Progressive Unspecified Motor Speech Disorder: A Longitudinal Single Case Study of an Older Subject

In a few cases, neurodegenerative diseases debut with a speech disorder whose differential diagnosis can be difficult.

We describe the case of a right-handed woman with a progressive speech impairment, which debuted when she was 80 years old. We report the results of neurological, neuropsychological, and imaging assessments with positron emission tomography (PET) over a period of nine years. Metabolic PET with 18F-FDG was performed at the age of 81 and repeated two years later due to the worsening of symptoms; amyloid PET with 18F-flutemetamol was performed at the age of 86. All PET results were quantitatively analyzed. A speech impairment remained the isolated neurological symptom for a long time, together with a mood disorder. Early FDG-PET showed hypometabolism in the left superior and inferior frontal areas, in the left superior temporal area, and in the right superior frontal area. Two years later, the hypometabolic area was more extensive. Amyloid PET was qualitatively and quantitatively normal. Nine years after the first symptoms, the speech production progressively worsened until complete anarthria, in association with writing impairment onset and signs of behavioral impairments. No signs of motor involvement were found.

A progressive articulatory disorder without an evolution of motor disorders may be a distinct neurological degenerative entity, mainly affecting speech production for very a long time and with a specific early metabolic pattern in brain FDG-PET in the language production area. Monitoring patients with FDG-PET could predict the disease evolution years before a clinical deterioration.