Editors' note: Diffuse Lewy body disease manifesting as corticobasal syndrome: A rare form of Lewy body disease

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The heterogeneity of pathology in corticobasal syndrome (CBS) is known but incompletely characterized. In this retrospective autopsy series by Kasanuki et al., investigators identified 11 patients with an antemortem diagnosis of CBS with autopsy-proven findings of diffuse Lewy body disease (DLBD). Compared with the autopsy specimens of 22 patients with dementia with Lewy bodies (DLBs), those with CBS clinically, but DLBD pathologically, were younger at symptom onset, had less REM sleep behavior disorder, more axial rigidity, and pyramidal symptoms, and histopathologically exhibited more significant cerebral atrophy and thinning of the corpus callosum. However, these are not the only features that may differentiate DLBs from CBS. Infante and colleagues recall 1 patient seen at their center with atypical CBS and phosphorylated α-synuclein (p-syn) deposits in cutaneous nerves of the skin. These authors suggest that skin biopsy may be helpful in circumstances where diagnostic uncertainty exists in clinically overlapping dementia syndromes. The heterogeneity of pathology in corticobasal syndrome (CBS) is known but incompletely characterized. In this retrospective autopsy series by Kasanuki et al., investigators identified 11 patients with an antemortem diagnosis of CBS with autopsy-proven findings of diffuse Lewy body disease (DLBD). Compared with the autopsy specimens of 22 patients with dementia with Lewy bodies (DLBs), those with CBS clinically, but DLBD pathologically, were younger at symptom onset, had less REM sleep behavior disorder, more axial rigidity, and pyramidal symptoms, and histopathologically exhibited more significant cerebral atrophy and thinning of the corpus callosum. However, these are not the only features that may differentiate DLBs from CBS. Infante and colleagues recall 1 patient seen at their center with atypical CBS and phosphorylated α-synuclein (p-syn) deposits in cutaneous nerves of the skin. These authors suggest that skin biopsy may be helpful in circumstances where diagnostic uncertainty exists in clinically overlapping dementia syndromes.