Multiple complete ring-shaped enhanced MRI lesions in acute disseminated encephalomyelitis

Lim, Keunpoong; Hsu, Yu‐Chao; Hsu, Wei-Kang; Chan, Cheng Yi

doi:10.1016/s0899-7071(02)00552-1

Cited by 29 publications

(15 citation statements)

References 8 publications

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“…Two patients were pediatric. As previously reported in pediatric patients, ADEM patients from our series presented sub cortical white matter aggression, which progressed as isolated acute encephalitis, multifocal myelitis or both, and polysymptomatic neurologic symptoms 10,11,13,30 . The optic neuritis, transverse demyelinating myelitis, like Devic's syndrome 14 , hemiparesia, ataxia and brainstem syndromes were the neurological syndromes seen in our ADEM cases, resembling MS and NMo symptoms.…”

Section: Discussionsupporting

confidence: 77%

Acute disseminated encephalomyelitis: clinical features, HLA DRB11501, HLA DRB11503, HLA DQA10102, HLA DQB10602, and HLA DPA1*0301 allelic association study

Alves-Leon

Veluttini-Pimentel

Gouveia

et al. 2009

Arq. Neuro-Psiquiatr.

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-We evaluated the frequency, demographic, clinical, disability evolution and genetic association of HLA DRB1*1501, DRB1*1503, DQA1*0102, DQB1*0602 and DPA1*0301 alleles in patients diagnosed as acute disseminated encephalomyelitis (ADEM) among a population of CNS demyelinating diseases. Fifteen patients (8.4%) of our series were diagnosed as ADEM. The mean age onset was 35.23 years (range 12 to 77), 53.3% were male and follow-up range was 8.5 to 16 years. Two cases (13.3%) had a preceding infection before neurological symptoms, one presented a parainfectious demyelinating, and one case had been submitted to hepatitis B vaccination four weeks before the clinical onset. The EDSS range was 3.0 to 9.5. Eight patients (53.3%) presented MRI with multiple large lesions. CSF was normal in 73.3%. The severe disability observed at EDSS onset improved in 86.66% patients. The genetic susceptibility for ADEM was significantly associated with the HLA DQB1*0602, DRB1*1501 and DRB1*1503 alleles (<0.05) in monophasic ADEM.KEy WoRDS: ADEM, HLA class II, demyelinating disease.Encefalomielite aguda disseminada: características clínicas e estudo de associação com os alelos HLA DRB1*1501, HLA DRB1*1503, HLA DQA1*0102, HLA DQB1*0602 e DPA1*0301Resumo -Avaliamos as frequencia, características demográficas, clínicas e de associação genética dos alelos HLA DRB1*1501, DRB1*1503, DQA1*0102, DQB1*0602 e DPA1*0301 em pacientes com diagnóstico de encefalomielite aguda disseminada (ADEM) em população com doença desmielinizante do SNC. Quinze (8,4%) pacientes de nossa série foram diagnosticados como ADEM. A média de idade foi 35,23 anos (variando entre 12 e 77), 53,3% eram homens e o tempo de acompanhamento variou entre 8,5 e 16 anos. Dois casos (13,3%) apresentaram infecção prévia, um apresentou processo desmielinizante para infeccioso e outro havia se submetido a vacinação para hepatite B quatro semanas antes. o EDSS variou entre 3,0 e 9,5. oito pacientes (53,3%) apresentaram grandes lesões na RM. o LCR foi normal em 73,3%. A incapacidade grave quantificada pelo EDSS foi seguida de melhora importante em 86,6% dos pacientes. A susceptibilidade genética na ADEM foi significativamente associada com os alelos HLA DQB1*0602, DRB1*1501 e DRB1*1503 (p<0,05) nos pacientes com quadro monofásico. PALAVRAS-CHAVE: ADEM, HLA classe II, doenças desmielinizantes.

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Section: Discussionsupporting

confidence: 77%

Acute disseminated encephalomyelitis: clinical features, HLA DRB11501, HLA DRB11503, HLA DQA10102, HLA DQB10602, and HLA DPA1*0301 allelic association study

Alves-Leon

Veluttini-Pimentel

Gouveia

et al. 2009

Arq. Neuro-Psiquiatr.

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“…Involvement of deep gray matter nuclei may also be seen [2]. In ADEM, the disease characteristically involves more than one of the above mentioned sites in an asymmetrical fashion, and the cerebral white matter is the most frequent target [1][2][3][4][5][6]. The presented case had unusual lesions confined to the mesencephalon.…”

Section: Discussionmentioning

confidence: 71%

“…Infrequently, hemorrhage and calcification are present and gadolinium enhancement may be observed as seen in the presented case. Nodular, diffuse or ring pattern of contrast enhancement may be observed [1][2][3]. Different degrees of enhancement in the same patient, if present, represent various levels of damage to the bloodbrain barrier.…”

Section: Discussionmentioning

confidence: 99%

“…Different degrees of enhancement in the same patient, if present, represent various levels of damage to the bloodbrain barrier. The damage may in part depend on the age of lesions [3].…”

Section: Discussionmentioning

confidence: 99%

“…The differential diagnosis of brainstem lesions includes abscess, tuberculomas, toxoplasmosis, histoplasmosis, metastases, multiple sclerosis, diffuse mesencephalic gliomas, encephalitis, resolving hematoma and vascular malformations [3,5]. In many cases, medical history, laboratory studies and sequential MRI are crucial for allowing the differentiation of ADEM.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

An unusual case of acute disseminated encephalomyelitis confined to brainstem

Fırat

Karakaş

Yakıncı

et al. 2004

Magnetic Resonance Imaging

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Progressive cavitating leukoencephalopathy: A novel childhood disease

Naidu

Bibat

Lin

et al. 2005

Annals of Neurology

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We report 19 patients with a previously undelineated neurodegenerative syndrome characterized by episodic acute onset of irritability or neurological deficits between 2 months and 3.5 years of age, followed by steady or intermittent clinical deterioration. Seven children died between 11 months and 14 years of age. Cranial magnetic resonance imaging (MRI) shows patchy leukoencephalopathy with cavities, and vascular permeability, in actively affected regions. Early lesions affect corpus callosum and centrum semiovale, with or without cerebellar or cord involvement. After repeated episodes, areas of tissue loss coalesce with older lesions to become larger cystic regions in brain or spinal cord. Diffuse spasticity, dementia, vegetative state, or death ensues. Gray matter is spared until late in the course. In some, incomplete clinical or MRI recovery occurs after episodes. The clinical course varies from rapid deterioration to prolonged periods of stability that are unpredictable by clinical or MRI changes. Elevated levels of lactate in brain, blood, and cerebrospinal fluid, abnormal urine organic acids, and changes in muscle respiratory chain enzymes are present but inconsistent, without identifiable mitochondrial DNA mutations or deletions. Pathological studies show severe loss of myelin sparing U-fibers, axonal disruption, and cavitary lesions without inflammation. Familial occurrence and consanguinity suggest autosomal recessive inheritance of this distinct entity.

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Multiple complete ring-shaped enhanced MRI lesions in acute disseminated encephalomyelitis

Cited by 29 publications

References 8 publications

Acute disseminated encephalomyelitis: clinical features, HLA DRB11501, HLA DRB11503, HLA DQA10102, HLA DQB10602, and HLA DPA1*0301 allelic association study

Acute disseminated encephalomyelitis: clinical features, HLA DRB11501, HLA DRB11503, HLA DQA10102, HLA DQB10602, and HLA DPA1*0301 allelic association study

An unusual case of acute disseminated encephalomyelitis confined to brainstem

Progressive cavitating leukoencephalopathy: A novel childhood disease

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Multiple complete ring-shaped enhanced MRI lesions in acute disseminated encephalomyelitis

Cited by 29 publications

References 8 publications

Acute disseminated encephalomyelitis: clinical features, HLA DRB1*1501, HLA DRB1*1503, HLA DQA1*0102, HLA DQB1*0602, and HLA DPA1*0301 allelic association study

Acute disseminated encephalomyelitis: clinical features, HLA DRB1*1501, HLA DRB1*1503, HLA DQA1*0102, HLA DQB1*0602, and HLA DPA1*0301 allelic association study

An unusual case of acute disseminated encephalomyelitis confined to brainstem

Progressive cavitating leukoencephalopathy: A novel childhood disease

Contact Info

Product

Resources

About

Acute disseminated encephalomyelitis: clinical features, HLA DRB11501, HLA DRB11503, HLA DQA10102, HLA DQB10602, and HLA DPA1*0301 allelic association study

Acute disseminated encephalomyelitis: clinical features, HLA DRB11501, HLA DRB11503, HLA DQA10102, HLA DQB10602, and HLA DPA1*0301 allelic association study