We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Deconstructing normal pressure hydrocephalus: Ventriculomegaly as early sign of neurodegeneration.
- Authors
Espay, Alberto J.; Da Prat, Gustavo A.; Dwivedi, Alok K.; Rodriguez‐Porcel, 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.; Rodriguez-Porcel, Federico
- Abstract
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.
- Subjects
BRAIN diseases; HYDROCEPHALUS; DIAGNOSIS of brain diseases; NEURODEGENERATION; PATIENTS; DIAGNOSIS; GAIT disorders; MAGNETIC resonance imaging; MEDLINE; NEUROLOGICAL disorders; ONLINE information services; DISEASE progression; DISEASE complications
- Publication
Annals of Neurology, 2017, Vol 82, Issue 4, p503
- ISSN
0364-5134
- Publication type
journal article
- DOI
10.1002/ana.25046