Development of Myelin in Inherited Disorders of Amino Acid Metabolism

Prensky, Arthur L.; Carr, Sheila; Moser, Hugo W.

doi:10.1001/archneur.1968.00480060022002

Cited by 61 publications

(17 citation statements)

References 17 publications

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“…Similar findings have been noted not only in cases of B-BSD, but also in other conditions such as phenylketonuria (Crome, Tymms, and Woolf, 1962;Menkes, 1966) and maple syrup urine disease (Prensky and Moser, 1966;and Prensky, Carr, and Moser, 1968). Based on chemical analysis, Kamoshita, Rapin, Suzaki, and Suzaki (1968) considered B-BSD to be primarily a disease of chronic oedema, with the myelin loss being secondary to water accumulation.…”

Section: Discussionsupporting

confidence: 61%

Infantile spongy degeneration of the central nervous system associated with glycogen storage and markedly fatty liver

Takei

Solitare

1972

Journal of Neurology, Neurosurgery & Psychiatry

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SUMMARY The clinical, biochemical, and pathological features of an unusual expression of infantile spongy degeneration of the central white matter are presented with emphasis on neuropathological observations. The topographical distribution of the spongy change along with the observed defect in myelination were such as to suggest an arrest in development of the white matter in late foetal life. Of additional interest, in the present case, is the observed deposition of glycogen in the brain, heart, and liver along with a markedly fatty liver. Our findings are compared with those in other cases of so-called spongy degeneration, as well as with certain of the aminoacidurias and, as a consequence, we wish to suggest that the basic pathogenetic factor probably lies in a disturbance of the biochemical energy supply system rather than in a disorder of myelin lipid metabolism per se.

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Section: Discussionsupporting

confidence: 61%

Infantile spongy degeneration of the central nervous system associated with glycogen storage and markedly fatty liver

Takei

Solitare

1972

Journal of Neurology, Neurosurgery & Psychiatry

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“…Several conditions beside ionizing radiations are associated with decreased myelin formation in the central nervous system. These include undernutrition (Chase et al, 1967;Dobbing, 1968), phenylketonuria (Crome et al, 1968), Border disease in the lamb (Davison et al, ----1966)) maple syrup urine disease (Prensky, 1968) and hypothyroidism (Balazs et al, 1969). Healthy animals were used to make the nerve tissue cultures used in the present work, and as discussed earlier, the culture conditions adopted resulted in healthy myelinated cultures from the majority of cultured explants.…”

Section: Discussionmentioning

confidence: 98%

Effects of ionizing radiations on myelin formation in rat brain cultures

Mamoon

1969

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“…These are well within the ranges of concentrations observed in the inborn errors of metabolism associated with these compounds. These inborn errors of metabolism do show "demyelination" (Prensky et al 1968, Schumann et al 1978, Harding et al 1984.…”

Section: Effect Of Phenylalanine On Brain Maturation: Implications Fomentioning

confidence: 99%