Autopsy proven sporadic frontotemporal dementia due to microvacuolar-type histology, with onset at 21 years of age

被引：16

作者：

Snowden, JS ^{[1
]}

Neary, D ^{[1
]}

Mann, DMA ^{[1
]}

机构：

[1] Univ Manchester, Hope Hosp, Greater Manchester Neurosci Ctr, Neurosci Res Grp, Salford M6 8HD, Lancs, England

来源：

JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY | 2004年 / 75卷 / 09期

关键词：

D O I：

10.1136/jnnp.2003.028498

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

We describe the clinical, neuropsychological, and neuropathological features of a 21 year old woman with frontotemporal dementia (FTD). The early presentation was of florid behavioural change involving hyperactivity and disinhibition. Magnetic resonance imaging and single photon emission computed tomography of the brain revealed atrophy and severe functional abnormalities of the frontal and temporal lobes, respectively. Electroencephalogram was normal. At autopsy, there was gross frontotemporal brain atrophy and the underlying histology was of a microvacuolar-type degeneration; no tau or ubiquitin immunoreactive, intraneuronal inclusions were seen. There was no family history of dementia and no mutation in the tau gene. We believe this patient represents the youngest ( so far) recorded case of FTD associated with this particular histological form of the disorder.

引用

页码：1337 / 1339

页数：3

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