The Clinical Overlap between the Corticobasal Degeneration Syndrome and Other Diseases of the Frontotemporal Spectrum: Three Case Reports

Abstract

The corticobasal degeneration syndrome has been suggested to be part of a complex of conditions (including the different subtypes of frontotemporal dementia and progressive supranuclear palsy), which reflect a spectrum of pathological substrates. This concept is supported by the frequent clinical overlap that can be observed among patients diagnosed with these conditions. We report three clinical cases, characterized by the overlap of the clinical features of corticobasal degeneration syndrome with, respectively, nonfluent progressive aphasia, progressive supranuclear palsy and semantic dementia. Current diagnostic criteria emphasize differences in clinical presentation, which probably reflect the preferential location of pathology in the early stages of disease. However, with disease progression, a considerable clinical overlap can be expected among the different syndromes. This concept should be extended not only to the cognitive and behavioural features of the frontotemporal dementia subtypes, but also to the movement disorders of corticobasal degeneration and supranuclear palsy.

Semantic dementia associated with corticobasal syndrome: a further variant of frontotemporal lobe degeneration?

“…Raggi et al [8] reported the case of a 56-year-old woman who presented with behavioural disorder associated with fluent aphasia and mildly asymmetric (left [ right) melokinetic and ideomotor apraxia. The patient did not show the typical motor features of CBD and the authors could not exclude an atypical presentation of Alzheimer's disease [8].The patient presented here is worthy of note because her case shows, for the first time, the presence of a highly selective semantic breakdown, consistent with semantic dementia, combined with the neurological signs of CBS. The patient shows none of the features of nonfluent primary progressive aphasia that have hitherto been linked to CBS.…”

“…Formal exploration of semantic memory impairment and visual agnosia was not undertaken. Raggi et al [8] reported the case of a 56-year-old woman who presented with behavioural disorder associated with fluent aphasia and mildly asymmetric (left [ right) melokinetic and ideomotor apraxia. The patient did not show the typical motor features of CBD and the authors could not exclude an atypical presentation of Alzheimer's disease [8].…”

“…To our knowledge, only one report [8] has referred to an association between semantic dementia and CBS. It is likely that the co-occurrence of semantic impairment and ideomotor apraxia reported here represents a rare but distinct clinical variant of frontotemporal lobar degeneration.…”

Neurogenic stuttering in corticobasal ganglionic degeneration: A case report

“…Based on this assessment, our patient appeared to fit in the motor-onset category of CBGD with her aphasia developing later in the course of her illness. Other researchers have suggested that non-fluent progressive aphasia and CBGD can appear in the same patient at different stages of disease progression related to the anatomical areas affected by the disease (Raggi et al, 2007). It has also been reported that there is clinical and pathological overlap between frontotemporal dementia and CBGD and between PSP and CBGD (Raggi et al, 2007) with the suggestion that PSP and CBGD may represent different phenotypes of the same disorder with genetic differences influencing disease progression.…”

Atypical association of semantic dementia, corticobasal syndrome, and 4R tauopathy

“…Infrequent cases of clinical MND are briefly mentioned (Hodges & Patterson, 2007;Ostberg & Bogdanovic, 2011). Some rare cases of concomitant SD and CBS/CBD have also been reported, though lacking neuropathological confirmation or detailed clinical description (Ikeda et al, 1996;Luzzi et al, 2012;McMonagle, Blair, & Kertesz, 2006;Raggi et al, 2007). One case of SD and neuropathological CBD, but without motor symptoms, has been published (Mathuranath, Xuereb, Bak, & Hodges, 2000).…”