Linked Life Data

18210850

Source:http://linkedlifedata.com/resource/pubmed/id/18210850

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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
11
pubmed:dateCreated
2008-1-23
pubmed:abstractText
During 1996 and 2006, we examined clinically 37 patients and neuropathologically 13 autopsy cases with amyotrophic lateral sclerosis/parkinsonism-dementia complex of the Kii peninsula (Kii ALS/PDC). The ages of onset were between 52 years and 74 years (mean age: 65.3 years). The male to female ratio was 1:1.85. The ratio of positive family history where ALS or PDC occurred within the fourth degree of the relatives was 78.4% in the patients with Kii ALS/PDC. The average duration of the illness was 6.47 years. Kii ALS/PDC was divided into five clinical subtypes, pure ALS form, ALS with dementia form, PDC with parkinsonism predominant form, PDC with dementia predominant form (that is called late-life dementia in Guam) and PDC with ALS features form. Unique pigmentary retinopathy was found in 33.3% of the patients with Kii ALS/PDC. CT/MRI images showed atrophy of the frontal and temporal lobes and SPECT images showed a decrease in the blood flow of the frontal and temporal lobes. The cardiac 123I-MIBG uptake was decreased in 4 out of 8 patients with ALS/PDC and the decrease in uptake correlated with the modified Hoehn-Yahr staging. The cardinal neuropathological features of Kii ALS/PDC were abundant neurofibrillary tangles (NFTs) associated with loss of nerve cells in the cerebral cortex and the brain stem, and findings of ALS neuropathology. Ultrastructurally, NFTs consisted of paired helical filaments. Tau protein, a main component of NFTs, was consisted of 3R and 4R tau isoforms, and phosphoryrated at 18 sites of tau phosphoryrated sites. The neurons of dentate gyrus of hippocampus and anterior horn cells were stained with anti-TDP-43 antibody. The clinical and neuropathological aspects of Kii ALS/PDC are regarded as being identical with those of Guam ALS/PDC.
pubmed:language
jpn
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Nov
pubmed:issn
0009-918X
pubmed:author
pubmed:issnType
Print
pubmed:volume
47
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
966-9
pubmed:meshHeading
pubmed:year
2007
pubmed:articleTitle
[Clinical aspects, imaging and neuropathology of Kii ALS/PDC].
pubmed:affiliation
Department of Neurology, Mie University School of Medicine.
pubmed:publicationType
Journal Article, English Abstract