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Deconstructing normal pressure hydrocephalus: Ventriculomegaly as early sign of neurodegeneration
Author(s) -
Espay Alberto J.,
Da Prat Gustavo A.,
Dwivedi Alok K.,
RodriguezPorcel Federico,
Vaughan Jennifer E.,
Rosso Michela,
Devoto Johnna L.,
Duker Andrew P.,
Masellis Mario,
Smith Charles D.,
Mandybur George T.,
Merola Aristide,
Lang Anthony E.
Publication year - 2017
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.25046
Subject(s) - normal pressure hydrocephalus , progressive supranuclear palsy , ventriculomegaly , medicine , pathological , dementia , shunting , hydrocephalus , disease , pediatrics , dementia with lewy bodies , pathology , radiology , surgery , genetics , pregnancy , fetus , biology
Idiopathic normal pressure hydrocephalus (NPH) remains both oversuspected on clinical grounds and underconfirmed when based on immediate and sustained response to cerebrospinal fluid diversion. Poor long‐term postshunt benefits and findings of neurodegenerative pathology in most patients with adequate follow‐up suggest that hydrocephalic disorders appearing in late adulthood may often result from initially unapparent parenchymal abnormalities. We critically review the NPH literature, highlighting the near universal lack of blinding and controls, absence of specific clinical, imaging, or pathological features, and ongoing dependence for diagnostic confirmation on variable cutoffs of gait response to bedside fluid‐drainage testing. We also summarize our long‐term institutional experience, in which postshunt benefits in patients with initial diagnosis of idiopathic NPH persist in only 32% of patients at 36 months, with known revised diagnosis in over 25% (Alzheimer's disease, dementia with Lewy bodies, and progressive supranuclear palsy). We postulate that previously reported NPH cases with “dual” pathology (ie, developing a “second” disorder) more likely represent ventriculomegalic presentations of selected neurodegenerative disorders in which benefits from shunting may be short‐lived, with a consequently unfavorable risk‐benefit ratio. Ann Neurol 2017;82:503–513