Steele‐Richardson‐Olszewski‐Syndrome: Reduction of dopamine D2 receptor binding relates to the severity of midbrain atrophy in vivo: 123IBZM SPECT and MRI study

Patients with the clinical diagnosis of progressive supranuclear palsy (PSP) show heterogeneous neuropathological findings. In neuropathologically proven cases with numerous neurofibrillary tangles of neuropil threads, the brainstem and striatum are always affected. We compared 123I‐iodobenzamide single photon emission computed tomography (IBZM‐SPECT) for imaging of striatal dopamine D2 receptors in vivo with high‐resolution magnetic resonance imaging (MRI) in 13 patients with possible or probable PSP. Clinically, all patients exhibited similar signs including supranuclear vertical–down‐gaze palsy, axial rigidity especially involving the neck, bradykinesia, instability of balance with easy falls, and a poor response to dopaminergic drugs.

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