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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 2003 May;74(5):671–673. doi: 10.1136/jnnp.74.5.671

Two clinicopathological cases of a dominantly inherited, adult onset orthochromatic leucodystrophy

F Letournel 1, F Etcharry-Bouyx 1, C Verny 1, A Barthelaix 1, F Dubas 1
PMCID: PMC1738413  PMID: 12700318

Abstract

The first patient, aged 58 years, had frontal dementia and epilepsy; the second, aged 38 years, had motor signs and dementia, but no epilepsy. The histopathological features of our two cases were leucodystrophy of orthochromatic subtype. However, the radiological features (MRI and mostly FLAIR sequences) of the first case did not suggest leucodystrophy.

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