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 DLB Case that Temporarily Showed CJD-like Decline 
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Joined: Fri Aug 11, 2006 1:46 pm
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Location: SF Bay Area (Northern CA)
Post DLB Case that Temporarily Showed CJD-like Decline
Based on this case of probable DLB and some others that were brought to light through a literature search, these Japanese researchers are proposing a "rapidly progressive clinical subtype of DLB" that looks like Creutzfeldt-Jakob disease (CJD).

The present case is of a 67-year-old man who was initially thought to be in the early stages of DLB. He was given Risperdal and Ativan to treat hallucinations and improve sleep. "However, his symptoms worsened, except for his sleep patterns, which improved. Myoclonus of his extremities and severe gait disturbance appeared. He became unable to recognize his wife and daughters. The second MMSE score was 15 points and CDT [clock drawing test] could not be carried out. Besides these symptoms, he became increasingly apathetic and lost a lot of weight."

Due to this rapid deterioration, it was thought this man had CJD. But after all kinds of testing, no evidence of CJD or any other encephalopathy could be found.

So it seems the researchers returned to the DLB diagnosis, and added Aricept (donepezil) to his "existing medications." "Approximately 1 month later, his cognition rapidly recovered. ... Visual hallucination, myoclonus and gait disturbance were also improved."

"After recovery of his clinical symptoms, risperidone and benzodiazepine were gradually reduced over the course of 2 months, and finally his medication was just 5 mg donepezil. However, after that, no hallucinations appeared again."

I imagine many of you are wondering, as I did, whether the Risperdal and/or Ativan caused the rapid deterioration of problems. I guess since the Aricept led to a huge improvement in symptoms, while the Risperdal and Ativan were still being given, this is evidence that it wasn't Risperdal or Ativan causing the deterioration.

The authors say: "The cause of rapidly progressive deterioration is unknown. However, at least in our case, it seemed to be associated with disturbance of consciousness... [Recovery] of the second EEG findings in accordance with improvement of clinical symptoms is further evidence that rapidly progressive deterioration is a result of a disturbance of consciousness."

After doing a literature search, the authors found 4 other cases of rapidly progressive DLB that looked like CJD. In 3 of these cases, Aricept was used to good effect. "In contrast, two patients, for whom donepezil therapy was not given or was unsuccessful, died after several months. We believe that our patient also would have died from this disease, if donepezil had not been given." (That's quite an advertisement for Aricept.)

As for autopsy confirmation of the DLB diagnoses... The present case is still alive. Two of the four cases found through literature searches did not donate their brains for autopsy. Two did: one had AD plus DLBD, and the other had pure Lewy body disease (by which I believe they mean "Parkinson's Disease").

These case reports can be fascinating and frustrating at the same time. It's wonderful that so many details are given on a particular patient but often I want to ask "why did you reach this conclusion?"

I've copied the abstract below.

Robin



Psychogeriatrics. 2010 Dec;10(4):201-5. Epub 2010 Nov 26.

A case of dementia with Lewy bodies that temporarily showed symptoms similar to Creutzfeldt-Jakob disease.

Ukai K, Matsuo K, Nakamura R, Sobue G, Kosaka K, Shibayama H, Iritani S, Ishihara R, Ozaki N.
Kamiiida Daiichi General Hospital, Departments of Psychiatry Neurology, Nagoya University Graduate School of Medicine, Nagoya HouYuu Hospital, Yokohama, Japan.

Abstract
We discuss a case of a 67-year-old male with dementia with Lewy bodies (DLB) that was initially suspected as Creutzfeldt-Jakob disease (CJD) or another type of encephalopathy, because he showed rapidly progressive deterioration, myoclonus, gait disturbance and a decline in activities of daily living. The present study describes a clinically atypical case with probable DLB and reviews similar cases in the literature, and we propose a rapidly progressive clinical subtype of DLB.

© 2010 The Authors. Psychogeriatrics © 2010 Japanese Psychogeriatric Society.

PubMed ID#: 21159056


Fri Dec 17, 2010 1:54 pm
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Joined: Fri Jan 15, 2010 9:33 pm
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Location: Vermont
Post Re: DLB Case that Temporarily Showed CJD-like Decline
Sounds a lot like my dad, when he went from living at home with 4 hours' assistance a day, living in his house by himself, to no ADLs and all 4 limbs almost totally paralyzed overnight. Lynn

_________________
Lynn, daughter of 89 year old dad dx with possiblity of LBD, CBD, PSP, FTD, ALS, Vascular Dementia, AD, etc., died Nov. 30, 2010 after living in ALF for 18 months.


Fri Dec 17, 2010 4:59 pm
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Joined: Fri Aug 11, 2006 1:46 pm
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Location: SF Bay Area (Northern CA)
Post Re: DLB Case that Temporarily Showed CJD-like Decline
Was CJD one of the possible diagnoses for your father? Usually those diagnosed with CJD die within a year of diagnosis; it's lightning fast.

Your father seemed to have had insight. Those with DLB don't seem to have this level of insight. Not sure about CJD.


Fri Dec 17, 2010 5:09 pm
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Joined: Fri Jan 15, 2010 9:33 pm
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Location: Vermont
Post Re: DLB Case that Temporarily Showed CJD-like Decline
No doctor ever suggested that - the only person who ever brought up the possibility of CJD was you, here on the forum. So, I started reading about it.
His physical decline was pretty noticeable since about the fall of 2007, but he was still mobile and doing most things for himself and living at home by himself until July, 2009. Overnight he could not stand, sit, walk, feed himself, use the remote control, turn over (this had been a problem for months I think prior to going to the ER and being admitted to the hosp.), His cognition took a nose-dive too, and he had hallucinations in the hosp. After 3 weeks in rehab he was able to walk with a walker, feed himself, and cooperate with the PTs. After he moved into the ALF he no longer cooperated with the PTs, only used his wheelchair, couldn't stand up by himself, quit walking, cognition came and went. By about winter 2010 he could barely use his arms/hands, and had to be fed. He died on 11/30/2010, so from his huge decline to death was about 18 months. But, he did have lucid minutes/hours during that 18 months. His long term memory was amazingly functional till he died. A few days before he died he was joking with some old friends - the same jokes he had told them before he became so ill. But, couldn't remember he'd just eaten or whatever.
With CJD, do people lose all cognition and touch with reality? Thanks, Lynn

_________________
Lynn, daughter of 89 year old dad dx with possiblity of LBD, CBD, PSP, FTD, ALS, Vascular Dementia, AD, etc., died Nov. 30, 2010 after living in ALF for 18 months.


Fri Dec 17, 2010 6:01 pm
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Joined: Fri Aug 11, 2006 1:46 pm
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Location: SF Bay Area (Northern CA)
Post Re: DLB Case that Temporarily Showed CJD-like Decline
It's a good question. I don't know that much about CJD except that it mimics many disorders including LBD, PSP, and CBD.


Fri Dec 17, 2010 9:52 pm
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Joined: Fri Jan 15, 2010 9:33 pm
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Location: Vermont
Post Re: DLB Case that Temporarily Showed CJD-like Decline
Interesting since some of my dad's many possibilities included LBD, CBD & PSP. No wonder a dx is so difficult and the symptoms are so overlapping. Lynn

_________________
Lynn, daughter of 89 year old dad dx with possiblity of LBD, CBD, PSP, FTD, ALS, Vascular Dementia, AD, etc., died Nov. 30, 2010 after living in ALF for 18 months.


Fri Dec 17, 2010 9:57 pm
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