Progressive subcortical gliosis

Introduction
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By Douglas J Lanska MD MS MSPH

Progressive subcortical gliosis is also known as or subsumes Primary subcortical gliosis (PSG) and Pick disease, type II. -ed.

In this clinical article, Dr. Douglas Lanska, staff neurologist at the VA Medical Center in Tomah, Wisconsin, 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.