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Table of Contents
Vol. 57, No. 1, 2007
Issue release date: January 2007
Section title: Historical Note
Eur Neurol 2007;57:59–61
(DOI:10.1159/000097121)

Baló’s Encephalitis Periaxialis Concentrica

Pearce J.M.S.
Emeritus Consultant Neurologist, Department of Neurology, Hull Royal Infirmary, Hull, UK
email Corresponding Author

Abstract

In 1928, Baló described a law student with an unusual fatal illness marked by aphasia and a right hemiplegia, with later optic neuritis and normal cerebrospinal fluid. At autopsy, he found a disease of the white matter characterised by foci varying in size from a lentil to that of a pigeon’s egg and presenting gray softening and, in part, concentricity, where the medullary sheaths were destroyed and the axis cylinders were intact. He was uncertain whether this was a variant of acute multiple sclerosis or of Schilder’s disease. The basis of concentric sclerosis is still unclear though current opinion favours a variant of acute multiple sclerosis.

© 2007 S. Karger AG, Basel


  

Key Words

  • Baló
  • Concentric sclerosis
  • Demyelination
  • Multiple sclerosis
  • Heat shock protein

Introduction


Schilder [1], in 1913, reported 3 cases in children that he referred to as ‘encephalitis periaxialis diffusa’, characterised by diffuse involvement of the cerebral hemispheres with severe myelin loss, which resembled multiple sclerosis because of the relative preservation of axons and the accumulation of lymphocytes; he also showed numerous fat-laden phagocytes and gliosis. Haberfield and Spieler [2] reported Schilder’s adrenoleucodystrophy in 1910 as did Siemerling and Creutzfeldt [3] in 1923. Patients reported with familial Schilder’s disease are probably instances of sudanophilic cerebral sclerosis, Krabbe disease or metachromatic leucoencephalopathy. Though phenotypic variations are common, the variant reported by Jozsef Baló [4] is remarkable and there is still uncertainty about whether his case represented a type of Marburg’s acute multiple sclerosis or a variant of Schilder’s disease [5]. Marburg, in a prescient paper described an acute illness, which terminated life within 1 year. He noted the close similarities to multiple sclerosis, with a perivascular distribution of lesions. He showed both demyelination and remyelination of the axons and, in line with present ideas, noted that the axons, too, were affected and atrophic in peripheral nerves as well as in the brain and cord. One of his 3 cases showed striking concentric sclerosis.

 

Baló’s Concentric Sclerosis


Jószef Baló (1895–1979) was a neuropathologist, born in Budapest, Hungary. Unfortunately, most of his biographical details [6] and publications [7] have not been translated into English or other European languages.

His first account was published in the Hungarian journal Ról Magy Orv Arch and was translated into English (‘Encephalitis periaxialis concentrica’) in 1928. Baló [4] wrote:

‘A law student aged 23 became ill with aphasia; then followed weakness of the inferior branch of the facial nerve and disappearance of the cremasteric reflex on both sides and of the abdominal reflexes on the right side. Later, a hemiplegia of the right side, incontinence of urine and feces and a total aphasia developed. Before death, tonic spasms occurred in the right arm and leg; trismus developed, and the patient lost consciousness. Simultaneously, the right knee reflex and the Achilles reflex became exaggerated, and the Babinski phenomena appeared on the right side. At the beginning, the fundi of the eyes were normal; later, optic neuritis developed (p. 254) ... one month before admission it became more difficult for him to write ... In certain words, he would stop and think how he should write the letter g for instance ...’

Examination showed the patient was afebrile, with normal blood pressure and normal general examination.

‘... Right inferior facial nerve weaker than left ... speech was difficult and dysarthric. The patient could not write certain words or letters though he knew them. ... No pathological reflexes, ataxia and Romberg sign and hypotonia were absent ... Neither adiadokokinesis nor astereognosis were present, sensation was normal. CSF was normal.’ (p. 244)

A pathological description follows (fig. 1):

FIG01
Fig. 1. Baló’s [4] summary of the pathology of concentric sclerosis.

Baló summarised the findings:

‘... an isolated disease of the white matter which is characterised by the occurrence of foci varying in size from a lentil to that of a pigeon’s egg and presenting gray softening and, in part, concentricity. In these foci, the medullary sheaths are destroyed, and the axis cylinders remain intact. Proliferation of the neuroglia follows the destruction of the medullary sheaths, and mast glia cells occur which partly undergo degeneration and become the subject of gliophagia. The concentric foci develop in such a manner that the degenerated layers alternate with normal layers of white matter (fig. 2). The changes about the vessels indicate the inflammatory character of the process (p. 263). Syphilis, apparently does not play a part in the etiology ... Intracerebral inoculation with material from the brain into rabbits did not transmit the disease ...’

FIG02
Fig. 2. Baló’s [4] concentric focus: gross pathology.

Baló provides an extensive discussion which repays study. He appraises the differential diagnosis and the possible relation of his findings to those in the earlier literature (fig. 3). He concludes:

FIG03
Fig. 3. Baló’s [4] classification.

‘Among the diseases of the brain known to date, the condition in the case reported resembles acute multiple sclerosis and the encephalitis periaxialis diffusa of Schilder. The disease differs from encephalitis periaxialis diffusa because of its focal character. It differs from multiple sclerosis because in that condition both brain and spinal cord and both the gray and the white matter are affected.’ (pp. 263–264)

Clinically, Baló’s concentric sclerosis commonly presents as an acute or subacute, sometimes monophasic, encephalopathy with a fulminating course. Diagnosis is ultimately proved by histopathology, but in life, MRI shows characteristic irregular, concentric zones of increased signal on T2-weighted images. More recent publications show examples of a less malignant course and some response to corticosteroids.

The basis of concentric sclerosis is still unclear though current opinion favours a variant of acute multiple sclerosis [8]. A recent example reported was associated with primary human herpesvirus 6 infection [5]. The active concentric lesions follow a patternof demyelination that resembles the tissue injury of hypoxia. Lesions show high expression of induciblenitric oxide synthase in macrophages and microglia, with oligodendrocytic dystrophy. At the edgeof active lesions, proteins involved in tissue preconditioning,such as hypoxia-inducible factor 1 and heat shock protein 70,are expressed mainly in oligodendrocytes. Thus, this rim may be resistant to further damage in an expandinglesion and may therefore remain as a layer of preserved myelinatedtissue [9].


References

  1. Schilder P: Zur Frage der Encephalitis periaxialis diffusa (sogenannte diffuse Sklerose). Z Gesamte Neurol Psychiatr 1913;15:359–376.

    External Resources

  2. Haberfeld W, Spieler F: Zur diffusen Hirn-Rückenmarksklerose im Kindesalter. Dtsch Z Nervenheilkd 1910;40:436–463. Cited by Moser HW, Moser AB, Naidu S, Bergin A: Clinical aspects of adrenoleukodystrophy and adrenomyeloneuropathy. Dev Neurosci 1991;13:254–261.

    External Resources

  3. Siemerling E, Creutzfeldt HG: Bronzekrankheit und sklerosierende Encephalomyelitis. Arch Psychiatr Nervenkr 1923;68:217–244.

    External Resources

  4. Baló J: Leukoencephalitis periaxialis concentrica. Ról Magy Orv Arch 1927;28:108–124 (translated: Encephalitis periaxialis concentrica. Arch Neurol Psychiatry 1928;19:242–264).

    External Resources

  5. Pohl D, Rostasy K, Krone B, Hanefeld F: Baló’s concentric sclerosis associated with primary human herpesvirus 6 infection. J Neurol Neurosurg Psychiatry 2005;76:1723–1725.
  6. Lapis K: Dr Jozsef Baló (1895–1979). Orv Hetil 1979;120:2902–2904.
  7. List of the publications of Dr Josef Baló. Acta Morphol Acad Sci Hung 1968;16:7–18.

    External Resources

  8. Capello E, Mancardi GL: Marburg type and Baló’s concentric sclerosis: rare and acute variants of multiple sclerosis. Neurol Sci 2004;25(suppl 4):S361–S363.

    External Resources

  9. Stadelmann C, Ludwin S, Tabira T, Guseo A, Lucchinetti CF, Leel-Ossy L, Ordinario AT, Bruck W, Lassmann H: Tissue preconditioning may explain concentric lesions in Baló’s type of multiple sclerosis. Brain 2005;128:979–987.

  

Author Contacts

J.M.S. Pearce
304 Beverley Road
Anlaby, East Yorks HU10 7BG (UK)

  

Article Information

Received: July 21, 2006
Accepted: July 23, 2006
Published online: November 20, 2006
Number of Print Pages : 3
Number of Figures : 3, Number of Tables : 0, Number of References : 9

  

Publication Details

European Neurology

Vol. 57, No. 1, Year 2007 (Cover Date: January 2007)

Journal Editor: Bogousslavsky, J. (Lausanne)
ISSN: 0014–3022 (print), 1421–9913 (Online)

For additional information: http://www.karger.com/ENE


Copyright / Drug Dosage / Disclaimer

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

Abstract

In 1928, Baló described a law student with an unusual fatal illness marked by aphasia and a right hemiplegia, with later optic neuritis and normal cerebrospinal fluid. At autopsy, he found a disease of the white matter characterised by foci varying in size from a lentil to that of a pigeon’s egg and presenting gray softening and, in part, concentricity, where the medullary sheaths were destroyed and the axis cylinders were intact. He was uncertain whether this was a variant of acute multiple sclerosis or of Schilder’s disease. The basis of concentric sclerosis is still unclear though current opinion favours a variant of acute multiple sclerosis.

© 2007 S. Karger AG, Basel


  

Author Contacts

J.M.S. Pearce
304 Beverley Road
Anlaby, East Yorks HU10 7BG (UK)

  

Article Information

Received: July 21, 2006
Accepted: July 23, 2006
Published online: November 20, 2006
Number of Print Pages : 3
Number of Figures : 3, Number of Tables : 0, Number of References : 9

  

Publication Details

European Neurology

Vol. 57, No. 1, Year 2007 (Cover Date: January 2007)

Journal Editor: Bogousslavsky, J. (Lausanne)
ISSN: 0014–3022 (print), 1421–9913 (Online)

For additional information: http://www.karger.com/ENE


Article / Publication Details

First-Page Preview
Abstract of Historical Note

Received: 7/21/2006
Accepted: 7/23/2006
Published online: 11/20/2006
Issue release date: January 2007

Number of Print Pages: 3
Number of Figures: 3
Number of Tables: 0

ISSN: 0014-3022 (Print)
eISSN: 1421-9913 (Online)

For additional information: http://www.karger.com/ENE


Copyright / Drug Dosage

Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

References

  1. Schilder P: Zur Frage der Encephalitis periaxialis diffusa (sogenannte diffuse Sklerose). Z Gesamte Neurol Psychiatr 1913;15:359–376.

    External Resources

  2. Haberfeld W, Spieler F: Zur diffusen Hirn-Rückenmarksklerose im Kindesalter. Dtsch Z Nervenheilkd 1910;40:436–463. Cited by Moser HW, Moser AB, Naidu S, Bergin A: Clinical aspects of adrenoleukodystrophy and adrenomyeloneuropathy. Dev Neurosci 1991;13:254–261.

    External Resources

  3. Siemerling E, Creutzfeldt HG: Bronzekrankheit und sklerosierende Encephalomyelitis. Arch Psychiatr Nervenkr 1923;68:217–244.

    External Resources

  4. Baló J: Leukoencephalitis periaxialis concentrica. Ról Magy Orv Arch 1927;28:108–124 (translated: Encephalitis periaxialis concentrica. Arch Neurol Psychiatry 1928;19:242–264).

    External Resources

  5. Pohl D, Rostasy K, Krone B, Hanefeld F: Baló’s concentric sclerosis associated with primary human herpesvirus 6 infection. J Neurol Neurosurg Psychiatry 2005;76:1723–1725.
  6. Lapis K: Dr Jozsef Baló (1895–1979). Orv Hetil 1979;120:2902–2904.
  7. List of the publications of Dr Josef Baló. Acta Morphol Acad Sci Hung 1968;16:7–18.

    External Resources

  8. Capello E, Mancardi GL: Marburg type and Baló’s concentric sclerosis: rare and acute variants of multiple sclerosis. Neurol Sci 2004;25(suppl 4):S361–S363.

    External Resources

  9. Stadelmann C, Ludwin S, Tabira T, Guseo A, Lucchinetti CF, Leel-Ossy L, Ordinario AT, Bruck W, Lassmann H: Tissue preconditioning may explain concentric lesions in Baló’s type of multiple sclerosis. Brain 2005;128:979–987.