Abstract
BackgroundHydrocephalus is an under-recognized presentation of progressive supranuclear palsy (PSP) and dementia with Lewy bodies (DLB).MethodsWe 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.ResultsDespite 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.ConclusionsEarly 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.Electronic supplementary materialThe online version of this article (doi:10.1186/2054-7072-1-2) contains supplementary material, which is available to authorized users.
Highlights
Hydrocephalus is an under-recognized presentation of progressive supranuclear palsy (PSP) and dementia with Lewy bodies (DLB)
We examined four patients referred to us for suspected normal pressure hydrocephalus (NPH) but whose pathology demonstrated PSP and DLB
The preliminary diagnosis of NPH in these patients was based on the interpretation of imaging features, namely the presence of hydrocephalus judged disproportionate to the extent of surrounding parenchymal atrophy
Summary
Hydrocephalus is an under-recognized presentation of progressive supranuclear palsy (PSP) and dementia with Lewy bodies (DLB). Between 1995 and 2003, from 411 patients referred as NPH to the Mayo Clinic, only 41 were tentatively endorsed as such and barely 14 experienced gait improvement after a trial of cerebrospinal fluid removal, the sine qua non for the diagnosis. The preliminary diagnosis of NPH in these patients was based on the interpretation of imaging features, namely the presence of hydrocephalus judged disproportionate to the extent of surrounding parenchymal atrophy. While a careful neurological examination suggested further NPH work up was unwarranted in three patients, cerebrospinal fluid diversion in one (case 4) supported this diagnosis by yielding subjective benefits in gait and urinary function. We suggest that PSP and DLB, as previously reported for Alzheimer disease (AD), is capable of generating a neuroimaging profile suggestive of communicating hydrocephalus, with ventriculomegaly disproportionate to the extent of parenchymal atrophy, prompting a diagnostic consideration for NPH and potentially misdirecting treatment toward cerebrospinal fluid diversion
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