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Late onset Krabbe's leukodystrophy: a report of four cases
M Phelps1, J Aicardi, M T Vanier
1Département de Pediatrie, Hôpital Necker Enfants Malades, Paris, France.
Journal of Neurology, Neurosurgery, and Psychiatry
|April 1, 1991
Abstract:
Four cases of late-onset Krabbe's leukodystrophy had varying clinical features. One sibling became wheelchair-bound while another leads an active working life. The computed tomography (CT) scan of a third patient showed occipital demyelination with contrast enhancement identical to that seen in an adrenoleucodystrophy. The fourth was the only patient with peripheral neuropathy. All patients had enzyme activity comparable to the classic infantile-onset form.