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Neuropathology of lubag (x-linked dystonia parkinsonism)

✍ Scribed by Dr. C. H. Waters; P. L. Faust; J. Powers; H. Vinters; C. Moskowitz; T. Nygaard; A. L. Hunt; S. Fahn

Publisher
John Wiley and Sons
Year
1993
Tongue
English
Weight
576 KB
Volume
8
Category
Article
ISSN
0885-3185

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✦ Synopsis

Abstract

Lubag is an x‐linked recessive dystonia parkinsonism that affects Filipino men originating principally from the Panay Island. Linkage analysis has confirmed the mode of inheritance and localized the disease gene to the proximal long arm of the x‐chromosome. We studied the brain of a 34 year old Filipino man affected with lubag. He developed truncal dystonia at age 30, which subsequently generalized. With disease progression, he also presented with parkinsonism including, rigidity, bradykinesia, and impaired balance. His symptoms were largely unaffected by medication and, at age 34, he underwent a right cryothalamotomy. He died suddenly 2 days after the procedure. The principal neuropathological findings were neuronal loss and a multifocal mosaic pattern of astrocytosis restricted to the caudate and lateral putamen. Similar findings have been reported in two other men with dystonia–one Filipino and the other non‐Filipino. The similar pathology of the two Filipino men suggests that this is the pathology of lubag. Recognition of this pathology in a non—Filipino man suggests that the mutation causing lubag may not be restricted to the Filipino population.

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## Abstract “Lubag” or X‐linked dystonia‐parkinsonism (XDP) is a genetic syndrome afflicting Filipino men. Intracranial surgical procedures for Lubag have been unsuccessful. We report a 45‐year‐old Filipino male with genetically confirmed XDP who underwent bilateral pallidal deep brain stimulation