Abstract
Corticobasal syndrome (CBS) is clinically characterized by progressive asymmetric rigidity and apraxia together with symptoms suggestive of cortical involvement and basal ganglia dysfunction. The spectrum of neurodegenerative diseases that can manifest with CBS is wide. The associations of CBS with corticobasal degeneration, progressive supranuclear palsy, Alzheimer's disease, frontotemporal lobar degenerations, Creutzfeldt–Jakob disease, or diffuse Lewy body pathology have been reported. We describe the case of a 71-year-old woman with CBS. The histopathological examination of brain tissue revealed concomitant pathology corresponding to the limbic stage of Lewy-related pathology and the intermediate stage of Alzheimer's-type pathology. To date, there have been only a few cases with a similar combination of pathology manifesting with the CBS phenotype that have been described in the literature. The extent and distribution of pathological changes in these cases were somewhat different from ours, and significance for clinical manifestation was attributed to only one of these pathologies. In our case, we assume that both types of pathology contributed to the development of the disease, considering the presumed specific spread of both types of pathological processes according to Braak's staging. Our case expands the spectrum of neurodegenerative pathological processes that may manifest with the typical CBS phenotype. Also, it points out the importance of identifying specific biomarkers that would enable more accurate in vivo differential diagnosis and more accurate determination of the underlying pathological processes of these diseases.
Highlights
Corticobasal syndrome (CBS) is clinically characterized by progressive asymmetric rigidity and apraxia together with symptoms suggestive of cortical involvement and basal ganglia dysfunction, e.g., bradykinesia, dystonia, or tremor (Boeve, 2011)
We present a patient with a pathological finding corresponding to a concomitant limbic type of Lewy-related pathology (McKeith et al, 1996; Braak et al, 2003) and intermediate category of Alzheimer’s-type pathology (A1, B3, C3) (Mirra et al, 1991; Montine et al, 2012), who presented with the clinical phenotype of CBS as another example of the clinicopathological heterogeneity of the CBS/corticobasal degeneration (CBD) spectrum
The immunohistochemical examination revealed α-synuclein positivity in the following regions: midbrain, pons, oblongata, basal ganglia, amygdala, hippocampus and insular and temporal cortices; frontal and parietal cortices were negative
Summary
Corticobasal syndrome (CBS) is clinically characterized by progressive asymmetric rigidity and apraxia together with symptoms suggestive of cortical involvement (e.g., alien limb phenomena, cortical sensory loss, myoclonus, or mirror movements) and basal ganglia dysfunction, e.g., bradykinesia, dystonia, or tremor (Boeve, 2011). She was referred with a 2-year history of progressive memory loss and speech difficulties. 1- to 2-μm-thick sections of formalin-fixed paraffin-embedded tissue from specific regions of the brain were examined: frontal, temporal, parietal, occipital and motor cortices, cingular gyrus, hippocampus and parahippocampal region, basal ganglia, thalamus, midbrain at the level of substantia nigra, pons, oblongata at the level of inferior olivary nucleus, and cerebellum. The immunohistochemical examination revealed α-synuclein positivity in the following regions: midbrain, pons, oblongata, basal ganglia, amygdala, hippocampus and insular and temporal cortices; frontal and parietal cortices were negative. The immunohistochemistry showed tau-protein positive deposits in the following regions: hippocampus, amygdala and cingular gyrus, neocortical regions (temporal, occipital, frontal, parietal, and motor cortices), basal ganglia, thalamus, and brainstem (midbrain and pons) (Figure 2D). The overall pathological picture corresponded to the limbic type of Parkinson’s disease (Braak stage V) (McKeith et al, 1996; Braak et al, 2003) and the intermediate category of Alzheimer’s-type pathology (A1B3C3) (Mirra et al, 1991; Montine et al, 2012)
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