Progressive Degenerative Subcortical Encephalopathy

Globus, Joseph H.

doi:10.1001/archneurpsyc.1928.02210180041003

Cited by 82 publications

(16 citation statements)

References 9 publications

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“…The examination of lesions of various sizes and at different times after exposure, by means of special staining techniques, showed clearly that the initial focal demyelinization was regularly followed, in the absence of notable inflammation or changes in the cerebral blood vessels, by a degeneration of the axis cylinders and later by a proliferation of the astrocytes and microglia, and by minor changes in the number and appearance of the oligodendroglia. In these respects the lesion resembles closely those that typify disseminated encephalomyelitis in human beings (14).…”

Section: Discussionsupporting

confidence: 53%

Demyelinization Induced in the Brains of Monkeys by Means of Fast Neutrons

Vogel

Pickering

1956

The Journal of Experimental Medicine

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Demyelinization was regularly conspicuous in the white matter of the rostral portions of the brains of 6 monkeys sacrificed 14 to 22 months after exposure of the ocular regions to 850 r.e.p. of 14 mev. neutron radiation and it was not present in the brain of a monkey 2 months after radiation under identical conditions; or in those of 5 non-radiated animals serving as controls. In early lesions, the individual myelin sheaths were varicose and fragmented, while the neurons, axons, and glial cells remained normal in appearance. With the passage of time, the degeneration of myelin became more marked and in later stages was accompanied by a degeneration of the axis cylinders, a proliferation of astrocytes and microglia, and minor cytological changes in the oligodendroglia, the whole process occurring essentially without inflammation or notable changes in the cerebral or meningeal blood vessels. The findings show that neutron radiation has the property of destroying myelin in the living animal and inducing changes that are notably similar in their pathogenesis to those that characterize disseminated encephalomyelitis in human beings.

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Section: Discussionsupporting

confidence: 53%

Demyelinization Induced in the Brains of Monkeys by Means of Fast Neutrons

Vogel

Pickering

1956

The Journal of Experimental Medicine

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“…Pathological features of the brain biopsy of this in fant are characteristic of a condition called 'spongy de generation of the white matter' [10,11]. An infant with this disorder was originally described by Globus and Strauss [10] in 1928 and Canavan [4] in 1931, but all three made a diagnosis of Schilder's disease.…”

Section: Discussion Of Pathological Features (Lucy B Rorke)mentioning

confidence: 99%

“…An infant with this disorder was originally described by Globus and Strauss [10] in 1928 and Canavan [4] in 1931, but all three made a diagnosis of Schilder's disease. It was not until Van Bogaert and Bertrand [5,11] examined several familial cases and investigated the unusual clinical and morphological features of case reports in the literature that it was clearly defined.…”

Section: Discussion Of Pathological Features (Lucy B Rorke)mentioning

confidence: 99%

Psychomotor Retardation and Macrocephaly in an Infant

et al. 1985

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“…Globus and Strauss (1928) were the first to recognise the spongy deteriorative effects of the disease, although the first clinical description was provided by Myrtelle Canavan (Canavan, 1931). Almost two decades later, Van Bogaert and Bertrand (1949) reported its autosomal recessive nature and increased prevalence amongst Ashkenazi Jewish population; notably, CD is also known as the "Canavan-Van Bogaert-Bertrand" disease.…”

Section: Introductionmentioning

confidence: 99%