Corticobasal degeneration presenting with progressive spasticity

Hasselblatt, Martin; Föllinger, S.; Steinbach, Peter; Schwan, A.; Paulus, W.

doi:10.1212/01.wnl.0000256729.99951.44

Cited by 8 publications

(4 citation statements)

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“…We found only 2 articles11, 12 that focused specifically on dystonia in CBD. Of the 404 selected cases, there were not sufficient data to conclude if there was dystonia in 108 (26.7%)14–30; of the remaining 296 cases, 111 (37.5%) were classified as CBD with dystonia,1, 2, 5, 10, 11, 31–67 and 185 (62.5%) were classified as CBD without dystonia1, 5, 10, 15, 18, 27, 28, 35, 36, 39, 40, 45, 47–50, 52, 55, 57, 57–59, 61, 62, 64, 66–80 (see Supplementary Tables 1 and 2, respectively, for all references). There was no significant difference between CBD with and without dystonia in age at onset (61.9 ± 16.5 vs 64.1 ± 6.7 years, respectively; P = .22), and disease duration (6.4 ± 2.7 vs 5.5 ± 2.6 years, respectively; P = .11).…”

Section: Resultsmentioning

confidence: 99%

Dystonia in corticobasal degeneration: A review of the literature on 404 pathologically proven cases

2012

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A B S T R A C T :Dystonia is considered one of the classical features of corticobasal degeneration and is reported in up to 83% in clinical, not pathologically confirmed, series. Here, we aimed to establish the frequency and the clinical characteristics of dystonia in CBD by reviewing the literature on 404 pathologically proven cases. Further, we aimed to identify the frequency and characteristics of dystonia in all described phenotypes with CBD pathology. Dystonia was present in only 37.5% of the 296 cases with adequate information. The majority of the cases with dystonia presented with a corticobasal syndrome, and dystonia occurred in the first 2 years from disease onset, affecting the upper limb. In cases with dystonia that presented with a ''dementia'' phenotype, dystonia tended to appear later in the disease course and to more affect the cervical region and the face. With regard to the distribution of the phenotypes, fifty-four percent of 374 cases presented as corticobasal syndrome, 15% as frontotemporal dementia, and 10.7% as progressive supranuclear palsy. Dystonia and myoclonus were present in about half of all cases with corticobasal syndrome, implying that these features may not be as frequent in corticobasal syndrome as are akinetic-rigid syndrome and apraxia (100% and 86.3%, respectively). Dystonia and myoclonus almost co-occurred in our analysis, suggesting a possible association. In conclusion, despite dystonia being an inclusion criterion in all sets of clinical criteria for corticobasal degeneration, this was present in only one third of the pathologically proven cases presented here. More accurate characterization of dystonia in corticobasal degeneration would be of importance for clinical diagnosis and development of treatment strategies. V C 2012 Movement Disorder Society

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Section: Resultsmentioning

confidence: 99%

Dystonia in corticobasal degeneration: A review of the literature on 404 pathologically proven cases

2012

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“…Such involvement would unavoidably lead to different types of extrapyramidal system impairment and spasticity in the late stage. 23 The combination of CBD and AD made the diagnosis in this case more complicated and difficult, although clear clinical and pathological patterns had been outlined. Both of them have in common the presence of the aberrant tau aggregates.…”

Section: Discussionmentioning

confidence: 88%

The overlap of corticobasal degeneration and Alzheimer changes: An autopsy case

et al. 2009

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The aspects of various neurodegenerative diseases can be observed overlapping with each other during autopsy. Corticobasal degeneration (CBD) is a rare neurodegenerative disease, whereas Alzheimer disease (AD) is the most common cause of dementia. In this article, we present the combination of CBD and AD in an autopsy case. The patient, an 82-year-old right-handed woman developed asymmetrical parkinsonism, visuospatial dysfunction and memory loss, as well as subsequent non-influent aphasia over the past 10 years. The autopsy revealed characteristic CBD-related pathology, ballooned neurons, globose tangles and astrocytic plaques, mainly in the frontal cortex and basal ganglia. The Alzheimer-related pathology was also present concomitantly. Senile plagues deposited diffusively throughout the hippocampus and neocortices. Neurofibrillary tangles (NFTs) were more confined to the hippocampus. The autopsy demonstrated pathological overlap of CBD and AD, which therefore explained the clinical early development of dementia and parkinsonism. We should suspect the concurrence of various neurodegenerative disorders in any case with atypical or complex clinical manifestations. Tau pathology is a prominent feature in both CBD and AD. Such a combination would be a clue for the pathogenesis of various tauopathies.

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“…Finally, Hasselblatt et al. reported a case of a progressive spastic paraparesis that turned out to be CBD at post mortem examination, but no clinicopathological correlation was proposed [15].…”

Section: Case Reportmentioning

confidence: 99%