Normal pressure hydrocephalus or progressive supranuclear palsy? A clinicopathological case series
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Normal pressure hydrocephalus or progressive supranuclear palsy? A clinicopathological case series Nadia K. Magdalinou • Helen Ling • James D. Shand Smith • Jonathan M. Schott Laurence D. Watkins • Andrew J. Lees
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Received: 11 September 2012 / Revised: 30 October 2012 / Accepted: 1 November 2012 / Published online: 23 November 2012 Ó Springer-Verlag Berlin Heidelberg 2012
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 postmortem. 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
N. K. Magdalinou H. Ling A. J. Lees (&) Reta Lila Weston Institute of Neurological Studies, UCL, 1 Wakefield Street, London WC1N 1PJ, UK e-mail: [email protected] N. K. Magdalinou e-mail: [email protected] N. K. Magdalinou H. Ling A. J. Lees Sara Koe PSP Research Centre, Institute of Neurology, University College London, London, UK N. K. Magdalinou H. Ling A. J. Lees Queen Square Brain Bank for Neurological Disorders, Institute of Neurology, University College London, London, UK J. D. S. Smith L. D. Watkins Victor Horsley Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, London, UK J. M. Schott Dementia Research Centre, Institute of Neurology, UCL, London, UK
awareness that PSP or PD can mimic the clinical symptoms of iNPH may help to avoid invasive and futile cerebrospinal fluid shunting procedures. Keywords Normal pressure hydrocephalus (NPH) Progressive supranuclear palsy (PSP) Ventriculoperitoneal (VP) shunt Pure akinesia with gait freezing (PAGF) Alzheimer’s disease (AD)
Background Normal pressure hydrocephalus (NPH) is a clinical syndrome characterised by unsteadiness of gait, urinary incontinence and cognitive decline, which can respond to cerebrospinal fluid (CSF) drainage [1]. The radiological ‘‘hallmark’’ of NPH is markedly enlarged ventricles in the absence of involutional change in the brain. NPH has been categorized as either idiopathic (iNPH) or secondary (following head trauma, intracranial haemorrhage, tumour, meningitis or complication of cranial surgery). iNPH remains a controversial entity, and of the relatively few patients coming to autopsy, a significant proportion have been noted to have co-existing neurodegenerative or vascular pathologies [2, 3]. Whether those pathologies are the primary cause of the syndrome or an additional incidental disease process in the elderly populatio
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