Twin pairs discordant for neuropathologically confirmed Lewy body dementia.
OBJECTIVE
Little is known about the concordance rate in twins for dementia with Lewy bodies (DLB). The rate of agreement between clinical and pathological diagnoses for DLB is typically low, necessitating confirmation of the diagnosis neuropathologically.


METHODS
Participants were 17 twin pairs enrolled in the Duke Twins Study of Memory in Aging in which at least one member of the pair had an autopsy confirmed diagnosis of DLB, Alzheimer's disease (AD) with Lewy bodies or frontotemporal dementia with Lewy bodies. The characteristics of those with dementia were assessed and rates of concordance for pathological confirmed dementia were examined.


RESULTS
Four monozygotic twin pairs had a proband with neuropathologically confirmed pure DLB; all remained discordant for dementia for periods up to 16 years or more. Five of 13 pairs in which the proband had AD plus DLB were concordant for dementia but only one pair was concordant for AD plus DLB, while the co-twins in the other four pairs had other types of dementia.


CONCLUSIONS
The present study indicates that even among twins, a diagnosis of DLB in one twin does not predict the same diagnosis in the other twin. Neuropathological discordance in type of dementia among monozygotic pairs hints at environmental or epigenetic factors playing a role in Lewy body pathology.
Department of Psychiatry, Tainan Hospital, Department of Health, Taiwan.
C Sheei-Meei
CS
Wang
J R
JR
Burke
D C
DC
Steffens
C M
CM
Hulette
J C S
JC
Breitner
B L
BL
Plassman
D017668:Age of Onset; D000368:Aged; D000544:Alzheimer Disease / Q000235:genetics / Q000473:pathology / Q000523:psychology; D001057:Apolipoproteins E / Q000235:genetics; D001921:Brain / Q000473:pathology; D004493:Education; D005260:Female; D005838:Genotype; D006801:Humans; D020961:Lewy Body Disease / Q000235:genetics* / Q000473:pathology / Q000523:psychology; D008297:Male; D008875:Middle Aged; D014429:Twins, Dizygotic; D014430:Twins, Monozygotic
D001057:Apolipoproteins E