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Familial Encephalopathy with Neuroserpin Inclusion Bodies

Abstract

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NORD is very grateful to Richard L. Davis MD, PhD, Assistant Professor of Pathology, SUNY Upstate Medical University, for assistance in the preparation of this report.

Synonyms of Familial Encephalopathy with Neuroserpin Inclusion Bodies

familial encephalopathy with Collins bodies
FENIB

Disorder Subdivisions

No subdivisions found.

General Discussion

Familial encephalopathy with neuroserpin inclusion bodies (FENIB) is a rare genetic degenerative disorder affecting the brain and spinal cord, or central nervous system (neurodegenerative disorder). Affected individuals display poor attention and concentration, declining work or academic performance, and language difficulties. Eventually, they experience a global decline in their intellectual abilities (dementia). Memory, however, is relatively well-preserved early in the course of the disease compared to the severe memory deficits that are typical of Alzheimer's disease. Some affected individuals develop additional symptoms such as uncontrolled, irregular muscle contractions and seizures. Changes in mood, such as apathy, depression, or anger frequently occur. Eventually, affected individuals require comprehensive medical care.

Organizations related to Familial Encephalopathy with Neuroserpin Inclusion Bodies

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