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* This is the Professional Version. *

Normal–Pressure Hydrocephalus

By Juebin Huang, MD, PhD

Normal-pressure hydrocephalus is characterized by gait disturbance, urinary incontinence, dementia, enlarged brain ventricles, and normal or slightly elevated CSF pressure.

Normal-pressure hydrocephalus is thought to result from a defect in CSF resorption in arachnoid granulations. This disorder accounts for up to 6% of dementias; dementia is chronic, global, usually irreversible deterioration of cognition.

Dementia should not be confused with delirium although cognition is disordered in both. The following helps distinguish them:

  • Dementia affects mainly memory, is typically caused by anatomic changes in the brain, has slower onset, and is generally irreversible.

  • Delirium affects mainly attention, is typically caused by acute illness or drug toxicity (sometimes life threatening), and is often reversible.

Other specific characteristics also help distinguish dementia and delirium (see Table: Differences Between Delirium and Dementia*).

Symptoms and Signs

The gait disturbance in normal-pressure hydrocephalus is usually nonspecific unsteadiness and impaired balance, although a magnetic gait (the feet appear to stick to the floor) is considered the characteristic gait disturbance.

Dementia may not occur until late in the disorder. The most common early symptoms of dementia are disturbances of executive function and attention; memory tends to become impaired later.

Urinary incontinence is common.

Diagnosis

  • Clinical evaluation

  • Neuroimaging

  • Sometimes removal of CSF

The classic symptoms (gait disturbance, urinary incontinence, and dementia), even combined, are nonspecific for normal-pressure hydrocephalus, particularly in the elderly. For example, some forms of vascular dementia can cause dementia, gait disturbance, and, less commonly, urinary incontinence.

A general diagnosis of dementia requires all of the following:

  • Cognitive or behavioral (neuropsychiatric) symptoms interfere with the ability to function at work or do usual daily activities.

  • These symptoms represent a decline from previous levels of functioning.

  • These symptoms are not explained by delirium or a major psychiatric disorder.

Evaluation of cognitive function involves taking a history from the patient and from someone who knows the patient plus doing a bedside mental status examination or , if bedside testing is inconclusive, formal neuropsychologic testing (see Assessment of cognitive function).

Brain imaging may show ventricular enlargement disproportionate to cortical atrophy; this finding is nonspecific but may support the diagnosis of normal-pressure hydrocephalus.

Lumbar puncture with removal of 30 to 50 mL of CSF can be done as a diagnostic trial. Improvement in gait, continence, and cognition after removal helps confirm the diagnosis, but improvement may not be evident until several hours after removal. Additional CSF may leak out after lumbar puncture, sometimes contributing to neurologic improvement.

Treatment

  • Sometimes ventriculoperitoneal shunting

Ventriculoperitoneal shunting is useful for patients with acceptable surgical risks. Improvements after lumbar puncture to remove CSF, done during diagnosis, may predict the response to shunting. In several case series (but in no randomized trials), patients improved substantially, typically in gait, continence, and daily functioning, after shunting; improvement in cognition was less common.

* This is the Professional Version. *