Abstract:
:We report a sporadic case of tauopathy with unusual clinical and neuropathological features. The patient presented with progressive symmetric rigid-akinetic parkinsonism and dementia of the subcortical type. Magnetic resonance imaging of the brain revealed atrophy resembling multiple system atrophy. The level of cerebrospinal fluid tau protein phosphorylated at serine 199 was markedly elevated. The autopsy revealed more glial than neuronal tauopathy, with much heavier involvement of subcortical white matter and the brainstem than of the cerebral cortex. Analysis of dephosphorylated tau revealed that hyperphosphorylated four-repeat tau isoforms were deposited in the brain of the patient. Despite morphological and biochemical resemblance to a certain form of familial fronto-temporal dementia, no mutation of the tau gene including exon 10 could be found. Our findings, taken together with those in previous similar case reports, indicate that the case represents an atypical form of corticobasal degeneration or a new variant of sporadic tauopathy.
journal_name
Neurosci Lettjournal_title
Neuroscience lettersauthors
Ohara S,Tsuyuzaki J,Oide T,Arai H,Higuchi S,Hasegawa M,Iwatsubo Tdoi
10.1016/s0304-3940(02)00616-xkeywords:
subject
Has Abstractpub_date
2002-09-13 00:00:00pages
84-8issue
1eissn
0304-3940issn
1872-7972pii
S030439400200616Xjournal_volume
330pub_type
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