have back/neck pain, spinal cord shock and poor prognosis, and the EDSS score at follow-up was higher. We also identified three risk factors for a poor outcome: absence of second-line therapies, high EDSS score at nadir and a positive MRI result. Compared with MRI-negative group, MRI-positive patients were more likely to have back/neck pain, spinal cord shock and poor prognosis, with a higher EDSS score at follow-up. The absence of second-line therapies, high EDSS score at nadir, and a positive MRI were risk factors for poor outcomes in patients with first-attack IATM. MRI-negative patients may have better prognosis, an active second-line immunotherapy for IATM patients may improve clinical outcome.

#2

Idiopathic acute transverse myelitis in a middle-aged woman with progression to nadir in less than one hour.

BMJ case reports2021 Jun 04

We report a case of a middle-aged woman, normally fit and well, presenting with acute onset neurological deficit with progression to nadir in <1 hour. Initial MRI spine showed no significant abnormality, although second MRI spine showed abnormal signal in three to four segments with no compressive lesion. CT aortic angiography excluded vascular or ischaemic abnormality. We made a diagnosis of idiopathic acute transverse myelitis (ATM). She was treated with steroids and made significant progress improving from T11 ASIA A paraplegia to T11 ASIA C paraplegia by the time of discharge. Awareness of idiopathic ATM presenting hyperacutely with initial MRI spine being normal is important for prompt diagnosis and management.

#3

Prognostic Parameters of Acute Transverse Myelitis in Children.

Journal of child neurology2020 Dec

Acute transverse myelitis is a rare and disabling disorder. Data on the imaging features in children are sparse. The aim of this study was to describe the clinical and magnetic resonance imaging findings characteristic of pediatric idiopathic acute transverse myelitis and to identify those with prognostic value. The database of a tertiary pediatric medical center was retrospectively reviewed for patients aged less than 18 years who were diagnosed in 2002-2017 with acute transverse myelitis that was not associated with recurrence of a demyelinating autoimmune event. Data were collected on clinical, laboratory, and imaging findings and outcome. A total of 23 children (11 male, 12 female) met the study criteria. Mean age at disease onset was 10 years, and mean duration of follow-up was 6 years 10 months. Spinal cord and brain magnetic resonance imaging scans were performed on admission or shortly thereafter. The most common finding was cross-sectional involvement, in 16 patients (70%). The mean number of involved spinal segments was 8. The most frequently involved region was the thoracic spine, in 17 patients (74%). Clinical factors predicting good prognosis were cerebrospinal fluid pleocytosis, absence of tetraparesis, and prolonged time to nadir. In conclusion, most children with acute transverse myelitis appear to have a good outcome. Prompt diagnosis and treatment are important. Further research is needed in a larger sample to evaluate the predictive value of imaging features.

#4

Etiologic spectrum and prognosis in noncompressive acute transverse myelopathies: An experience of 80 patients at a tertiary care facility.

Neurology India2018

We evaluated the spectrum of acquired demyelinating and inflammatory disorders in patients presenting with an acute transverse myelopathy. We also studied differences between an acute idiopathic transverse myelitis and myelitis resulting from other etiologies. Eighty consecutive patients with acute transverse myelopathy were included. At inclusion, clinical profile, serum and cerebrospinal fluid parameters, brain and spinal cord magnetic resonance imaging, and visual evoked potentials were obtained. All patients were given methylprednisolone therapy. Patients were followed up for 6 months. Outcome was assessed using modified Barthel index. A modified Barthel index score of ≤12 indicated a poor prognosis. Majority (n = 49; 61.25%) of patients had idiopathic acute transverse myelitis. Eleven cases had neuromyelitis optica spectrum disorders (8 had anti-aquaporin antibody positivity). Multiple sclerosis was diagnosed in 7 cases. Eight cases had infectious or parainfectious myelitis. Longitudinally extensive transverse myelitis was noted in 66 (82.5%) patients. Seventeen patients had abnormalities in the brain. Majority of patients improved following methylprednisolone therapy. On univariate analysis, delay in administering methylprednisolone therapy, poor modified Barthel index at discharge, and extensive cord involvement were associated with severe residual disability. On multivariate analysis, delayed initiation of methylprednisolone was identified as a poor prognostic factor. A variety of inflammatory, infective, demyelinating, and autoimmune disorders present with acute transverse myelopathy. Early institution of methylprednisolone reduces the disability in these patients.

#5

A pediatric patient of hemorrhagic acute transverse myelitis.

Brain &amp; development2017 Mar

An 11-year-old boy presented with progressive leg hypesthesia but no history of trauma. Dysuria and constipation appeared subsequent to gait difficulty. He was admitted 8days after onset. Spinal magnetic resonance imaging (MRI) revealed longitudinal hyperintensity with cord swelling and hypointensity on T2-weighted images, suggesting severe inflammation and microbleeding change, respectively. Gadolinium contrast-enhanced MRI demonstrated mild enhancement in the lesions. Platelet count and coagulation findings were normal, and cerebrospinal fluid analysis showed no pleocytosis. He was diagnosed with idiopathic acute transverse myelitis (ATM), and intravenous methylprednisolone pulse therapy and plasmapheresis were initiated. On day 14, motor dysfunction aggravated suddenly, accompanied by expanding hemorrhagic lesions. Thereafter, administration of intravenous immunoglobulin, repeated intravenous methylprednisolone pulse therapy and prednisolone for one month resulted in complete recovery four months later. Both anti-aquaporin-4 and anti-myelin oligodendrocyte glycoprotein antibodies were negative. We presented the first pediatric case showing hemorrhagic spinal lesions in the clinical course of ATM. This severe complication should be recognized in the management of ATM.

Publicações recentes

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Comparison between MRI-negative and positive results and the predictors for a poor prognosis in patients with idiopathic acute transverse myelitis.
    BMC neurology· 2024· PMID 38951761mais citado
  2. Idiopathic acute transverse myelitis in a middle-aged woman with progression to nadir in less than one hour.
    BMJ case reports· 2021· PMID 34088683mais citado
  3. Prognostic Parameters of Acute Transverse Myelitis in Children.
    Journal of child neurology· 2020· PMID 32808576mais citado
  4. Etiologic spectrum and prognosis in noncompressive acute transverse myelopathies: An experience of 80 patients at a tertiary care facility.
    Neurology India· 2018· PMID 29322962mais citado
  5. A pediatric patient of hemorrhagic acute transverse myelitis.
    Brain &amp; development· 2017· PMID 27686688mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:139423(Orphanet)
  2. MONDO:0015344(MONDO)
  3. GARD:19914(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55785411(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Mielite transversa aguda idiopática
Compêndio · Raras BR

Mielite transversa aguda idiopática

ORPHA:139423 · MONDO:0015344
Prevalência
Unknown
Herança
Not applicable
CID-10
G37.3 · Mielite transversa aguda em doenças desmielinizantes do sistema nervoso central
CID-11
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C1719404
EuropePMC
Wikidata
Papers 10a
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