Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal changes: broadening the clinical picture to include progressive supranuclear palsy

The frontotemporal lobar degenerations (FTLDs) are a group of disorders in which the clinical picture is not necessarily predictive of the underlying neuropathology. The FTLD with ubiquitin-only-immunoreactive neuronal changes (FTLD-U) subtype is pathologically characterized by ubiquitin-positive, t...

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Bibliographic Details
Published in:Brain (London, England : 1878) England : 1878), 2004-11, Vol.127 (11), p.2441-2451
Main Authors: Paviour, D. C., Lees, A. J., Josephs, K. A., Ozawa, T., Ganguly, M., Strand, C., Godbolt, A., Howard, R. S., Revesz, T., Holton, J. L.
Format: Article
Language:English
Subjects:
Aged
Autopsy
Biological and medical sciences
Brain - pathology
CAA = cerebral amyloid angiopathy
CBD = corticobasal degeneration
Cerebrospinal fluid. Meninges. Spinal cord
clinico-pathological correlation
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Dementia - metabolism
Dementia - pathology
Diagnosis, Differential
DUI = dementia with tau-negative ubiquitin-positive inclusions
EMG = electromyography
Female
FTD = frontotemporal dementia
FTDP-17 = frontotemporal dementia with parkinsonism linked to chromosome 17
FTLD = frontotemporal lobar degeneration
FTLD-MND
FTLD-MND = FTLD with motor neuron disease
FTLD-U
FTLD-U = FTLD with ubiquitin-only-immunoreactive neuronal changes
Humans
ITSNU = inclusions
Male
Medical sciences
Middle Aged
MMSE = mini-mental state examination
MND = motor neuron disease
MND-ID = motor neuron disease inclusion body dementia
Nervous system (semeiology, syndromes)
Neurology
Neurons - chemistry
NINDS-SPSP = National Institute of Neurological Disorders and Stroke–Society for Progressive Supranuclear Palsy
OKN = optokinetic nystagmus
PNFA = progressive non-fluent aphasia
progressive supranuclear palsy
PSP = progressive supranuclear palsy
Retrospective Studies
riMLF = rostral interstitial nucleus of the medial longitudinal fascicle
SD = semantic dementia
Supranuclear Palsy, Progressive - diagnosis
Supranuclear Palsy, Progressive - metabolism
Supranuclear Palsy, Progressive - pathology
tau and α-synuclein-negative
Ubiquitin - analysis
ubiquitinated
WAIS-R = revised Weschler adult intelligence scale
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Summary:The frontotemporal lobar degenerations (FTLDs) are a group of disorders in which the clinical picture is not necessarily predictive of the underlying neuropathology. The FTLD with ubiquitin-only-immunoreactive neuronal changes (FTLD-U) subtype is pathologically characterized by ubiquitin-positive, tau and α-synuclein-negative neuronal cytoplasmic inclusions in the frontotemporal cortex and hippocampal dentate fascia. When similar pathological changes are accompanied by histological features of motor neuron disease (MND), the term FTLD-MND is used. The latter pathological changes may be found in patients with or without clinical evidence of MND. We retrospectively reviewed the clinical details of three patients with a rapidly progressive, levodopa-unresponsive bradykinetic-rigid syndrome and frontal cognitive impairment. A diagnosis of progressive supranuclear palsy (PSP) had been considered in all three cases at initial presentation. Two of the cases fulfilled clinical diagnostic criteria for PSP, which was the final clinical diagnosis during life. Pathological analysis showed typical histological appearances of FTLD-MND in two cases and of FTLD-U in one case. Semi-quantitative analysis of pathological load seemed to correlate with the clinical phenotype. FTLD-U or FTLD-MND should be considered in the differential diagnosis of progressive frontal dementia with an akinetic rigid syndrome and supranuclear gaze palsy or Steele–Richardson–Olszewski disease.
ISSN:0006-8950
1460-2156
1460-2156
DOI:10.1093/brain/awh265