Neuronal Ultrastructural Abnormalities in a Patient with Frontotemporal Dementia and Motor Neuron Disease

1995 ◽  
Vol 6 (1) ◽  
pp. 1-8 ◽  
Author(s):  
Linda Chang ◽  
Marcia Cornford ◽  
Bruce L. Miller ◽  
Hideo Itabashi ◽  
Ismael Mena
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Frontotemporal Dementia

Neuronal and glial inclusions in frontotemporal dementia with or without motor neuron disease are immunopositive for p62

2003 ◽  
Vol 342 (1-2) ◽  
pp. 41-44 ◽  
Author(s):  
Tetsuaki Arai ◽  
Takashi Nonaka ◽  
Masato Hasegawa ◽  
Haruhiko Akiyama ◽  
Mari Yoshida ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Frontotemporal Dementia

Globular glial tauopathies (GGT) presenting with motor neuron disease or frontotemporal dementia: an emerging group of 4-repeat tauopathies

2011 ◽  
Vol 122 (4) ◽  
pp. 415-428 ◽  
Author(s):  
Zeshan Ahmed ◽  
Karen M. Doherty ◽  
Laura Silveira-Moriyama ◽  
Rina Bandopadhyay ◽  
Tammaryn Lashley ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Frontotemporal Dementia

scholarly journals 016 One disease or three: is frontotemporal dementia – motor neuron disease a distinct entity?

2019 ◽  
Vol 90 (e7) ◽  
pp. A6.2-A6
Author(s):  
Zhe (Jill) Long ◽  
Muireann Irish ◽  
David Foxe ◽  
John Hodges ◽  
Olivier Piguet ◽  
...  
Keyword(s):  
Cluster Analysis ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Frontotemporal Dementia ◽  
Cortical Atrophy ◽  
Voxel Based Morphometry ◽  
Disease Group ◽  
Cognitive Behavioural ◽  
Data Driven Approach ◽  
Patient Subgroups

IntroductionFrontotemporal dementia-motor neuron disease (FTD-MND) is diagnosed when patients meet criteria for the diagnosis of both FTD and MND, but the mode presentation of this disorder is currently unknown. This study aimed to compare the mode of presentation, and profiles of behavioural and language disturbances, of FTD-MND with that of other FTD phenotypes using a data-driven approach.Methods31 FTD-MND, 119 bvFTD, 47 PNFA, 42 SD patients and 127 controls underwent comprehensive clinical, neuropsychological and neuroimaging assessments. Z-transformed scores were used to compare the severity of behavioural and language domains in each disease group. Two-step cluster analysis profiled patient subgroups. Voxel-based morphometry investigated differential patterns of cortical atrophy between groups.ResultsOverall, FTD-MND patients presented with behavioural or language disturbances less frequently than FTD phenotypes, but mixed behavioural-language presentations were more common. FTD-MND patients demonstrated less severe disinhibition, apathy and semantic deficits relative to bvFTD and SD respectively.Behavioural and language deficits were of comparable severity in FTD-MND, unlike other FTD phenotypes where behaviour was worse than language (bvFTD) or language worse than behaviour (PNFA, SD).In cluster analysis, FTD-MND patients were evenly distributed across three subgroups designated as ‘mild mixed’, ‘language dominant’ and ‘behavioural dominant’. Relative to the ‘mild mixed’ group, ‘language dominant’ patients demonstarted more atrophy of the anterior temporal lobe and peri-insular regions, while ‘behavioural dominant’ patients displayed more prefrontal atrophy.ConclusionFTD-MND does not present as a uniform syndrome. Rather, there may be at least three subgroups that demonstrate distinctive cognitive, behavioural, and neuroanatomical characteristics.

One disease or three: Is frontotemporal dementia – Motor neuron disease a distinct entity?

2019 ◽  
Vol 405 ◽  
pp. 70
Author(s):  
Z. Long ◽  
M. Irish ◽  
D. Foxe ◽  
J. Hodges ◽  
O. Piguet ◽  
...  
Keyword(s):  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Frontotemporal Dementia ◽  
Distinct Entity
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