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 Case report - DLB with ataxia, myoclonus, and blindness 
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Joined: Fri Aug 11, 2006 1:46 pm
Posts: 4811
Location: SF Bay Area (Northern CA)
Post Case report - DLB with ataxia, myoclonus, and blindness
A British case report was recently published* of a man diagnosed at age 67 with Alzheimer's Disease. Several months later, the diagnosis was changed to CJD (Creutzfeldt-Jakob disease) due to rapidly pressing dementia (over less than 2 years), ataxia, blindness, and myoclonus. He died about a year after the AD diagnosis. The family donated the brain for medical research. It was found he had Dementia with Lewy Bodies and Alzheimer's pathology as well.

The authors state: "This case of DLB appears unique in which the cortical blindness was associated with frequent Lewy bodies in the visual cortex. It was also unusual for the history of dementia without hypokinesia (despite Lewy body pathology in the substantia nigra) and the subacute final illness masquerading as CJD. Myoclonus is a common feature of DLB with ataxia occurring less common. Blindness can rarely be a feature of DLB. ... There are only two reported cases with DLB and blindness."

Here are my definitions of a few terms used above:
hypokinesia = reduced movements
myoclonus = jerky movements
ataxia = uncoordinated movement

I've copied the citation below.

Robin



* Letter to the Editor
Dementia with Lewy bodies associated with ataxia, myoclonus, and cortical blindness

Movement Disorders. 2010 Mar 22.

Hindle JV, Bridges L, Chohan G.
Department of Care of the Elderly, School of Medical Sciences, Bangor University, Bangor, United Kingdom


Wed Mar 24, 2010 5:11 pm
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Joined: Wed Dec 30, 2009 1:46 pm
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Location: WA
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Wow! I guess that's something we can be thankful for--that our LO isn't blind!


Wed Mar 24, 2010 5:19 pm
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Joined: Fri Aug 11, 2006 1:46 pm
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Location: SF Bay Area (Northern CA)
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Yesterday, I sat with a widow and a neurologist to review the neuropathology report. The confirmed diagnosis was DLBD. The neuropathologist's report indicated that the patient must've been blind in one eye because they had severe optic atrophy. The wife said that the husband never mentioned it! She thinks he must've been blinded when he was 18 and got into a street fight. So, apparently you can be blind in one eye and not even know it.


Wed Mar 24, 2010 5:26 pm
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Joined: Wed Dec 30, 2009 1:46 pm
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Location: WA
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You'd think when they renew their driver's license? [Before LBD sets in, that is].


Wed Mar 24, 2010 5:32 pm
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Joined: Fri Aug 11, 2006 1:46 pm
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Location: SF Bay Area (Northern CA)
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I'm not sure you have to cover one eye to take the DL eye test.


Wed Mar 24, 2010 5:58 pm
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Joined: Wed Dec 30, 2009 1:46 pm
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Location: WA
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In WA, they test one eye at a time. If you are blind in one eye, it affects your depth perception.


Wed Mar 24, 2010 6:05 pm
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Joined: Fri Jan 15, 2010 9:33 pm
Posts: 3430
Location: Vermont
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I would think that a 67 year old person would have had a number of eye tests in his lifetime..... This is really strange..... Perhaps he knew it but never told anyone, like his wife? I'd have thought there would be obvious symptoms of not having depth perception over that many years.


Wed Mar 24, 2010 9:43 pm
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