Progressive subcortical gliosis

Douglas J Lanska MD FAAN MS MSPH (Dr. Lanska of the Great Lakes VA Healthcare System and the University of Wisconsin School of Medicine and Public Health has no relevant financial relationships to disclose.)
Originally released May 25, 1993; last updated January 29, 2017; expires January 29, 2020

This article includes discussion of progressive subcortical gliosis; PSG; primary subcortical gliosis; Pick disease, type II; and Pick disease, type 2. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

The author explains the clinical presentation, etiology, differential diagnosis, and diagnostic workup of progressive subcortical gliosis, a chromosome-17-linked dementia with unique pathologic features. Microscopically, the major pathologic change is a marked fibrillary astrocytosis, particularly in the area of the short cortical association tracts at the junction of cortical lamina VI and the subcortical white matter and in the subpial cerebral cortex. Overall, the survival of patients with progressive subcortical gliosis averages about 10 years and is similar to that of other types of frontotemporal dementia.

Key points

 

• Progressive subcortical gliosis is a rare dementing disorder resembling Pick disease but with distinctive neuropathologic features. The clinical manifestations are those of a frontotemporal dementia and overlap with those of other frontotemporal dementias.

 

• Progressive subcortical gliosis has an insidious onset, generally in the fifth or sixth decade. The course is progressive, generally over 5 to 15 years, but both fulminant and protracted courses occur.

 

• Common initial manifestations include personality and emotional changes, lack of judgment and insight, deterioration in social behavior, delusions, paranoia, auditory and visual hallucinations, and depression.

 

• Microscopically, the major pathologic change is a marked fibrillary astrocytosis, particularly in the area of the short cortical association tracts at the junction of cortical lamina VI and the subcortical white matter and in the subpial cerebral cortex.

 

• Most cases have been sporadic, but some hereditary forms are recognized, with linkage demonstrated in 1 kindred to a region on the long arm of chromosome 17 (17q21-22).

 

• Overall, the survival of patients with progressive subcortical gliosis averages about 10 years and is similar to that of other types of frontotemporal dementia.

Historical note and terminology

Progressive subcortical gliosis is a rare dementing disorder resembling Pick disease but with distinctive neuropathologic features. In 1949, Neumann described 3 cases under the appellation of "Pick disease, type II" (Neumann 1949). In 1967, Neumann and Cohn reported 4 additional cases and suggested the designations "primary subcortical gliosis" and "progressive subcortical gliosis" (Neumann and Cohn 1967); although neither of these terms is ideal, the latter has become established in the literature (Brun and Gustafson 2011).

Although rare reports of familial cases were reported beginning in the 1940s, it was not until the late 1980s and 1990s that it was recognized that progressive subcortical gliosis could be transmitted as an autosomal dominant trait (Currier et al 1986; Lanska et al 1991; Lanska et al 1994). Progressive subcortical gliosis was linked to a tau mutation on the long arm of chromosome 17 (Petersen et al 1995; Goedert et al 1999) and, thus, included in the "frontotemporal dementias and Parkinsonism linked to chromosome 17" group (Foster et al 1997; Brun and Gustafson 2011).

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