Alterations of GABAergic neurons in the basal ganglia of patients with progressive supranuclear palsy

Author:

Levy R.,Ruberg M.,Herrero M. T.,Villares J.,Javoy-Agid F.,Agid Y.,Hirsch E. C.

Abstract

Article abstract-We analyzed postmortem GABAergic neurons in the basal ganglia of three patients with progressive supranuclear palsy (PSP) and four matched controls by means of glutamic acid decarboxylase (Mr 67,000 (GAD67)) mRNA in situ hybridization. In PSP, we found a 50 to 60% decrease in the number of neurons expressing GAD67 mRNA in the caudate nucleus, ventral striatum, and the external and internal pallidum. The expression of GAD67 mRNA per neuron was reduced in the caudate nucleus and putamen (-43%), the ventral striatum (-55%), and the external and internal pallidum (-59% and -68%). Our data indicate that striatal and pallidal GABAergic neurotransmission is markedly reduced in PSP and we suggest that this alteration may account for the motor and cognitive symptoms observed in PSP. Furthermore, the destruction of the basal ganglia output systems may explain the lack of responsiveness to L-dopa therapy of PSP patients.NEUROLOGY 1995;45: 127-134

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Neurology (clinical)

Reference54 articles.

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2. Jellinger KA, Bancher C. Neuropathology, chapter 4. In: Litvan I, Agid Y, eds. Progressive supranuclear palsy. New York: Oxford University Press, 1992:44-88.

3. Hauw J-J, Daniel SE, Dickson D, et al. Preliminary NINDS neuropathologic criteria for Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Neurology 1994; 44:2015-2019.

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