Natural History and Evolution of the Concept of Schilder's Diffuse Sclerosis

Poser, Charles M.; Bogaert, Ludo van

doi:10.1111/j.1600-0447.1956.tb09694.x

Cited by 93 publications

(15 citation statements)

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“…Eventually, however, many other demyelinating and even dysmyelinating diseases became loosely defined as varieties of Schilder's diffuse sclerosis [2], Schilder [5,6] himself fur ther confused the issue by later describing 2 additional cases in 1913 and 1924 which in fact were separate disor ders, ALD and subacute sclerosing panencephalitis. The confusion surrounding the many disorders was adressed by Poser and Van Bogeart [7] when they reviewed the his torical case reports and correctly diagnosed the specific disorders. Poser [8] further subcategorized the demye linating diseases into defects associated with myelinogene sis, ALD or myelinoclastic states; Balo's concentric sclero sis, Devic's neuromyelitis, MS, and transitional sclerosis.…”

Section: Discussionmentioning

confidence: 99%

Myelinoclastic Diffuse Sclerosis Presenting as a Mass Lesion in a Child with Turner’s Syndrome

et al. 1995

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A rare case of myelinoclastic diffuse sclerosis (MDS), occasionally referred to as Schilder’s disease, is reported in a child with Turner’s syndrome. The child originally presented with a 3-week history of nausea, vomiting and frontal headaches. Magnetic resonance imaging showed a large, contrast-enhancing, right frontal lobe mass which was ultimately resected uneventfully. Complete laboratory investigations and pathological evaluation of the resected specimen verified the case to be MDS. The clinical presentation, laboratory evaluation, imaging characteristics and diagnosis are discussed in this review of the disease. The importance of including demyelinating diseases in the differential diagnosis for newly discovered mass lesions in the pediatric population is underscored by this case.

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Section: Discussionmentioning

confidence: 99%

Myelinoclastic Diffuse Sclerosis Presenting as a Mass Lesion in a Child with Turner’s Syndrome

et al. 1995

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“…They outlined the way the term Schilder's disease should be redesignated and fully supported the opinion put for ward by Bielschowsky and Henneberg [2]. After a series of contributions by Heubner [3] and Marburg [4], Schilder [5][6][7] described in three papers what now ap pears to represent three different conditions: (1) an acute form of multiple sclerosis in a 14-year-old girl (1912); (2) a familial leukodystrophy (1913), and (3) a subacute sclerosing leukoencephalitis (1924). After these descriptions, an era of considerable confu sion followed since the name of Schilder's disease cov ered in fact a host of unrelated and heterogeneous dis eases.…”

Section: Introductionmentioning

confidence: 99%

“…The natural history and the evolution of the concept of Schilder's diffuse sclerosis have been described in a masterly fashion by Poser and van Bogaert [1] in 1956. They outlined the way the term Schilder's disease should be redesignated and fully supported the opinion put for ward by Bielschowsky and Henneberg [2].…”

Section: Introductionmentioning

confidence: 99%

“…We shall give an overview of some of the conditions diffusely affecting the white matter according to the clas sification given by Poser and van Bogaert [1] and by Poser [14] to emphasize the fact that they represent clearly different entities which do no longer need to be regrouped under any unifying concept.…”

Section: Introductionmentioning

confidence: 99%

“…Various classifications followed which reflected the current state of knowledge concerning the mechanisms of demyelination. Some of these classifications [11,12] are outdated while others have better withstood the test of time, including three groups according to the prototypes described by Schilder (myelinoclastic diseases, leukodystrophies and the sub acute encephalitides) [1] or the myelinoclastic diseases and the dysmyelinating diseases [13,14].…”

Section: Introductionmentioning

confidence: 99%

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Schilder's Diffuse Sclerosis

Martin

Guazzi

1991

Dev Neurosci

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The natural history and the evolution of the concept of Schilder''s diffuse sclerosis have been described by Poser and van Bogaert in 1956 and there is really not much to add to their analysis. The major progress made in the clinical, genetic, enzymatic and biochemical workup of many of the conditions previously grouped under the denomination of Schilder''s disease confirms its heterogeneity. We will discuss (1) the myelinoclastic disorders; (2) the leukodystrophies with disorders such as adrenoleukodystrophy and the still very ill-defined sudanophilic leukodystrophies; (3) the subacute sclerosing leukoencephalitis, better known today as the subacute sclerosing panencephalitis. In conclusion, the name of Schilder is to be remembered as are all the names of the great neurologists and neuropathologists of the past but it would be better to avoid nowadays the eponym of Schilder''s disease in order to prevent confusion between different disorders.

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Genetic Aspects of Neurological Disease

Zellweger¹

1965

Arch Intern Med

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Natural History and Evolution of the Concept of Schilder's Diffuse Sclerosis

Cited by 93 publications

References 94 publications

Myelinoclastic Diffuse Sclerosis Presenting as a Mass Lesion in a Child with Turner’s Syndrome

Myelinoclastic Diffuse Sclerosis Presenting as a Mass Lesion in a Child with Turner’s Syndrome

Schilder's Diffuse Sclerosis

Genetic Aspects of Neurological Disease

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