Clin Geriatr Med 22 (2006) 645–657

Normal Pressure Hydrocephalus:

Diagnosis and New Approaches
to Treatment
Ronan Factora, MDa,*, Mark Luciano, MD, PhDb
a
Section of Geriatric Medicine, Department of General Internal Medicine,
Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA
b
Department of Neurosurgery, Cleveland Clinic Foundation, 9500 Euclid Ave,
Cleveland, OH 44195, USA

Impairment of gait occurs commonly in the elderly and has a profound

impact on individual functional capacity and quality of life. Dementia
and urinary incontinence are other common problems encountered in the el-
derly. In many circumstances, these problems are mutually exclusive, with
treatments chosen to address each disease entity separately. When they ap-
pear over time in the same patient, however, these symptoms raise the pos-
sibility of normal pressure hydrocephalus (NPH).
NPH first was described in 1965 in a series of papers by the Columbian
neurosurgeon, Dr. Solomon Hakim. Subsequently, estimates of the preva-
lence of NPH have varied and the role of ventricular shunting debated.
The diagnosis of NPH in elderly patients is suspected on the basis of
enlarged cerebral ventricles with one or more of the Adam’s triad of
symptoms: gait imbalance, dementia, and urinary incontinence [1]. The in-
cidence of NPH remains uncertain, as diagnostic features and prevalence
rates vary widely across reporting centers. Rates vary from 1.3 per million
to 4 per 1000, depending on diagnostic criteria for NPH and populations
sampled. Recent estimates quote an annual incidence of 1.8 per 100,000 in-
habitants using a survey of 49 centers in Europe [2]. NPH usually occurs in
the sixth to seventh decades of life [1,3].
Because treatment for NPH differs from that for other gait disorders and
success of treatment may be dramatic, it is worthwhile to identify appropri-
ate patients for cerebrospinal shunting. The challenge in the elderly

* Corresponding author. Cleveland Clinic Foundation, Section of Geriatric Medicine,

Department of General Internal Medicine, Desk A91, 9500 Euclid Avenue, Cleveland, OH 44195.
E-mail address: factorr@ccf.org (R. Factora).

0749-0690/06/$ - see front matter Ó 2006 Elsevier Inc. All rights reserved.
doi:10.1016/j.cger.2006.05.001 geriatric.theclinics.com
646 FACTORA & LUCIANO

population is sorting through changes that result from normal aging and
those comorbid illnesses commonly encountered when making this
determination.
The diagnosis and treatment of NPH is facilitated by communication and
collaboration between geriatricians and neurosurgeons. At the authors’ insti-
tution, this coordination takes the form of an interdisciplinary clinic, which
attempts to differentiate multiple causes of cognitive impairment and gait de-
cline; to optimize medical management of these causes; to screen for patients
who potentially may benefit from cerebrospinal fluid (CSF) shunting; and to
follow, evaluate, and optimize NPH patients’ course post shunting.

Pathophysiology
The exact mechanism of the ventriculomegaly in NPH remains unclear,
but various hypotheses exist. One proposes that in adult hydrocephalus,
ventriculomegaly develops as a result of a combination of a slightly elevated
baseline CSF pressure and intermittent increased CSF pressure waves [4,5].
Another theory states that increases in subependymal CSF accumulation
along with stretching of the periventricular white matter leads to changes
in subcortical white matter; subsequently, ventriculomegaly results from
diminished elasticity of brain [6]. Development of a transmantle gradient
(between the ventricles and the cortical subarachnoid space) resulting
from outflow obstruction at the basilar cisterns also are believed to lead
to continuous ventriculomegaly or enlargement [7], although the existence
of these gradients are not well established.
Development of ventriculomegaly likely produces clinical symptoms
through compression of adjacent brain tissues and decreased cerebral blood
flow [8]. The gait apraxia is multifactorial in nature, attributed to motor def-
icits, impaired postural righting reflexes, impaired smooth pursuit, and im-
paired suppression of vestibuloocular reflexes. The ‘‘magnetic gait’’ refers to
the characteristic wide-based stance, short small steps, and reduced floor
clearance in patients who have NPH [9,10]. Gait impairment typically is
the first symptom noted, which is attributed to lateral ventricle compression
of the fibers of the corticospinal tracts that supply the legs along the corona
radiata [9,11]. Similarly, compression of sacral fibers along the corona radi-
ata may be responsible for impairment of inhibitory fibers supplying the
bladder, leading to subsequent urinary urgency and incontinence [9].

Diagnosis and diagnostic challenges

Identification of symptoms in Adam’s triad is simple, but determining
which patients truly have NPH and will benefit from shunting not always
is straightforward. Although patients need not have all three symptoms
for NPH to be considered, the gait deficit is considered the most crucial
for diagnosis. Difficulty in turning occurs because patients feel unsteady
 NORMAL PRESSURE HYDROCEPHALUS 647

and make the transition in very small broad-based, multiple steps. The shuf-
fling aspect of this gait deficit has some similarities to and often is confused
with Parkinson’s disease. Unlike Parkinson’s disease, however, patients who
have NPH do not have tremor. Reflex findings are variable and not consis-
tently diagnostic, and sensory examination usually is intact.
If patients have cognitive deficits without gait involvement, the diagnosis
of NPH is highly unlikely. In one study, presence of the complete Adam’s
triad in patients who had a clinical suspicion of NPH had a positive predic-
tive value of 64% and negative predictive value of 82% [12]. As the devel-
opment of symptoms in NPH is a continuum, not all of the symptoms
may be severe enough to be noticed initially. As time passes, the probability
that all of Adam’s triad is present increases.
Timing in the onset of these symptoms can be helpful in determining
whether or not the presence of these symptoms truly represents NPH or is
explained by other diagnoses. Typically, gait impairment is the first symp-
tom, with cognitive symptoms (eg, executive dysfunction) occurring later.
Typically, urinary frequency with urgency is noted first, before the develop-
ment of incontinence, as a result of dementia or reduced ability to get to the
bathroom in time.

Differential diagnosis
Evaluation of gait should take into consideration potential causes aside
from NPH. History significant for back pain or lower extremity weakness
and radicular pain can lead to a diagnosis of lumbar canal stenosis. MRI
of the lumbosacral spine aids in diagnosis. A steppage gait suggests the
presence of peripheral neuropathy and is distinguishable from the wide
base and diminished step gait associated with NPH. Observed shuffling
gait along with tremor or bradykinesia may lead to an incorrect diagnosis
of parkinsonism. Appearance of the gait may resemble shuffling: resting
‘‘pill-rolling’’ tremor of the hands, bradykinesia, rigidity, and freezing,
all characteristic of parkinsonism, help differentiate it from NPH. Features
of Parkinson-plus syndromes, such as gaze palsy or autonomic dysfunc-
tion (dysphagia, arrhythmias, orthostatic hypotension, or urinary reten-
tion), in the presence of a shuffling gait reduce the clinical suspicion of
NPH. Parkinson-like features may be present in late NPH and typically
do not respond to levodopa.
The possibility of the coexistence of multiple causes for gait impairment is
higher in the elderly population. Consequently, identification of these etiol-
ogies should be coupled with the determination of which cause is the greater
contributing factor to gait impairment. For example, patients who have fea-
tures of NPH also can have significant lumbar canal stenosis impeding gait.
Lumbar canal stenosis can interfere with a trial of CSF drainage used for
predictive purposes in NPH. It also may limit rehabilitative potential if
shunt placement proceeds. In this case, it may be reasonable to pursue
648 FACTORA & LUCIANO

treatment of lumbar canal stenosis rather than moving directly to shunt

placement.
The presenting symptoms of cognitive impairment can be similar in NPH
and other dementias in elderly patients. Historical clues and physical exam-
ination findings are the most useful factors in distinguishing between them.
Alzheimer’s disease typically presents insidiously over years, with progres-
sive memory impairment and development of additional features, including
anomia, apraxia, agnosia, visuospatial dysfunction, and executive dysfunc-
tion. Vascular dementia classically presents as a stepwise decline over
time, with more prominent loss of higher-order cognitive functions, includ-
ing executive function and visuospatial perception. Patients who have vascu-
lar dementia also may have emotional lability, may develop sundowning,
and may have focal neurologic deficits on examination.
Dementia in NPH typically has subcortical features, leading to slowness
of movement and function, forgetfulness (without complete memory loss),
and impaired executive function. Absence of cortical features, such anomia,
apraxia, and frank memory loss, differentiate it from Alzheimer’s disease.
There is greater difficulty separating the features of cognitive impairment as-
sociated with NPH from other comorbidities associated with subcortical def-
icits (vascular dementia, depression, and frontal lobe dementias). Table 1
helps illustrate the differences between cognitive impairment encountered in
NPH and the classical presentations of Alzheimer’s disease and vascular
dementia.
When evaluating dementia, identifying specific cognitive deficits can be
helpful in correct diagnosis. Using the brief Mini–Mental State Examination
(MMSE) aids in diagnosing dementia, although MMSE has its limitations
in assessing severity of subcortical deficits (which may be identified more

Table 1
Comparison of dementia characteristics
Normal pressure
Alzheimer’s disease Vascular dementia hydrocephalus
Memory impairment X X Impaired retrieval
Executive X X X
dysfunction
Impaired X Xa
visuospatial
process
Impaired language X Xa Bradyphrenia
Impaired complex X Xa
motor skills
Psychomotor X
slowing
Impaired X
attentiveness
a
Can occur based on location of infarction.
 NORMAL PRESSURE HYDROCEPHALUS 649

easily using the Clock Drawing Test or Trail-Making Test). More advanced
neuropsychiatric testing can be useful to aid in appropriate diagnosis when
evaluating patients whose pattern of cognitive deficits is unclear. It also can
be useful in measuring response to interventions or progression over time.
Depression also can present with memory loss or executive dysfunction
and can be mistaken for dementia. Cognitive deficits are similar to those
found in subcortical dementias. The 15-question Geriatric Depression
Scale may be able to identify depressed individuals to initiate proper
therapy.
In the elderly population, urinary incontinence is common. Identifiable
causes may include bladder outlet obstruction resulting from benign prostatic
hypertrophy, retention resulting from neurogenic bladder (from long-
standing diabetes or related to Parkinson’s disease), and pelvic floor insuf-
ficiency contributing to stress incontinence. Identifying the specific type of
incontinence (eg, urge, stress, overflow, or functional) is useful in deter-
mining the underlying cause. Judicious use of cystoscopy and urodynamic
testing can help in evaluation of patients whose diagnosis is unclear.

Diagnostic evaluation
Neuroimaging in the form of CT or MRI is needed to confirm the pres-
ence of enlarged ventricles. MRI may be more useful than CT in identifying
other CNS disorders and providing greater detail, especially regarding the
presence of cerebrovascular disease. Presence of temporal lobe or hippocam-
pal atrophy also may support a diagnosis of Alzheimer’s disease. By gating
the MRI to the cardiac cycle and CSF oscillatory flow (MRI cine CSF flow),
movement of the CSF can be monitored to identify blockages, especially in
the aqueduct between the thirdrd and fourth ventricles. Detection of ob-
struction as a cause of chronic hydrocephalus obviates CSF drainage tests,
and surgical intervention is considered. Hyperdynamic flow demonstrated in
the aqueduct supports a diagnosis of NPH.
Although ventricular enlargement may seem straightforward, the criteria
of pathologic enlargement in the aging population is not established. Be-
cause some degree of atrophy is ubiquitous in this age group and may
even be exacerbated by known vascular disease, a common problem is dis-
tinguishing true hydrocephalus (a problem in CSF circulation) from ventri-
culomegaly ex vacuo (a parenchymal disease). Increases in fluid spaces in the
hemispheric sulci and subarachnoid spaces proportional to ventricular ex-
pansion may be more likely to be present in ex vacuo hydrocephalus. Ven-
tricular enlargement with gyral effacement against the skull is more
suggestive of acute hydrocephalus.
Measurement of the Evans ratio is a crude unidimensional measurement
of ventricular width, which is useful because it can be performed easily on
available CT or MRI scans. This value is defined as the ratio of the maxi-
mum width of the anterior ventricular horns at the level of the foramen
650 FACTORA & LUCIANO

of Monro to the maximum width of the calvarium at the same level (Fig. 1).
A ratio greater than or equal to 0.3 defines ventriculomegaly, but most pa-
tients who have NPH have a ratio greater than or equal to 0.4 [13]. Al-
though Evan’s ratio is a handy rule of thumb for identifying the existence
of ventricular enlargement, it does not identify the cause of the enlargement
and, therefore, cannot define hydrocephalus without other criteria or differ-
entiate ventriculomegaly resulting from NPH or atrophy.
Newer technologies (positron emission tomography, single photon
emission CT, and functional MRI) provide information regarding blood
flow, oxygen delivery, and metabolism but have not added accuracy to di-
agnosis or determining responsiveness to shunting. These techniques,
along with xenon CT blood flow studies (with use of acetazolamide)
and MRI have been useful in experimental settings to study the pathology
of NPH.
Without validated and definitive clinical criteria for the diagnosis of
treatable cases of NPH, the authors’ group has created a classification
scheme based on clinical experience to aid in determining candidacy for
shunting in patients who have suspected NPH (Table 2). Patients are divided
into four quadrants based on a combination of clinical symptoms (with an
emphasis on gait impairment) and neuroimaging findings.
In an ideal situation, patients who have NPH present with the symptoms
of Adam’s triad, imaging consistent with true hydrocephalus without signif-
icant atrophy and without confounding comorbidities, such as parkinson-
ism, and are considered to have features of NPH. Many neurosurgeons

Fig. 1. Evans ratiodthe ratio of the maximum width of the anterior horns of the lateral ven-
tricles to the maximum width of the calvarium at the same level of the foramen of Monro.
 NORMAL PRESSURE HYDROCEPHALUS 651

Table 2
Categorization of patients for shunting, by quadrants, based on results of clinical examination
and neuroimaging
Ventriculomegaly present Ventriculomegaly absent
Symptoms NPH symptoms I Proceed with drainage II Consider drainage trial;
present trial; probable shunting possible shunting
NPH symptoms III Further evaluation needed; IV Not Candidate for shunt
absent NPH very unlikely

have considered this ‘‘ideal’’ patient (quadrant I) as a candidate for shunting

without further testing. Given the unclear long-term benefits of shunting in
these patients, the authors proceed with further diagnostic evaluation to in-
clude a trial of CSF drainage (described later) via a lumbar puncture (LP) or
extended lumbar catheter drainage, to quantify clinical response before
a permanent shunt is placed. In this way, nonresponders may be spared
the surgical risk. In the absence of a true gold standard for the diagnosis
of NPH, a substantial response to CSF drainage becomes the criterion stan-
dard we use to define NPH response to a shunt procedure.
In many situations, however, patients are not ideal candidates for shunt-
ing. Patients may have only one element of Adam’s triad, for example, with-
out significant gait impairment, raising doubt over the diagnosis of NPH. In
other patients, the diagnosis of NPH is left uncertain, as when cognitive def-
icits overshadow or precede gait deficits, symptoms are atypical, or comor-
bidities cloud the diagnosis or limit long-term benefits of shunting. If these
patients have imaging consistent with hydrocephalus (quadrant II), how-
ever, they may be considered for further testing for NPH, such as a trial
CSF drainage. In the authors’ experience, some of these patients demon-
strate significant improvements after drainage despite atypical presentation
or confounding comorbidities. Patients who do not have radiologic evidence
of ventriculomegaly (whether or not they have symptoms suggestive of NPH
[quadrant III] or atypical presentations [quadrant IV]) should not be consid-
ered to have NPH. The authors do not routinely recommend a trial of CSF
drainage or a shunt for patients in quadrant III or IV.

Ancillary testing
Much research has been conducted using various tests to increase the di-
agnostic accuracy of NPH and to determine whether or not placement of
a shunt likely is effective in patients who have suspected NPH. Cisternogra-
phy involves imaging of the ventricular system using a radiolabeled isotope,
which is introduced via LP and allowed to distribute within the ventricular
and subarachnoid system (over 24–48 hours). It helps to determine the pres-
ence of ventricular reflux and to detect poor absorption of CSF (especially in
cases of obstructions in the ventricle or cisternal spaces) but has been
652 FACTORA & LUCIANO

relatively insensitive in detecting NPH. Cisternography has not been useful

in predicting response to shunting in NPH.
The CSF infusion test is used to measure impedance of flow of CSF ab-
sorption pathways via infusion of artificial CSF to the subarachnoid space.
Calculation of the outflow resistance helps to determine a patient’s response
to shunt placement. A calculated value greater than 18 units indicates
a problem with absorption and is useful in diagnosing hydrocephalus. De-
spite its value, significant technical expertise is needed to perform the test,
thereby limiting its application in clinical practice.
The CSF ‘‘tap-test’’ involves removal of 40 to 50 mL of CSF, with assess-
ment of gait and cognitive abilities before and after drainage to measure
symptom improvement. Performance of this procedure can be done in an
outpatient setting. Although noted improvements may be helpful in diag-
nosing NPH, many patients who have NPH may be missed with this single
test. Additionally, evidence is limited for its usefulness in predicting shunt
responsiveness.
Performance of multiple LPs may increase sensitivity, but there is signif-
icant discomfort for patients. Continuous lumbar drainage involves removal
of up to 720 mL of CSF over 72 hours using an indwelling percutaneous
catheter. As with previously noted studies, the definition of improvement
in this test also varies. Additionally, there is an increased risk of complica-
tions (eg, headache) associated with prolonged catheter drainage. In clinical
studies, removal of large volumes of CSF (10 mL/h for 3 days) has been use-
ful in aiding in the diagnosis of NPH and in determining if shunting is likely
to improve the symptoms of NPH (gait in particular). Typically, compari-
sons of gait and cognitive function are made before and after the external
lumbar drainage to provide objective evidence of a clinically significant re-
sponse. Sensitivity and specificity of this test in detecting significant changes
range from 50% to 100% and 60% to 100%, respectively [14]. Potential se-
lection bias, however, limits the predictive value of this procedure in com-
munity practice, as the true prevalence rate of NPH is unknown. In the
authors’ experience, external lumbar drainage when performed in patients
in quadrant I (described previously) is the test most highly predictive of
a good outcome after a shunt.
Continuous intracranial pressure monitoring is used to reveal the presence
of increased pressure spikes (B waves) during sleep that are considered pa-
thognomonic for NPH. Because of its invasive nature and controversies in
interpretation of findings, it is of limited application in clinical practice.
The great number of techniques studied and used by neurosurgeons re-
flects the lack of diagnostic accuracy or predictive value of an individual
test. There are wide ranges in sensitivity and specificity of these tests
(Table 3), denoting the underlying difficulty in obtaining large cohorts for
study. Additionally, without knowledge of the true prevalence of NPH
and a gold standard comparison for accurate diagnosis, these estimates
may be limited in their clinical usefulness by selection bias.
 NORMAL PRESSURE HYDROCEPHALUS 653

Table 3
Characteristics of ancillary studies used in diagnosis of normal pressure hydrocephalus and de-
termination of benefit for surgical intervention
Cerebrospinal fluid Cerebrospinal External lumbar
Cisternography outflow resistance fluid tap test drainage
Sensitivity n/a 57%–100% 26%–62% 50%–100%
Specificity n/a 44%–92% 33%–100% 60%–100%
Positive n/a 75%–92% 73%–100% 80%–100%
predictive
value
Negative n/a 27%–92% 23%–42% 36%–100%
predictive
value
Abbreviation: n/a, not available.
Data from Marmarou A BM, Klinge P, Relkin N, et al. The value of supplemental prognos-
tic tests for the preoperative assessment of idiopathic normal-pressure hydrocephalus. Neurol-
ogy 2005;57(Suppl 2):S2-17–28.

Treatment
Pharmacotherapy has not been successful in the treatment of hydroceph-
alus or for NPH. Acetazolamide, a carbonic anhydrase inhibitor and di-
uretic, reduces the production of CSF by 30% to 50% and sometimes is
used to palliate cases of mild hydrocephalus but usually cannot be used
for definitive treatment [15–20].
For the past 4 decades, CSF shunting through an implanted catheter and
valve system has been the mainstay in treatment in most forms of hydro-
cephalus, including NPH. As with other forms of hydrocephalus, CSF usu-
ally is drained from the ventricle into the peritoneal cavity; spinal catheters
and shunt into the right atrium via the facial or subclavian vein also are pos-
sible. Although shunting has remained the same in principle for the past
4 decades, there have been improvements in implantation method and
systems. Neuroendoscopic third ventriculostomy, a surgical treatment mak-
ing a fenestration directly between the third ventricle and the subarchnoid
space without any implanted hardware, has been used in limited cases.
Although third ventriculostomy success in cases of obstructive NPH is
more established, its use for the more common communicating NPH re-
mains uncertain.
The first shunts in the treatment of hydrocephalus drained CSF into the
venous system or right atrium (ventriculoatrial shunting). Later, drainage
into the peritoneal cavity became the favored method (ventriculoperitoneal
shunting), primarily because of fears of complications from vascular cathe-
ter access. Shunting CSF from the lumbar spine to the peritoneal cavity also
is described for NPH, although it is used less frequently because of perceived
lower reliability. Fig. 2 helps illustrate the effects of shunting on NPH.
Shunt surgery usually takes approximately 30 minutes and involves one
overnight stay in the hospital. One or two scalp incisions and a small
654 FACTORA & LUCIANO

Fig. 2. (A) Brain of a patient who had NHP before shunt placement; note the large collections
of CSF in the sulcal spaces. (B) Brain with NPH in same patient after shunt placement (right
lateral ventricle)dsulcal collections of fluid are reduced significantly after shunting.

abdominal incision are required. With modern laparoscopic placement, the

abdominal incision is 3 to 5 mm and chances of adhesions and infection are
reduced greatly.
The shunt system includes a one-way resistance valve, which is implanted
under the scalp. These valves have undergone many changes and many
kinds are available. The simplest first-generation valves offered a fixed resis-
tance to CSF flow to avoid overdrainage. Later, generations of valves of-
fered features to prevent uneven surges of drainage with changes in
position and activities. Most recently, valves that can be adjusted after im-
plantation have been developed. These systems allow optimization of drain-
age in each person without further surgically invasive procedures. In this
way, the amount of CSF drainage can be titrated, much in the same way
a dose of medication may be adjusted for maximum benefit.
The special case of NPH symptoms caused by chronic obstruction is
worth discussion, because its diagnosis is different and these patients may
be treated surgically but without a shunt. Patients who have long-standing
aqueductal stenosis, where there is a blockage in CSF flow between the third
and fourth ventricle, may present in mid or late adulthood with NPH symp-
toms. A LP is not useful or advisable in the diagnostic workup, and if symp-
toms are consistent with chronic hydrocephalus, they should be treated with
a procedure called endoscopic third ventriculostomy (ETV). In this proce-
dure, a neuroendoscope is introduced into the lateral ventricle via a right
frontal burr hole and extended down to the floor of the third ventricle,
 NORMAL PRESSURE HYDROCEPHALUS 655

where a hole is made allowing fluid escape [21]. No implanted device is

needed, although ETV fenestrations may fail.

Outcomes: benefits and risks

Any surgery in the elderly population has risks. Early studies have quoted
risks as high as 30% to 40% in shunting, with severe morbidity or death in
6% to 8% [22]. These risks justify preoperative testing to ensure optimal pa-
tient selection. Although there is no strict age cutoff after which patients are
considered too old for the procedure, contraindications are based on comor-
bidity, such has cardiac disease, uncontrolled hypertension, diabetes, or any
other form of general systemic disease or progressive disease that limits life
expectancy. Surgical risks are reduced by the short length of the surgery and
the use of small incisions. The rate of intracerebral hemorrhage with cathe-
ter placement is approximately 1% to 2%. Subdural hematoma from over-
drainage occurs less than 5% of the time. Shunt infection in hydrocephalus,
generally, is reported to be approximately 8% to 10%. Most often these in-
fections are insidious and chronic from slow growing Staphylococcus epider-
midis or Propionibacterium acnes species adherent to the shunt system, not
causing a general systemic illness but instead causing poor healing or shunt
dysfunction. If the system becomes infected, the entire shunt system must be
explanted and replaced after antibiotic treatment. Modern shunt catheters
with antibiotic impregnation hold promise for reduced incidence of
infection.
After shunt placement, improvement in gait is the most frequent observ-
able benefit. Although gait improvement is observed after CSF trial drain-
age, it may continue to improve gradually in the months after shunting,
with benefits persisting indefinitely for years, providing the shunt continues
its drainage. Improvements in cognition, memory, and alertness often are
reported by patients. With neuropsychologic testing, however, these cogni-
tive changes are variable and less impressive than the gait improvements.
Urologic symptoms can improve dramatically, restoring urinary continence
and improving patient quality of life.
The reported rate of clinical improvement seen in gait, cognition, and uri-
nary incontinence after shunting varies from as low as 25% to over 90%.
The historic variability in success reflects differences in patient selection, ad-
vances in shunting methods and materials, and definitions of success. In ad-
dition, higher failure rates in earlier studies likely reflect less diagnostic
precision in selecting patients for shunting, based only on symptoms and
large ventricles. In more recent series, where ancillary tests are used to select
shunt responsive patients, greater improvements in clinical outcomes are
seen. In the authors’ experience, the CSF drainage trial has improved the
process of patient selection for a shunt and, therefore, is recommended.
Successful treatment also depends on continuing medical and surgical
follow-up to ensure proper shunt function (ie, draining the optimal
656 FACTORA & LUCIANO

amount). If patients’ symptoms recur or headaches or new symptoms de-

velop, CT or MRI should be performed, and a CSF tap may be indicated.
Follow-up imaging, CSF flow adjustments, shunt taps, and revisions may be
required periodically. Without careful follow-up of shunted patients, gains
made in elderly patients’ function through appropriate shunting may be
lost in the successive years by the shunt failure the development of other ce-
rebral diseases (eg, stroke, Alzheimer’s disease) or systemic diseases (eg, in-
tracerebrovascular disease).

Summary
NPH is a chronic adult hydrocephalus characterized by gait deficit and,
possibly, cognitive slowing and urinary urgency and incontinence. It is a dis-
ease of CSF circulation and parenchymal changes. Essential to the diagnosis
of NPH are ventriculomegaly and gait disturbance, whereas cognitive and
urinary problems also are seen often and, with gait impairment, constitute
Adam’s triad. The existence of other neurologic problems, such as cerebr-
vascular disease, does not eliminate the possibility of NPH but has a negative
impact on the benefits of ventricular shunting. The diagnosis of NPH may
be reinforced with ancillary tests, where the removal of several hundred mil-
liliters of CSF over an extended period of time may be performed optimally
through a lumbar drain. Other tests, including MRI with cine CSF flow
study, CSF infusion studies, intracranial pressure monitoring, and cisterno-
grams, may be useful in reinforcement of the diagnosis but may have little
additional impact on diagnostic accuracy.
Referral for an evaluation by an experienced neurosurgeon should be
considered strongly if symptoms include gait impairment, unexplained by
spinal disease or other progressive neurologic disorder, and ventriculome-
galy as identified on either CT or MRI (ie, quadrant I patients). If ventricu-
lomegaly exists, NPH still may be considered even in the context of other
symptoms and comorbidities (such as spinal or vascular disease) and
some of these patients also may undergo CSF drainage trial to evaluate po-
tential improvement (quadrant II).
Advancements in the technique and materials of CSF shunting and im-
provements in follow-up and postoperative CSF drainage have increased
the possibility of benefit after treatment and reduced complications. Optimal
treatment of NPH requires vigilant patient selection, meticulous surgical im-
plantation, and careful follow-up with optimization. The failure to identify
and treat these patients, however, results in a definite shortening of quality
of life in the elderly population.

References
[1] Hakim S, Adams RD. The special clinical problem of symptomatic hydrocephalus with nor-
mal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics. J Neu-
rol Sci 1965;2:307–27.
 NORMAL PRESSURE HYDROCEPHALUS 657

[2] Krauss JK, Halve B. Normal pressure hydrocephalus: survey on contemporary diagnostic
algorithms and therapeutic decision-making in clinical practice. Acta Neurochir (Wien)
2004;146:379–88 [discussion: 88].
[3] Petersen RC, Mokri B, Laws ER Jr. Surgical treatment of idiopathic hydrocephalus in
elderly patients. Neurology 1985;35:307–11.
[4] Symon L, Dorsch NW, Stephens RJ. Pressure waves in so-called low-pressure hydrocepha-
lus. Lancet 1972;2:1291–2.
[5] Vanneste JA. Three decades of normal pressure hydrocephalus: are we wiser now? J Neurol
Neurosurg Psychiatry 1994;57:1021–5 [comment].
[6] Pang D, Altschuler E. Low-pressure hydrocephalic state and viscoelastic alterations in the
brain. Neurosurgery 1994;35:643–55 [discussion: 55–6].
[7] Conner ES, Foley L, Black PM. Experimental normal-pressure hydrocephalus is accompa-
nied by increased transmantle pressure. J Neurosurg 1984;61:322–7.
[8] Owler BK, Momjian S, Czosnyka Z, et al. Normal pressure hydrocephalus and cerebral
blood flow: a PET study of baseline values. J Cereb Blood Flow Metab 2004;24:17–23.
[9] Corkill RG, Cadoux-Hudson TA. Normal pressure hydrocephalus: developments in deter-
mining surgical prognosis. Curr Opin Neurol 1999;12:671–7.
[10] Estanol BV. Gait apraxia in communicating hydrocephalus. J Neurol Neurosurg Psychiatry
1981;44:305–8.
[11] Nutt JG, Marsden CD, Thompson PD. Human walking and higher-level gait disorders, par-
ticularly in the elderly. Neurology 1993;43:268–79 [comment].
[12] Vanneste J, Augustijn P, Tan WF, et al. Shunting normal pressure hydrocephalus: the pre-
dictive value of combined clinical and CT data. J Neurol Neurosurg Psychiatry 1993;56:
251–6.
[13] Vanneste JA. Diagnosis and management of normal-pressure hydrocephalus. J Neurol 2000;
247:5–14.
[14] Marmarou A BM, Klinge P, Relkin N, et al. The value of supplemental prognostic tests for
the preoperative assessment of idiopathic normal-pressure hydrocephalus. Neurology
2005;57(Suppl 2):S2-17–28.
[15] Tanaka A, Kimura M, Nakayama Y, et al. Cerebral blood flow and autoregulation in nor-
mal pressure hydrocephalus. Neurosurgery 1997;40:1161–5 [discussion: 1165–7].
[16] Shinnar S, Gammon K, Bergman EW Jr, et al. Management of hydrocephalus in infancy: use
of acetazolamide and furosemide to avoid cerebrospinal fluid shunts. J Pediatr 1985;107:
31–7.
[17] Miyati T, Mase M, Banno T, et al. Frequency analyses of CSF flow on cine MRI in normal
pressure hydrocephalus. Eur Radiol 2003;13:1019–24.
[18] Miyake H, Ohta T, Kajimoto Y, et al. Diamox((R)) challenge test to decide indications for
cerebrospinal fluid shunting in normal pressure hydrocephalus. Acta Neurochir (Wien)
1999;141:1187–93.
[19] Klinge P, Ruckert N, Schuhmann M, et al. Neuropsychological sequels to changes in global
cerebral blood flow and cerebrovascular reserve capacity after shunt treatment in chronic hy-
drocephalusda quantitative PET-study. Acta Neurochirurgica Suppl 2002;81:55–7.
[20] Chang CC, Kuwana N, Ito S, et al. Impairment of cerebrovascular reactivity to acetazol-
amide in patients with normal pressure hydrocephalus. Nucl Med Commun 2000;21:139–41.
[21] Pople IK, Edwards RJ, Aquilina K. Endoscopic methods of hydrocephalus treatment. Neu-
rosurg Clin North Am 2001;12:719–35.
[22] Vanneste J, Augustijn P, Dirven C, et al. Shunting normal-pressure hydrocephalus: do the
benefits outweigh the risks? A multicenter study and literature review. Neurology 1992;42:
54–9.