Re: RESEARCH: unusual case of sporadic tauopathy>Pam

[Guest guest]

Pam,

Thank you. I have printed this out and I am going to take it to my

doctor. I am also going to look into this more.

Love and hugs,

Belinda

> A clinical and neuropathological study of an unusual case of

sporadic

> tauopathy. A variant of corticobasal degeneration?

>

> Shinji Ohara, , a, Jun Tsuyuzakia, Takashi Oidea, Hiroyuki Araib,

Susumu

> Higuchic, Masato Hasegawad and Takeshi Iwatsuboe

>

> a Department of Neurology, National Chushin-Matsumoto Hospital, 811

> Kotobuki, Matsumoto 399-0021, Japan

> b Department of Geriatrics, Tohoku University School of Medicine,

Sendai,

> Japan

> c Institute of Clinical Research, Kurihama National Hospital,

Kanagawa,

> Japan

> d Department of Molecular Neurology, Tokyo Institute of Psychiatry,

Tokyo,

> Japan

> e Department of Neuropathology and Neuroscience, Graduate School of

> Pharmaceutical Sciences, University of Tokyo, Tokyo, Japan

>

> Received 18 April 2002; revised 4 June 2002; accepted 6 June 2002.

> Available online 29 August 2002.

>

>

>

> 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.