Cerebellar ataxia in progressive supranuclear palsy : a clinico-pathological case report

Several clinical subtypes of progressive supranuclear palsy (PSP) have been described. We present a PSP patient with predominant cerebellar ataxia (PSP-C) and a pathologically confirmed PSP diagnosis. Cerebellar ataxia was observed at disease onset in this patient of European descent. The patient developed a symmetric akinetic-rigid syndrome with supranuclear gaze palsy later in the disease. Dopamine transporter imaging was abnormal and magnetic resonance imaging showed evidence of mesencephalic atrophy and moderate cerebellar atrophy. Post-mortem examination revealed tau-positive pathology in a classical topography for PSP. The initial presentation of PSP-C can show overlapping features with late-onset cerebellar ataxia syndromes. Early falls, absence of autonomic dysfunction and presence of supranuclear gaze palsy in the first years after symptom onset can serve as clinical signs to distinguish PSP-C from late-onset spinocerebellar ataxias, idiopathic late-onset cerebellar ataxia and multiple system atrophy.