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Familial progressive subcortical gliosis

D J Lanska¹, R D Currier, M Cohen

¹Department of Neurology, University of Kentucky Medical Center, Lexington 40536-0084.

|September 1, 1994

Summary

This study details a rare, inherited neurological disorder causing progressive dementia and behavioral changes. Pathological findings reveal widespread gliosis in the brain

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Area of Science:

Neurology
Neuroscience
Genetics

Background:

Familial forms of progressive subcortical gliosis are rare neurological disorders.
Understanding the clinical and pathological features is crucial for diagnosis and management.

Purpose of the Study:

To describe the clinical and pathological findings of a familial progressive subcortical gliosis.
To characterize the inheritance pattern and disease progression.

Main Methods:

Clinical case study of two kindreds with the disorder.
Autopsy findings from seven end-stage patients.
Histopathological examination of brain tissue.

Main Results:

The disorder followed an autosomal dominant inheritance pattern with presenium onset.

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Clinical manifestations included personality changes, dementia, and later, mutism and dysphagia.

Autopsy revealed generalized cerebral atrophy, prominent subcortical gliosis, and astrocytosis in specific brain regions, without myelin loss or amyloid deposits.

Conclusions:

This familial progressive subcortical gliosis presents with distinct clinical and pathological features.
The findings contribute to the understanding of inherited white matter disorders affecting the brain.