Gait apraxia as a presenting sign of Gerstmann‐Sträussler‐Scheinker disease

Abstract

AbstractGerstmann‐Sträussler‐Scheinker disease (GSS) is a rare inherited prion disease that shows variable clinical symptoms, including cerebellar ataxia, dementia, and leg weakness. These symptoms may result in gait disturbance which frequently occurs in the early stage of the disease; however, precise descriptions focused on its phenomenology and etiology have been scarce. Here, we report a unique case of GSS presenting with gait apraxia as the initial symptom. Moreover, SPECT showed hypoperfusion in the bilateral superior frontal gyri, which suggests that dysfunction in the lesions is largely responsible for gait apraxia.