Clinical and ultrastructural ocular histopathologic studies of adult-onset metachromatic leukodystrophy

Harry A Quigley, W. Richard Green

Research output: Contribution to journalArticle

Abstract

A twin brother and sister with adultonset metachromatic leukodystrophy developed progressive central acuity loss and optic disk pallor. Both had normal electroretinograms and fluorescein angiography. Postmortem examination of the sister's eyes by histochemistry and electron microscopy revealed ganglion cell loss and optic atrophy. Cerebroside sulfate had accumulated in optic nerve glial cells. Optic atrophy was more advanced than in previously reported cases of infantile-onset metachromatic leukodystrophy. The pathologic process seemed to be retrograde optic nerve degeneration due to abnormal myelin metabolism.

Original languageEnglish (US)
Pages (from-to)472-479
Number of pages8
JournalAmerican Journal of Ophthalmology
Volume82
Issue number3
StatePublished - 1976

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Metachromatic Leukodystrophy
Optic Atrophy
Optic Nerve
Pallor
Nerve Degeneration
Fluorescein Angiography
Optic Disk
Pathologic Processes
Myelin Sheath
Neuroglia
Ganglia
Autopsy
Electron Microscopy
Neurons
cerebroside sulfate

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Clinical and ultrastructural ocular histopathologic studies of adult-onset metachromatic leukodystrophy. / Quigley, Harry A; Green, W. Richard.

In: American Journal of Ophthalmology, Vol. 82, No. 3, 1976, p. 472-479.

Research output: Contribution to journalArticle

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