Encephalitis Periaxialis Concentrica

J, Balo

doi:10.1001/archneurpsyc.1928.02210080044002

Cited by 160 publications

(65 citation statements)

References 7 publications

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“…Restricted diffusion in the outer rings was demonstrated in axial diffusionweighted imaging ( Figure 1d). The typical pattern is consistent with a diagnosis of Baló's concentric sclerosis (BCS), which was first described by Marburg in 1906, and was advanced by Jószef Baló as a rare variant of multiple sclerosis in 1928 [1]. The patient was treated with intravenous methyl prednisolone (1000 mg/day) for 5 days followed by a reducing dose of oral prednisolone.…”

supporting

confidence: 64%

Baló's Concentric Sclerosis

Wei¹,

Chen²

2015

J Neu neurol

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A 38-year-old woman had a one-month history of progressively dysphasia, dysphagia and mild cognitive impairment. Previous medical history was unremarkable. Neurologic examination revealed a pseudobulbar palsy with a moderate dysarthria and dysfunction of cranial nerves IX, X and XII. Laboratory investigations disclosed a normal complete blood cell count, biochemistry and the results of the serologic tests including antineutrophil cytoplasmic antibodies and antinuclear antibodies, besides an elevated C-active protein level (13.1mg/l) and an increased erythrocyte sedimentation rate (80 mm/hr). The results of cerebrospinal fluid examination were normal with an immunoglobulin G index of 0.412 and negative oligoclonal bands. Axial magnetic resonance imaging (MRI) scans showed multiple lesions in a concentric ring pattern consisting of alternating layers of higher and lower signal intensity in the both centrum semiovale (Figure 1). The lesions were typically irregular concentric areas of iso and low signal in T1-weighted imaging (Figure 1a) while iso and high signal in T2-weighted imaging ( Figure 1b). There was no or mild focal, peripheral enhancement in the lesions with contrast enhancement in the ( Figure 1c). Restricted diffusion in the outer rings was demonstrated in axial diffusionweighted imaging ( Figure 1d). The typical pattern is consistent with a diagnosis of Baló's concentric sclerosis (BCS), which was first described by Marburg in 1906, and was advanced by Jószef Baló as a rare variant of multiple sclerosis in 1928 [1]. The patient was treated with intravenous methyl prednisolone (1000 mg/day) for 5 days followed by a reducing dose of oral prednisolone. Her condition improved dramatically. On follow-up examination two years later, she was completely recovered with normal neurological examination and no further relapses were reported. Unfortunately, no further MR images of this patient are available.BCS occurs more in young adults and is more common in Asiatic populations than in Caucasoids. It is histopathologically characterized by larger white matter lesions with concentric alternating layers of myelin preservation and loss, which present with onion-like appearance with a minor mass effect on MRI [2,3]. It may occur as solitary lesions, along with the plaques typically seen in multiple sclerosis. In the differential diagnosis, other demyelinating lesions such as acute disseminated encephalomyelitis and gliomas should be considered. Clinicians used to regard its prognosis as similar to that of Marburg multiple sclerosis, which has a monophasic rapidly progressive course with a fatal outcome. Recently, an increasing number of cases have been described as having prolonged survival, spontaneous remission, or relapsing-remission [4,5] Neurology, Xinqiao Hospital, Third Military Medical University, 183 Xinqiao Main Street, Shapingba District, Chongqing, 400037, China, Figure 1: The axial magnetic resonance images show multiple lesions with alternating hypointense and isointense rings on the T1-weighted imag...

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supporting

confidence: 64%

Baló's Concentric Sclerosis

Wei¹,

Chen²

2015

J Neu neurol

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“…A doença apresenta evolução rapidamente progressiva, mas nem sempre fatal principalmente quando precocemente diagnosticada 1 Depois de feito o diagnóstico da doença desmielinizante e descartadas as hipóteses de lesão expansiva intracerebral, acidente vascular encefálico e esclerose múltipla, foi instituído tratamento pulsoterápico com altas doses de corticóides por 14 dias. Salvo algumas intercorrências decorrentes da imunossupressão, a paciente obteve melhora clínica rápida e Figura 2.…”

Section: Discussionunclassified

“…A esclerose concêntrica de Baló (ECB), também conhecida por esclerose difusa mielinoclástica, doença de Schilder, esclerose transicional ou desmielinização pseudotumoral, é uma doença desmielinizante de caráter auto-imune sem causa conhecida do sistema nervoso central, sem etiologia conhecida, mas provavelmente se trata de uma variante rara da esclerose múltipla e foi descrita inicialmente por Jószef Baló em 1927 1,2 . Na esclerose múltipla (EM), entretanto, as placas de desmielinização estão bem delimitadas, afetando o SNC com preferência por certas áreas como a substância branca periventricular, o sistema óptico, cerebelo, tronco encefálico e medula espinhal 3 .…”

Section: Introductionunclassified

Esclerose concêntrica de Baló – relato de caso

Volpato

Klaus

Carvalho

et al. 2007

Rev. Med. (São Paulo)

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RESUMO: Objetivos:O caso descrito a seguir busca incentivar a suspeição do médico frente a um provável diagnóstico de Esclerose Concêntrica de Baló (ECB). Métodos: Relato de caso associado à revisão da literatura científi ca a partir artigos publicados sobre o tema. Resultados: Há grande difi culdade na determinação da etiopatologia da ECB, já que há amplas áreas de desmielinização do sistema nervoso central (SNC) que podem ser observadas em várias doenças neurológicas. A RNM faz grande suspeição diagnóstica, mas a confi rmação só é realmente obtida através de biópsia cerebral. O tratamento consiste no uso de esteróides em doses altas com neuroimagem seriada de controle. Discussão: Uma vez determinada a natureza infl amatória da lesão, a difi culdade de determinar sua etiopatologia reside no fato de que amplas áreas de desmielinização do sistema nervoso central (SNC) podem ser observadas em várias doenças neurológi-cas. Clinicamente os pacientes com esclerose concêntrica de Balo se apresentam com sintomas agudos ou subagudos, sugerindo lesão expansiva encefálica. A doença tem um curso imprevisível, que varia de uma rápida evolução em semanas ou meses sem remissão até uma condição autolimitada que incluem vários sintomas cognitivos como cefaléia, afasia, distúrbios de comportamento, disfunção visual e ataxia. DESCRITORES:Esclerose múltipla/etiologia. Esclerose cerebral difusa de Schilder/ diagnóstico. Relatos de casos [tipo de publicação].

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“…1,2 Baló suggested that 'encephalitis periaxialis concentrica' might represent a disease entity distinct from multiple sclerosis, partly because of the absence of spinal cord lesions. 3 However, more recently Moore et al have reported a case demonstrating lesions of the Baló type in the lumbar spinal cord as well as in the medulla oblongata 6 and Yao et al have described the histopathological findings of two patients with concurrent typical multiple sclerosis plaques and concentric sclerosis lesions and of another patient with these two lesion types in continuity. 2 The prior episode of left optic neuritis in our patient is consistent with the concept that Baló's concentric sclerosis is a variant of multiple sclerosis.…”

Section: Discussionmentioning

confidence: 99%

“…It is characterized by lesions with an unusual concentric pattern of alternating demyelinated and partially myelinated bands in the cerebral white matter. 1,2 In 1928, Baló reported this pattern in lesions found at autopsy in a 23-year-old male with right hemiparesis, aphasia and papilloedema 3 but the first reported case was that described by Marburg in 1906 as 'acute multiple sclerosis'. 4 Concentric sclerosis usually affects young adults and has an acute or subacute onset and progressive course.…”

Section: Introductionmentioning

confidence: 98%

Baló's concentric sclerosis in a woman from Papua New Guinea

Hulcombe

Bradfield

Tannenberg

et al. 1999

Journal of Clinical Neuroscience

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We report a case of Baló's concentric sclerosis (a variant of multiple sclerosis) from Papua New Guinea. A 42-year-old woman with a past episode of optic neuritis presented with a left hemiparesis. Magnetic resonance imaging revealed a solitary large tumour-like right cerebral lesion with a pattern of concentric bands of different signal intensities. The diagnosis was established by biopsy of the lesion. To our knowledge, this is the first reported case of Baló's concentric sclerosis in the indigenous population of Papua New Guinea.

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Encephalitis Periaxialis Concentrica

Cited by 160 publications

References 7 publications

Baló's Concentric Sclerosis

Baló's Concentric Sclerosis

Esclerose concêntrica de Baló – relato de caso

Baló's concentric sclerosis in a woman from Papua New Guinea

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