Progressive Rolandic Apraxia

We provide a comprehensive account of the distinctive clinical history and neuroimaging characteristics of a patient seen in a tertiary referral center, experiencing progressive dysfunction of the somato-cognitive action network and degeneration of the Rolandic association cortex. The patient is a 51-year-old woman who presented with progressive left arm rigidity, apraxia, spastic dysarthria, and dyspnea while speaking and eating. The patient underwent neurologic examination and neuroimaging in the form of a brain MRI and FDG-PET CT, as well as a comprehensive pulmonary evaluation. Her pulmonary evaluation, including chest x-ray, pulmonary function testing, and chest CT were unremarkable. Her brain FDG-PET revealed hypometabolism of the right hemispheric Rolandic motor association cortex. Our patient, with unique clinical and imaging features, is the first reported case of inter-effector region network dysfunction due to a likely neurodegenerative condition impacting Rolandic motor association cortex. We propose to refer to this clinical entity as “Progressive Rolandic Apraxia”.