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We describe the clinical and neuropathologic features of patients with Lewy body spectrum disorder (LBSD) carrying a nonsense variant, c.604C>T; p.R202X, in the glucocerebrosidase 1 (<i>GBA</i>) gene. While this <i>GBA</i> variant is causative for Gaucher’s disease, the pathogenic role of this mutation in LBSD is unclear. Detailed neuropathologic evaluation was performed for one index case and a structured literature review of other <i>GBA</i> p.R202X carriers was conducted. Through the systematic literature search, we identified three additional reported subjects carrying the same <i>GBA</i> mutation, including one Parkinson’s disease (PD) patient with early disease onset, one case with neuropathologically-verified LBSD, and one unaffected relative of a Gaucher’s disease patient. Among the affected subjects carrying the <i>GBA</i> p.R202X, all males were diagnosed with Lewy body dementia, while the two females presented as PD. The clinical penetrance of <i>GBA</i> p.R202X in LBSD patients and families argues strongly for a pathogenic role for this variant, although presenting with a striking phenotypic heterogeneity of clinical and pathological features.