PMID: 9164693May 1, 1997Paper

A rare form of adult onset leukodystrophy: orthochromatic leukodystrophy with pigmented glia

The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques
P ShannonS Nag

Abstract

Orthochromatic leukodystrophy with pigmented glia and scavenger cells is a rare leukodystrophy of unknown etiology. This report describes a 42-year-old man with a history of depression, dementia and parkinsonism having the pathological features of orthochromatic leukodystrophy with pigmented glia. We reviewed the clinical history and pathology of autopsy and brain biopsy material. Imaging revealed bilateral cerebral white matter hypodensities. At autopsy, the brain demonstrated a leukodystrophy affecting predominantly the cerebral hemispheres and characterized by demyelination, and cytoplasmic pigment deposits in oligodendroglia and astrocytes. The pigment had the staining properties of ceroid-lipofuschin and on ultrastructural examination was composed of membrane-bound lipid and electron-dense inclusions which had a fingerprint-like pattern. Similar pigment inclusions were not observed on ultrastructural examination of renal, splenic or hepatic tissue obtained at autopsy. The brain biopsy contained cerebral cortex with sparse subcortical white matter in which a few oligodendroglia and fewer astrocytes at the grey/white junctions showed cytoplasmic pigmentary inclusions identical to those described above. However, due to the pa...Continue Reading

References

Dec 1, 1992·Italian Journal of Neurological Sciences·L CalandrielloG Palladini
Jan 1, 1991·Acta Neuropathologica·J Constantinidis, T M Wisniewski
Sep 1, 1987·Journal of Neuropathology and Experimental Neurology·F GrayJ Poirier

❮ Previous
Next ❯

Citations

Jul 11, 2007·Journal of Neuropathology and Experimental Neurology·Zarina S AliJames M Powers
Oct 12, 2011·Dementia and Geriatric Cognitive Disorders·Janice C WongLili-Naz Hazrati
Oct 17, 2013·Journal of Neuropathology and Experimental Neurology·Murad AlturkustaniLee-Cyn Ang
Nov 28, 2002·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·J VergheseI Rapin

❮ Previous
Next ❯

Related Concepts

Related Feeds

Astrocytes

Astrocytes are glial cells that support the blood-brain barrier, facilitate neurotransmission, provide nutrients to neurons, and help repair damaged nervous tissues. Here is the latest research.