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Computed tomography in white matter diseases.

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Computed Tomography
in White Matter Diseases
Donna Ferriero, MD, and Thomas K. Koch, M D
Farrell and colleagues 12) recently reported contrastenhancing lesions on computed tomography (CT) in the
periventricular frontal regions, caudate nuclei, and thalami in
an infant with pathologically proved Alexander’s disease.
The authors suggest that this pattern of enhancement may be
specific for early stages of this disease.
Holland and Kendall [3] previously reported low attenuation of the deep frontal white matter on non-contrastenhanced scans in two patients with Alexander’s disease.
One patient who was scanned early in the course of the
disease had dramatic contrast enhancement of the white
matter.
We describe a patient with biochemically proved globoid-cell leukodystrophy (Krabbe’s disease) whose C T scan
closely resembles that described by Farrell and associates 12J.
The C T brain scan of our patient at 6 months of age disclosed symmetrical high density of the periventricular white
matter and thalami that enhanced after contrast infusion
(Figure).
The existence and distribution of areas of contrast enhancement with CT scanning in various leukodystrophies
appear to correspond to the temporal evolution of white
matter destruction and are not specific for a given disease.
Serial CT scanning of white matter diseases usually shows
characteristic changes heralded by low density of the white
A
314 Annals of Neurology Vol 17 N o 3 March 1985
matter which later returns to normal and is then accompanied by atrophy and ventricular enlargement [ 11. Enhancement patterns similar to those described here have been seen
in some of the leukodystrophies and are thought to be due to
the disruption of the blood-brain barrier, indicating an active
lesion [6}.
It therefore seems likely that the evolution of white matter
changes reflected on C T can be extended to include a very
early stage of contrast enhancement, followed by low-density
changes and finally atrophy. Since this progression has now
been observed in Alexander’s disease C2, 31, Krabbe’s disease, adrenoleukodystrophy [4],
and other leukodystrophies
[6,71, the finding of white matter enhancement cannot be
considered specific for a given disease. Until specific biochemical assays are available for all of the white matter diseases, brain biopsy is still needed to confirm the antemortem
diagnosis in those diseases of uncertain etiology.
Departments of Neurology and Pediatrics
School of Medicine
Univwsity of California, San Francisco
San Francisco, CA 9414.3
Non-contrast-enhanced axial computed tomographic scan taken
with a GE 8800 scanner shows increased density of the thalami
(A).There is mild contrast enhancement (B). The ventrides are
normal in size. (Theperiventricular white matter changes are
not seen in these particular sections.)
B
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