Magnetic Resonance Imaging of Normal Pressure Hydrocephalus

Normal pressure hydrocephalus (NPH) is a syndrome found in the elderly, which is characterized by ventriculomegaly and deep white matter ischemia (DWMI) on magnetic resonance imaging (MRI) and the clinical triad of gait disturbance, dementia, and urinary incontinence. NPH has been estimated to account for up to 10% of cases of dementia and is significant because it is treatable by ventriculoperitoneal shunting. Patients with a known cause of chronic communicating hydrocephalus, that is, meningitis or hemorrhage, tend to respond better than patients with the so-called “idiopathic” form, most likely because of poor selection criteria in the past. Good response to shunting has been associated with hyperdynamic cerebrospinal fluid (CSF) flow through the aqueduct. In the early days of MRI, patients with a large CSF flow void extending from the foramen of Monro through the aqueduct to the fourth ventricle had an excellent chance of responding to ventriculoperitoneal shunting (P < 0.003). Today, we use phase-contrast MRI to measure the volume of CSF flowing through the aqueduct in either direction over a cardiac cycle. When this aqueductal CSF stroke volume is sufficiently elevated, there is an excellent chance of shunt responsiveness (100% positive predictive value in 1 study). Idiopathic NPH appears to be a “two-hit” disease—benign external hydrocephalus (BEH) in infancy followed by DWMI in late adulthood. As BEH occurs when the sutures are still open, these infants present with large heads, a finding also noted in patients with NPH. Although BEH has been attributed to immature arachnoidal granulations with decreased CSF resorptive capacity, this now appears to be permanent and may lead to a parallel pathway for CSF resorption via the extracellular space of the brain. With DWMI, the myelin lipid is lost, exposing the polar water molecules to myelin protein, increasing resistance to CSF outflow and leading to backing up of CSF and hydrocephalus.