Adreno leukodystrophy (sex linked Schilder disease). Ultrastructural demonstration of specific cytoplasmic inclusions in the central nervous system

H. H. Schaumburg, J. M. Powers, K. Szuki, C. S. Raine

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Abstract

An ultrastructural study of the central nervous system of 4 male patients with adreno leukodystrophy was performed. Membrane like cytoplasmic inclusions similar to those described in adrenal cortex, testis, and peripheral nerve were present in macrophages. These changes in widely scattered organs are probably the structural representation of a generalized metabolic disorder and are consistent with the authors' previous hypothesis of a membrane stability defect in these organs. It is mentioned that this study has demonstrated for the first time membrane like cytoplasmic inclusions in the CNS of patients with A LD similar to those previously reported in adrenal cortical cells, testis, and Schwann cells. These profiles were present in the CNS of every case of A LD examined ultrastructurally, and they established a morphologic link between the inflammatory demyelinating process in the CNS, previously considered a primary immunologic disorder similar to multiple sclerosis, and the noninflammatory changes in other organs. The presence of intranuclear inclusions composed of 160 Å filaments in a metabolic disease (A LD), identical to those described in postmortem material from several multiple sclerosis patients, renders their role as possible etiologic agents in this demyelinating disease sub judice.

Original languageEnglish (US)
Pages (from-to)210-213
Number of pages4
JournalArchives of Neurology
Volume31
Issue number3
StatePublished - 1974

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Diffuse Cerebral Sclerosis of Schilder
Inclusion Bodies
Metabolic Diseases
Central Nervous System
Multiple Sclerosis
Membranes
Testis
Intranuclear Inclusion Bodies
Adrenal Cortex
Schwann Cells
Demyelinating Diseases
Peripheral Nerves
Macrophages
Inclusion
Organs
Membrane

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Adreno leukodystrophy (sex linked Schilder disease). Ultrastructural demonstration of specific cytoplasmic inclusions in the central nervous system. / Schaumburg, H. H.; Powers, J. M.; Szuki, K.; Raine, C. S.

In: Archives of Neurology, Vol. 31, No. 3, 1974, p. 210-213.

Research output: Contribution to journalArticle

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