The Japanese journal of neuropsychology

Full Text of this Article
in Japanese PDF (1839K)

ArticleTitle	Neurodegenerative disorders presenting with progressive focal cortical dysfunction
Language	J
AuthorList	Kenji Ishihara
Affiliation	Department of Neurology, Showa University School of Medicine
Publication	Japanese Journal of Neuropsychology: 25 (2), 118-127, 2009
Received
Accepted
Abstract	1) I reviewed historical aspects of the neurodegenerative disorders presenting with progressive focal cortical dysfunction in the early stage, which ultimately reveal generalized dementia. In 1982, Mesulam reported six cases presenting with slowly progressive aphasia without generalized dementia. Many cases of progressive aphasia have been reported since then. These days, the spectrum of "frontotemporal lobar degeneration (FTLD)", including the clinical subgroup of progressive aphasia, is widely accepted all over the world. The pathlogical diagnostic criteria of FTLD reported in 2007 was introduced. 2) Four cases of neurodegenerative disorders presenting with progressive cortical dysfunction were described. Clinical and pathological diagnosis of each case were shown and discussed in the context of pathlogical diagnostic criteria of FTLD. 3) The nosological significance of "slowly progressive focal cortical dysfunction" was discussed. Both clinical and pathological approaches are required for the accurate clinical diagnosis in future, leading to the effective therapy. Clinical analysis using video images would be useful for the accurate description of clinical features in each case presenting with progressive focal cortical dysfunction.
Keywords	progressive focal cortical dysfunction, neurodegenerative disorder, video image