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Parkinson's Disease

Evidence of CJD

06/03/1998

Question:

My aunt has suffered from dementia since 1983 and we thought it was Alzheimer and indeed until 1995 she was diagnosed as having Alzheimer. But the physician said the CT of the head showed atrophy without evidence of infarct. Whatever that means and that she has CJD. But her condition hasn't changed substantially. How could this be CJD and when she was hospitalized for six months and given a 1001 tests no one said CJD. Is this enough to determine CJD because now the nursing home won't give her any physical treatment and for the last three years she just sits in a "Jerry" chair and sleeps in bed. That's it in three years. We can't take her out of the home of day trips. We can't feed her by mouth (she may suffocate they say.) She doesn't lose weight. She just cries when she sees us. We thought CJD was worse than AIDS and quick and deadly how can someone have it for years and not change drastically? How can we get them to change the diagnosis? We know they want to make her DNR but we're against it we know she isn't well and won't get well but she can still smile and giggle. And she continues to touch our heart.

Answer:

CJD (Creutzfeld Jakob Disease) is a rapidly progressive dementia. It belongs to a group of disorders called prion diseases. You are right in assuming that it progresses rapidly (we are talking 6 months to a year) to death. Certainly with onset of dementia in 1983 this diagnosis is very unlikely. There are no specific tests for CJD short of brain biopsy. EEG recordings may show a characteristic finding. MRI findings are non-specific.

Other possible diagnoses include: Alzheimer's (still a possibility; again, no blood tests or MRI findings can clinch the diagnosis), Corticobasal degeneration, and Diffuse Lewy body disease. Unfortunately none of these is a curable condition.

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Response by:

Arif Dalvi, MD
Assistant Professor
College of Medicine
University of Cincinnati