Reader response: Diffuse Lewy body disease manifesting as corticobasal syndrome: A rare form of Lewy body disease

Abstract

We read with great interest the article by Kasanuki et al.1 that described 11 cases of pathologically confirmed diffuse Lewy body disease, antemortem clinically manifesting as corticobasal syndrome (CBS), in line with previous findings. We recently evaluated a 70-year-old woman referred to our center with possible CBS according to clinical criteria.2 The patient presented with a 3-year history of limb bradykinesia and dystonia prevalent in the right side associated with limb apraxia. Brain MRI detected bilateral parietal cortical atrophy, mainly in the left side, and 18F-fluorodeoxyglucose PET showed left parietal hypometabolism. Additional features suggested an “atypical” atypical parkinsonism3: a slow parkinsonism progression, moderate improvement of bradykinesia with levodopa therapy, and a 10-year history of memory and language impairment associated with mild dysphagia and postural instability. The patient underwent skin biopsy, unveiling abnormal deposition of phosphorylated α-synuclein (p-syn) in skin nerves, unexpected in CBS.