ArticleAnnals of clinical and translational neurology2026
Posterior Disconnection Syndrome in Early-Stage Adult-Onset Cerebral Adrenoleukodystrophy.
Article in Annals of clinical and translational neurology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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14 authors.
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Abstract
Adult-onset cerebral adrenoleukodystrophy is potentially treatable but often difficult to recognize before advanced cerebral involvement. Herein, we describe three men with early-stage disease who initially presented with subtle visual complaints rather than subcortical dementia. Targeted neuropsychological testing revealed higher-order visual dysfunction and posterior interhemispheric disconnection signs, including visual-field-dependent reading or naming deficits and dichotic listening abnormalities. Diffusion tensor imaging showed preferential involvement of posterior callosal and visual stream-related white-matter pathways. These findings define a posterior disconnection phenotype of adult-onset cerebral adrenoleukodystrophy and provide a clinically useful framework for early recognition and timely therapeutic consideration of this treatable leukodystrophy.
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