Primary progressive apraxia: an unusual ideomotor syndrome

Abstract

Objective: We describe and demonstrate by video 2 cases of primary ideomotor progressive apraxia with progressive difficulty with hand dexterity and manipulation and mild parkinsonism in the absence of significant radiologic findings. Background: Neurodegenerative disorders usually present with cognitive and physical findings, with localizing imaging findings. Our patients have physical exams showing hand apraxia with no imaging correlates. Methods: Case report Results: Patient 1, 72-year-old man. The patient could not show 2 fingers on the left hand, tap fingers or mimic hand postures. There was preserved knowledge of how to use tools. He was unable to pantomime movements, write letteremarkable in our two cases for a lack of any discernable patternrs on a page or copy figures. There was vertical gaze apraxia and inability to smile on command. Spontaneous smile was intact. MRI of the brain showed moderate cerebral atrophy, chronic right frontal cortical infarcts and white matter ischemic changes. FGD PET showed nonfocal decreased metabolism in the frontal lobes, basal ganglia and brainstem. Patient 2, 72-year-old woman. Her speech, language, prosody, and comprehension were intact. Voice was slightly hypophonic. There was minimal left wrist rigidity. Her hands were severely impaired. She was unable to pantomime, had body utilization, was unable to show an "OK" or a salute. She was unable to do rapid alternating movements with hands. MRI and FDG PET scan were normal for age. Conclusions: We report two unusual presentations of what are most likely neurodegenerative disorders, possibly tauopathies. In both cases, there is preserved cognition, language skills, muscle power and tone, and gait in the setting of profound progressive hand apraxia. Imaging is , specifically a lack of parietal lobe involvement. Both cases demonstrated a lack of response to Sinemet.