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Normal pressure hydrocephalus or progressive supranuclear palsy? A clinicopathological case series

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Abstract

Idiopathic normal pressure hydrocephalus (iNPH) is a poorly understood condition, which typically presents with the triad of balance impairment, urinary incontinence and subacute cognitive decline, while brain imaging shows a marked enlargement of the cerebral ventricles. Few patients with iNPH have come to post-mortem. We identified four patients from the Queen Square Brain Bank archival collection, who had received a diagnosis of iNPH during life, and reviewed their clinical, radiological and pathological characteristics. At post mortem examination, one patient had Parkinson’s disease (PD) while the other three had progressive supranuclear palsy (PSP). All four had presented with pure akinesia with gait freezing, accompanied by unsteadiness and falls. An awareness that PSP or PD can mimic the clinical symptoms of iNPH may help to avoid invasive and futile cerebrospinal fluid shunting procedures.

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Acknowledgments

We wish to thank the donors and their families, without whom this work would not have been possible. We received funding from the Reta Lila Weston Trust.

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On behalf of all authors, the corresponding author states that there is no conflict of interest.

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All human studies must state that they have been approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down by the 1964 Declaration of Helsinki.

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Correspondence to Andrew J. Lees.

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Magdalinou, N.K., Ling, H., Smith, J.D.S. et al. Normal pressure hydrocephalus or progressive supranuclear palsy? A clinicopathological case series. J Neurol 260, 1009–1013 (2013). https://doi.org/10.1007/s00415-012-6745-6

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  • DOI: https://doi.org/10.1007/s00415-012-6745-6

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