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Krabbe's Leukodystrophy (Globoid Cell Leukodystrophy)An Ultrastructural Study
Kinuko Suzuki, MD;
Warren D. Grover, MD
Arch Neurol. 1970;22(5):385-396.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings. |
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KRABBE'S leukodystrophy or globoid cell leukodystrophy (GLD) is a familial neurological disorder of early infancy characterized clinically by tonic seizures, generalized convulsions, spastic quadriplegia, cortical blindness or optic atrophy, deafness, pseudobulbar palsy, and a rapidly progressive course.
Its pathological picture is unique because the disease process is confined almost entirely to the central nervous system and is characterized by the accumulation of epithelioid and globoid cells, marked paucity of myelin, and severe gliosis of white matter. Biochemical analysis reveals an increase in the ratio of cerebroside to sulphatide, although glycolipids as a whole are reduced in the cerebral white matter.1,2 This excessive accumulation of cerebroside in the white matter, particularly in globoid cells, presumably is due to deficient cerebroside sulphotransferase activity.3
There are several histopathologic and histochemical studies of GLD4-8 but only one ultrastructural body of GLD has been performed by Nelson et al.
. . . [Full Text PDF of this Article]
Author Affiliations
Philadelphia
From the Department of Pathology, Division of Neuropathology, University of Pennsylvania School of Medicine (Dr. Suzuki) and the Section of Pediatric Neurology, St. Christopher's Hospital (Dr. Grover), Philadelphia.
Footnotes
Submitted for publication Aug 15, 1969; accepted Sept 16.
Reprint requests to Department of Pathology (Neuropathology), Hospital of the University of Pennsylvania, Philadelphia 19104 (Dr. Suzuki).
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