Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

An autopsied case of senile onset frontotemporal lobar degeneration

Yasushi Iwasaki, M.D.1), Keiko Mori, M.D.1), Masumi Ito, M.D.1), Akira Deguchi, M.D.2) and Mari Yoshida, M.D.3)

1)Department of Neurology, Oyamada Memorial Spa Hospital
2)Department of Internal Medicine, Oyamada Memorial Spa Hospital
3)Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University

A Japanese male with no family history of neurological disease or dementia showed behavioral abnormalities including egocentric and antisocial behavior at the age of 80. Over the next few years, other psychiatric symptoms such as allotriophagy and stereotypical behavior were also observed and his abnormal behavior became a social problem. Neurological examination revealed no apparent motor abnormalities, pyramidal and extrapyramidal signs, or ataxia. Aphasia, including semantic dementia was not apparent. The severity of memory disturbance was relatively milder than his psychiatric symptoms. Daily living activities and conversational ability were relatively maintained until shortly before his death at the age of 86. The clinical diagnosis was Alzheimer disease. Autopsy revealed that the brain weighed 950 g; frontotemporal atrophy with lateral ventricular dilatation was apparent. Neuron loss, gliosis, and tissue rarefaction were recognized in the frontotemporal cortex, subiculum, transentorhinal cortex, amygdala, and insular cortex and were particularly noticeable in the superficial layer of the cortex. Many ubiquitin-positive/TDP-43 positive but tau-negative dystrophic neurites with a few neuronal cytoplasmic inclusions were widely observed. Neuronal cytoplasmic inclusions were also observed in the dentate gyrus of the hippocampus. Although the spinal cord was not investigated, there was no apparent involvement of the motor neuron system. Small numbers of neurofibrillary tangles and senile plaques were observed, corresponding to Braak stage II and CERAD stage B, respectively. Argyrophilic grains, Lewy bodies and Pick bodies were not observed. The patient was pathologically diagnosed with frontotemporal lobar degeneration with ubiquitinpositive/TDP-43-positive inclusions (FTLD-TDP) and without motor neuron disease. No mutation was found in the TDP-43 gene. We considered the psychiatric symptoms and head CT findings of the present patient to be important observations for helping to discriminate between Alzheimer disease or other neurodegenerative diseases with dementia, and FTLD-TDP.
Full Text of this Article in Japanese PDF (727K)

(CLINICA NEUROL, 51: 426|429, 2011)
key words: frontotemporal lobar degeneration, psychiatric symptom, dystrophic neurite, ubiquitin, TDP-43

(Received: 10-Nov-10)