Clinicoradiological Features in Progressive Supranuclear Palsy Comorbid with Argyrophilic Grains.

Background: Contrary to pure cases, the influence of comorbid argyrophilic grain disease (AGD) in progressive supranuclear palsy (PSP) has not been sufficiently evaluated.

Objectives: We compared the clinicoradiological features of 12 patients with PSP with (PSPw/AG) and 8 patients without AGD (PSPw/oAG).

Methods: Medical records and magnetic resonance imaging were checked retrospectively from a single brain bank database.

Results: Other than AGD, no differences were observed in any other neurodegenerative pathologies between the 2 groups. Ages at onset and deaths of patients with PSPw/AG were higher than those of patients with PSPw/oAG (77.9 ± 4.9 vs. 68.9 ± 5.9, and 87.0 ± 5.7 vs. 78.1 ± 5.0;  = 0.003 and  = 0.002, respectively). In addition to the later onset of motor symptoms, initial amnestic presentations were limited to 5 patients with PSPw/AG. Both characteristic midbrain atrophy and severe ambient gyrus atrophy were detected exclusively in 8 patients with PSPw/AG.

Conclusions: Initial amnestic presentations and ambient gyrus atrophy may be characteristic of PSPw/AG.