Background: Hydrocephalus is an under-recognized presentation of progressive supranuclear palsy (PSP) and dementia with Lewy bodies (DLB).
Methods: We describe four normal pressure hydrocephalus (NPH)-like presentations of pathology-proven PSP (n = 3) and DLB (n = 1) and review the literature on the hydrocephalic presentation of these atypical parkinsonisms.
Results: Despite the presence of ventriculomegaly disproportionate to the extent of parenchymal atrophy, all patients demonstrated early postural impairment and/or oculomotor abnormalities that encouraged a diagnostic revision. Hallucinations were the only early atypical manifestation of the hydrocephalic DLB presentation.
Conclusions: Early postural impairment, falls, oculomotor impairment, and/or hallucinations are inconsistent with the diagnosis of NPH and suggest PSP or DLB as the underlying NPH mimic. We postulate that previously reported cases of "dual" pathology (e.g., NPH and PSP) actually represent the hydrocephalic presentation of selected neurodegenerative disorders.
Keywords: Dementia with Lewy bodies; Normal pressure hydrocephalus; Progressive supranuclear palsy; Ventriculomegaly.