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Abstract
We describe the magnetic resonance (MR) imaging findings in two patients with the
clinical diagnosis of progressive neuronal degeneration of childhood with liver disease
(Alpers' syndrome). One patient showed atrophy of both occipital lobes, and one patient
showed high signal intensity in deep gray matter nuclei and diffuse atrophy. Although
the imaging findings were nonspecific, they correlated well with the patients' clinical
findings.
Keywords
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References
- Progressive neuronal degeneration of childhood with liver disease (Alpers-Huttenlocher syndrome): a personal review.J Child Neurol. 1990; 5: 273-287
- Progressive neuronal degeneration of childhood with liver disease. Computed tomographic features.Neuroradiology. 1987; 29: 74-180
- Diffuse progressive degeneration of the gray matter of the cerebrum.Arch Neurol Psychiatry. 1931; 25: 469-505
- Mitochondrial disorders: analysis of their clinical and imaging characteristics.AJNAR. 1993; 14: 1119-1137
- Mitochondrial encephalomyopathies in childhood. II. Clinical manifestations and syndromes.J Pediatr. 1991; 119: 251-256
- Transmission of Alpers disease (chronic progressive encephalopathy) produces experimental Creutzfeldt-Jakob disease in hamsters.Neurology. 1989; 39: 615-621
Article info
Publication history
Accepted:
December 15,
1994
Received:
October 25,
1994
Identification
Copyright
© 1996 Published by Elsevier Inc.
