Book ChapterDOI
Frontal lobe dementia and motor neuron disease
TLDR
The nosological status of FLD-MND remains enigmatic in the absence of defined pathological and molecular markers, whereas others suggest it represents an interface between FLD and "classic" (non-dementing) motor neuron disease (CMND).Abstract:
Frontal lobe dementia (FLD) (syn frontotemporal dementia and dementia of frontal type) is a generic term that describes a clinical syndrome in which patients manifest a profound breakdown in personality and social conduct, together with adynamic spontaneous speech, culminating in mutism This pattern of cognitive impairment implicates bilateral frontal lobe dysfunction, an assumption supported by functional neuroimaging findings of anterior cerebral abnormality Patients with FLD can go on to develop motor neuron disease (FLD-MND), although the clinical features of MND may accompany or occasionally precede the onset of dementia The emergence of MND is responsible for death within 3 years of onset Frontotemporal lobar pathology in FLD-MND is characterized by loss of large cortical neurons, spongiform change and mild astrocytic gliosis Ubiquitinated (but not tau-positive) inclusions are present within the frontal cortex There is severe nigral cell loss (without Lewy bodies), and marked hypoglossal and spinal motor neuron degeneration, together with ubiquitinated (but not tau-positive) inclusions within the spinal neurons Some authors suggest that FLD-MND is a separate disease entity, whereas others suggest it represents an interface between FLD and "classic" (non-dementing) motor neuron disease (CMND) An association with CMND is supported by findings in these patients of failure in tasks sensitive to "frontal lobe" dysfunction, and patterns of functional neuroimaging abnormality which are identical in distribution, but less severe than those encountered in FLD-MND However, the nosological status of FLD-MND remains enigmatic in the absence of defined pathological and molecular markersread more
Citations
More filters
Journal ArticleDOI
Frontotemporal lobar degeneration A consensus on clinical diagnostic criteria
David Neary,Julie S. Snowden,L. Gustafson,U. Passant,Donald T. Stuss,Sandra E. Black,Morris Freedman,Andrew Kertesz,Philippe Robert,Marilyn S. Albert,Kyle B. Boone,Bruce L. Miller,Jeffrey L. Cummings,D. F. Benson +13 more
TL;DR: Consensus criteria for the three prototypic syndromes-frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia-were developed by members of an international workshop on frontotem temporal lobar degeneration and ought to provide the foundation for research work into the neuropsychology, neuropathology, genetics, molecular biology, and epidemiology of these important clinical disorders.
Journal ArticleDOI
Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration
Nigel J. Cairns,Eileen H. Bigio,Ian R. A. Mackenzie,Manuela Neumann,Virginia M.-Y. Lee,Kimmo J. Hatanpaa,Charles L. White,Julie A. Schneider,Lea T. Grinberg,Glenda M. Halliday,Charles Duyckaerts,James Lowe,Ida Elisabeth Holm,Markus Tolnay,Koichi Okamoto,Hideaki Yokoo,Shigeo Murayama,John Woulfe,David G. Munoz,Dennis W. Dickson,Paul G. Ince,John Q. Trojanowski,David M. A. Mann +22 more
TL;DR: The aim of this study was to improve the neuropathologic recognition and provide criteria for the pathological diagnosis in the neurodegenerative diseases grouped as frontotemporal lobar degeneration (FTLD) and incorporate up-to-date neuropathology in the light of recent immunohistochemical, biochemical, and genetic advances.
Journal ArticleDOI
Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria
Michael J. Strong,Sharon Abrahams,Laura H. Goldstein,Susan C. Woolley,Paula M. McLaughlin,Julie S. Snowden,Eneida Mioshi,Angie Roberts-South,Michael Benatar,Tibor Hortobágyi,Jeffrey Rosenfeld,Vincenzo Silani,Paul G. Ince,Martin R Turner +13 more
TL;DR: These revised consensus criteria expand upon those of 2009 and embrace the concept of the frontotemporal spectrum disorder of ALS (ALS-FTSD), which is a re-conceptualisation that neuropsychological deficits in ALS fall along a spectrum.
Journal ArticleDOI
Cognitive impairment in amyotrophic lateral sclerosis
TL;DR: Understanding of cognitive impairment in ALS will improve care for patients and their families and provide valuable insights into the pathogenesis of neurodegeneration.
Journal ArticleDOI
Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease-dementia-aphasia syndrome
TL;DR: The finding of a selective impairment of verb/action processing in association with the dementia/aphasia syndrome of MND suggests that the neural substrate underlying verb representation is strongly connected to anterior cortical motor systems.
References
More filters
Journal ArticleDOI
Dementia of frontal lobe type.
TL;DR: Comparisons of DFT and Alzheimer patients revealed qualitative differences in clinical presentation, neurological signs, profile of psychological disability, electroencephalography, single photon emission tomography and demography, which suggests DFT may be more common than is often recognised.
Journal ArticleDOI
Frontal lobe dementia and motor neuron disease.
TL;DR: The clinical picture and pathological findings resembled those of dementia of frontal-lobe type and were distinct from those of Alzheimer's disease, and have implications for the understanding of the spectrum of non-Alzheimer forms of primary degenerative dementia.
Journal ArticleDOI
Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review
Journal ArticleDOI
Frontal lobe degeneration of non-Alzheimer type. I. Neuropathology
TL;DR: FLD is in some morphological respects similar to other dementing disorders such as the ALS dementia complex and progressive subcortical gliosis, though with both clinical and clear-cut pathoanatomical differences.
Journal ArticleDOI
Frontal lobe degeneration: clinical, neuropsychological, and SPECT characteristics.
Bruce L. Miller,Jeffrey L. Cummings,Javier Villanueva-Meyer,Kyle B. Boone,C. M. Mehringer,Ira M. Lesser,Ismael Mena +6 more
TL;DR: The clinical, neuropsychological, and cerebral blood flow characteristics of eight patients with frontal lobe degeneration were studied and showed selective impairment of frontal and memory tasks with relative sparing of attention, language, and visuospatial skills.