The role of the neurologist in the longitudinal management of normal pressure hydrocephalus.

Background: Since normal pressure hydrocephalus (NPH) was first described in 1965, neurologists have been involved in the initial diagnostic evaluation for it but have rarely followed patients specifically to monitor response to therapy after shunt surgery.

Review Summary: The potential role for the neurologist in the longitudinal management of NPH has broadened, partly because of improvement in the tools used to diagnose and treat NPH and partly because of progress in understanding how NPH mimics and interacts with other common disorders of the elderly. The interplay of Alzheimer dementia, vascular dementia, Parkinson disease, and spinal stenosis with NPH presents the clinician with a patient profile that may be challenging to assess. Neurologists also face a broad differential spectrum of less common neurologic diseases that may present with similar signs (including ventriculomegaly) and symptoms; these diseases include frontotemporal dementia, progressive supranuclear palsy, Lewy body disease, corticobasal degeneration, Huntington disease, spongiform encephalopathy, and multiple-system atrophy.

Conclusions: The neurologist is in a unique position to differentiate NPH from alternative diagnoses, to suggest management strategies for patients with concomitant NPH and another neurologic disorder, and to participate in longitudinal management of NPH.