Research summary
Hyperphosphorylated, ubiquitinated, and C-terminally cleaved TDP-43 was identified as the major disease protein in frontotemporal lobar degeneration with ubiquitin-positive inclusions and in amyotrophic lateral sclerosis; pathologic TDP-43 was recovered only from affected CNS regions including hippocampus, neocortex and spinal cord [1]. The 2005 DLB Consortium revised clinical and pathologic criteria for dementia with Lewy bodies, giving REM sleep behavior disorder, severe neuroleptic sensitivity and reduced striatal dopamine-transporter activity greater diagnostic weight as suggestive features [2]. The 2017 DLB Consortium update incorporated MIBG myocardial scintigraphy and other neuroimaging, electrophysiologic and laboratory investigations, added minor modifications to pathologic methods including Alzheimer-disease neuropathologic change assessment and substantia nigra neuronal loss, and noted limited randomized-trial evidence for clinical management [3]. The 2012 NIA-Alzheimer's Association neuropathologic guidelines updated 1997 criteria to recognize a pre-clinical AD stage, added amyloid accumulation alongside neurofibrillary change and neuritic plaques, and established protocols for Lewy body disease, vascular brain injury, hippocampal sclerosis and TDP-43 inclusion assessment [4]. The 2008 second consensus statement on multiple system atrophy revised 1998 diagnostic criteria across parkinsonian, cerebellar, autonomic, neuropathologic and imaging aspects through a 2007 consensus conference [5].
Recent publications
- α-Synuclein in Lewy bodiesDOI
- Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral SclerosisDOI
- Diagnosis and management of dementia with Lewy bodiesDOI
- Hypothetical model of dynamic biomarkers of the Alzheimer's pathological cascadeDOI
- Tracking pathophysiological processes in Alzheimer's disease: an updated hypothetical model of dynamic biomarkersDOI
- Diagnosis and management of dementia with Lewy bodiesDOI
- Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17DOI
- National Institute on Aging–Alzheimer's Association guidelines for the neuropathologic assessment of Alzheimer's diseaseDOI
- Second consensus statement on the diagnosis of multiple system atrophyDOI
- National Institute on Aging–Alzheimer’s Association guidelines for the neuropathologic assessment of Alzheimer’s disease: a practical approachDOI
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External profiles
- ORCID: https://orcid.org/0000-0002-9239-8794
- OpenAlex: openalex.org
Profile compiled from public sources (Researchmap, OpenAlex, The University of Tokyo faculty directory). Last refreshed 2026-05. Report incorrect information.