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February 1993

Psychopathology in Metachromatic Leukodystrophy

Author Affiliations

Departments of Psychiatry and Neurology Albert Einstein College of Medicine Jacobi Room 1020 Bronx, NY 10461

Arch Neurol. 1993;50(2):131. doi:10.1001/archneur.1993.00540020011005

To the Editor.  —Hyde and colleagues1 speculate that psychosis in metachromatic leukodystrophy "can result from and perhaps depends on dysfunctional connectivity between certain extrafrontal and subcortical structures with the frontal lobe." They appear to be unaware of several previously published articles on psychopathology in metachromatic leukodystrophy,2-4 including an article entitled "A Proposed Etiology for Psychotic Symptoms in White Matter Dementia"2 in which we earlier put forward a similar hypothesis. Therein we reported a case of metachromatic leukodystrophy with psychosis and proposed that psychotic manifestations were "a consequence of profound deterioration of white matter tracts, including those traversing the frontal lobes" resulting in "the pathological disinhibition of limbic catecholaminergic systems."Metachromatic leukodystrophy with psychosis may be an extremely fruitful disorder for understanding the relationship between altered brain function and psychosis.

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