REVIEW

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The diagnosis and treatment of idiopathic normal

pressure hydrocephalus
Gary L Gallia, Daniele Rigamonti and Michael A Williams*

INTRODUCTION

S U M M A RY
Idiopathic normal pressure hydrocephalus (INPH) is a syndrome that
is characterized by gait impairment, cognitive decline and urinary
incontinence, and is associated with ventriculomegaly in the absence of
elevated cerebrospinal fluid (CSF) pressure. There is significant variation
in the clinical presentation and progression of this disorder, and its
diagnosis often represents a challenge for neurologists and neurosurgeons.
Various supplemental tests, including the CSF tap test, external CSF
drainage via spinal catheter, and CSF outflow resistance determination,
can improve the accuracy of predicting a response to surgical treatment.
CSF shunting provides significant symptom improvement in the majority
of appropriately evaluated patients. In 2005, an international study group
published evidence-based guidelines for the diagnosis and management
of INPH. This review will highlight the clinical presentation, radiographic
findings, supplemental prognostic tests, differential diagnosis, surgical
treatment and outcomes of INPH.
KEYWORDS cerebrospinal fluid shunt, diagnosis, idiopathic normal pressure
hydrocephalus, hydrocephalus, surgery

REVIEW CRITERIA
PubMed was used for a literature search from 1966 to the present for articles
on normal pressure hydrocephalus. The abstracts of retrieved citations were
reviewed and prioritized by relative content. Full publications were obtained and
also searched for relevant references and related articles.

Normal pressure hydrocephalus (NPH), which

was first introduced into the English clinical
literature in 1965, describes the syndrome of gait
disturbance, cognitive deterioration and urinary
incontinence that is associated with ventricular
enlargement in the absence of elevated cerebrospinal fluid (CSF) pressure.1,2 When it occurs
secondarily to other disease processes, including
subarachnoid hemorrhage, traumatic brain
injury, cerebral infarction and meningitis, this
syndrome is referred to as secondary NPH.3,4
NPH in patients without known precipitants
is termed primary or idiopathic NPH (INPH).
In 2002, an international NPH study group
was assembled to develop guidelines for INPH.
These guidelines, which were published in 2005,
covered four major topics: the clinical diagnosis
of INPH, the value of supplementary diagnostic
tests, surgical management, and outcome of CSF
shunting in INPH.59 Television and internet
media campaigns have increased public awareness of INPH,10 and many patients and their
families are now asking their physicians about
the possibility of a diagnosis of NPH.
CLINICAL PRESENTATION
AND RADIOGRAPHIC EVALUATION

GL Gallia is a neurosurgery resident, D Rigamonti is Professor of

Neurosurgery, Radiation Oncology and Molecular Sciences, Radiology, and
Physical Medicine and Rehabilitation, and MA Williams is an associate
professor in the Departments of Neurology and Neurosurgery, all at the Johns
Hopkins Hospital, Baltimore, MD, USA.
Correspondence
*Johns Hopkins Hospital, Adult Hydrocephalus Program, 600 N. Wolfe Street, Phipps 100,
Baltimore, MD 21287, USA
michael.a.williams@jhmi.edu
Received 31 March 2006 Accepted 12 May 2006
www.nature.com/clinicalpractice
doi:10.1038/ncpneuro0237

JULY 2006 VOL 2 NO 7

INPH is characterized clinically by the triad

of gait disturbance, dementia and urinary
incontinence.1,2 Symptoms typically develop
insidiously, and generally occur between the sixth
and eighth decades of life.1114 Gait disturbances
are typically the first signs of INPH, and have
been variously described as apraxic, bradykinetic,
glue-footed, magnetic, parkinsonian and shuffling.8,15 Patients often present with a history of
falls. The aberrant ambulation observed in INPH
is characterized by a slow, wide-based gait, short
shuffling steps, and difficulty in turning and
tandem walking. Notably, there is no significant
motor weakness.
The cognitive deficits are typically of the
subcortical type, characterized by inattention,
psychomotor retardation and difficulty with

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Figure 1 Neuroimaging of two patients with idiopathic normal pressure

hydrocephalus. (A) Head CT scan demonstrating ventriculomegaly without
significant cortical atrophy. (B) Brain MRI demonstrating ventriculomegaly and
evidence of subcortical ischemic changes. Both patients idiopathic normal
pressure hydrocephalus symptoms improved following shunt placement.

executive function.1,16 Apraxia, agnosia and

aphasia are rare in INPH.
Urinary incontinence is the third primary
symptom of INPH. In early stages of INPH,
urinary frequency and urgency are present.
With disease progression, urinary and even
fecal incontinence can be observed. Urodynamic
testing demonstrates bladder hyperactivity.17
There is significant variation in the clinical
presentation, severity and progression of these
symptoms, and the entire triad need not be
present in order to consider a diagnosis of INPH.
Typically, however, gait and balance impairment appear either before or concurrently with
urinary incontinence or the onset of dementia.
The full diagnosis of INPH requires evidence
from the patients clinical history, physical
examination and neuroimaging.
An essential part of the evaluation of patients
with suspected INPH is neuroimaging with
either CT or MRI to assess ventricular size
(Figure 1). Although no findings on imaging
studies of the brain are sufficient on their own
to diagnose INPH, ventricular enlargement is
necessary to establish the diagnosis of INPH for
patients with appropriate symptoms. A frontal
horn ratio (Evans index), defined as the maximal
frontal horn ventricular width divided by the
transverse inner diameter of the skull, signifies ventriculomegaly if it is 0.3 or greater.8,18
Other radiographic findings associated with
INPH include the following: periventricular

376 NATURE CLINICAL PRACTICE NEUROLOGY

hyperintensities, which are often associated

with subcortical microvascular ischemia (socalled small-vessel disease), but do not exclude
the possibility of INPH; increased CSF flow
velocity in the aqueduct; thinning and elevation of the corpus callosum on sagittal images;
and no visible evidence of obstruction to
CSF flow.8,14,1922
Several other brain imaging techniques
have been investigated in patients with INPH,
including single-photon emission CT, PET,
nuclear cisternography, and CSF flow velocity.
The diagnostic value of these tests has not been
established, and, at present, these examinations
are not part of the routine work-up of patients
with suspected INPH.7,8
DIFFERENTIAL DIAGNOSIS

Because INPH is a disease of the elderly population, an age-group in which gait difficulties, dementia, and urinary incontinence are
common, a diverse differential diagnosis of the
individual symptoms should be considered,
including neurodegenerative diseases, vascular
etiologies and urological disorders. INPH is one
of many disorders affecting gait; other common
conditions include peripheral neuropathy,
cervical or lumbar stenosis, arthritis, vestibular
diseases and Parkinsons disease. The differentiation between INPH and Parkinsons disease
can be challenging. Both diseases share a hypokinetic gait owing to a decreased stride length,
but specific features associated with INPH
include a broad-based gait pattern with outward
rotated feet and a diminished step height, relatively preserved arm swing, and erect trunk.23
Additionally, external cues only mildly improve
gait in INPH, whereas they are effective at raising
the stride length and cadence in patients with
Parkinsons disease.23
Dementia is a common clinical syndrome
in the elderly and has numerous underlying
causes.24 The cognitive impairment observed in
INPH has some similarity to other subcortical
dementias, including Parkinsons disease, diffuse
Lewy body disease and vascular dementia. The
absence of apraxia, agnosia and aphasia can help
differentiate INPH from cortical dementias,
including the most common dementing illness,
Alzheimers disease.
As with gait disturbances and cognitive
decline, there are numerous etiologies of urinary
incontinence in the elderly, all of which should
be evaluated in the work-up of INPH. Urinary

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incontinence might reflect prostate disease in

men, or stress incontinence or chronic urinary
tract infections in women.
SUPPLEMENTAL PROGNOSTIC TESTS

According to the INPH consensus guidelines,

patients can be categorized as having probable,
possible or unlikely INPH on the basis of history,
neurological examination and neuroimaging (see
Supplementary Box 1 online).8 Without additional testing, between 46% and 61% of probable
and possible INPH patients will improve with
surgical treatment.7 There are several supplemental tests that improve diagnostic accuracy and
should be considered in patients with probable
and possible INPH. These tests include a CSF tap
test, external CSF drainage via spinal drainage,
and CSF outflow resistance determination.7
The CSF tap test, also called a large-volume
lumbar puncture, involves the withdrawal of
4050 ml of CSF by means of lumbar puncture. Symptomatic improvement after CSF
removal increases the likelihood of a favorable
response to shunt placement (positive predictive value 73100%).7 The CSF tap test has a
low sensitivity (2661%), however, and a negative test cannot be used to exclude a diagnosis
of INPH.7 The opening pressure should also be
measured. The range of INPH opening pressure is 60240 mm H2O, or 4.417.6 mmHg.7,8
Detailed documentation of the clinical examination findings by a physician or other qualified
health-care professional before and after CSF
removal is strongly recommended, as patients
and families might be biased by their hope of
recovery, and can over-interpret changes in
function after the lumbar puncture.
Assessing clinical response to prolonged CSF
drainage via a spinal catheter has a combination of high sensitivity (50100%), specificity
(60100%) and positive predictive value (80
100%).7 This approach requires hospitalization and nursing staff trained and competent
in managing external CSF drainage, and can
be associated with a higher complication rate
(infection, nerve root irritation). Consequently,
it is currently only performed at a limited
number of centers in the US. Identification of
abnormally elevated CSF outflow resistance also
increases the likelihood of a favorable response
to shunt placement compared with the clinical
and radiographic evaluations,7 and this technique is in more common use in Europe than
in the US.

TREATMENT

The treatment for INPH is surgical diversion

of CSF. This is accomplished by implanting a
shunt to drain CSF from either the intracranial
ventricular system or the lumbar subarachnoid
space to a distal site, such as the peritoneal or
pleural cavity or the venous system, where the
CSF can be reabsorbed. The most common
shunts utilized today are ventriculoperitoneal
(VP) and ventriculoatrial (VA) shunts. Several
factors are considered when evaluating patients
for placement of a shunt, including the risk:
benefit ratio of the procedure, sites of the proximal and distal catheter, valve specifics, and
shunt-related complications.6
Placement of a shunt is a neurosurgical
procedure performed under general anesthesia,
taking less than an hour to complete. There are
few intraoperative and perioperative complications. As with other invasive surgical procedures,
a risk:benefit ratio evaluation should take into
account comorbidities, functional status and life
expectancy of the patient.
The selection of the proximal and distal catheter sites and valve type is individualized. The
proximal catheter is typically placed within
the ventricles, although the lumbar subarachnoid
space can be used in patients for whom there is
concern of injury to the brain from inserting
a ventricular catheterfor example, a patient
with previous injury to the right hemisphere,
in whom a complication of shunt insertion into
the left hemisphere could result in bilateral cerebral injury. The distal catheter site depends on
assessment of the patients anatomy and surgical
history. For example, previous abdominal
surgery or a history of peritonitis might render
the peritoneal cavity less suitable for CSF absorption. In these situations, VA shunts are useful; a
third option is to place the distal catheter into
the pleural cavity.
Various types of valve design are available,
including differential pressure valves (DPVs),
and flow-limiting valves. In the case of DPVs,
the shunt opens and CSF flows when the pressure difference across the valve exceeds a set
value. These valves can generally be classified
as low, medium or high pressure. With a DPV,
change in body position from supine to upright
can cause overdrainage of CSF by a siphoning
effect if the hydrostatic pressure gradient (i.e.
the vertical distance between the ventricles
and the distal catheter) is greater than the
opening pressure of the DPV. To reduce this

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Figure 2 Development and noninvasive treatment of bilateral subdural hygromas following placement
of a programmable shunt for idiopathic normal pressure hydrocephalus. (A) Postoperative head CT
shows no evidence of subdural fluid collections. (B) Head CT demonstrating bilateral subdural hygromas
(arrows). The patient noted worsening gait and balance similar to her preoperative symptoms. To treat the
hygromas, the pressure setting of the shunt was increased. (C) Head CT scan 4 weeks later demonstrating
resolution of the subdural fluid collections. The patients symptoms concomitantly improved.

gravity-dependent drainage, anti-siphon devices

were developed.25,26 Flow-limiting valves were
designed to operate more physiologically by
maintaining a constant flow rate over a range
of pressure differentials. The flow through these
valves is typically regulated by increasing the
resistance as intracranial pressure increases.
Under conditions of high intracranial pressure, however, these valves operate in a high
flow-rate mode. As yet, there is no evidence that
one particular shunt design or configuration
produces better outcomes in treating INPH than
any other, and the selection usually depends as
much on the surgeons preference as it does on
other factors.
The most recent advance in shunt valve design is
the development of adjustable or programmable
valves.2731 These valves, which are designed
to enable a range of pressure settings from
20200 mm H2O, depending on the make and
model, can be adjusted transcutaneously by the
use of a magnetic device. These valves are particularly beneficial in the management of INPH,
because both overdrainage and underdrainage
can be managed noninvasively (Figure 2).
An important limitation of adjustable shunts
is that they are susceptible to external magnetic
fields. Although this magnetic susceptibility is
not usually problematic for adults in daily life,
magnetic fields from MRI scanners and even
small magnets (such as kitchen magnets) that are

378 NATURE CLINICAL PRACTICE NEUROLOGY

placed too close to the shunt might alter the pressure setting of the valve.32,33 As a general rule,
patients with adjustable shunts can have MRI
scans, but they should be advised to have the
shunt re-programmed as soon as possible
afterwards to limit the risk of inadvertent
overdrainage or underdrainage.
RISKS AND COMPLICATIONS

Although CSF shunting is a relatively straightforward neurosurgical procedure, it is associated with numerous potential complications.
These complications can be categorized into
three main groups: first, those related to the
operative procedure (e.g. intracerebral hematoma, catheter malposition, shunt infection);
second, those related to the shunt system (e.g.
valve malfunction, proximal or distal catheter
obstruction); and third, those attributable to
the flow characteristics of the shunt system (e.g.
overdrainage-associated headaches, or subdural
hygroma or hematoma).
The most common complication after shunt
placement is obstruction. In INPH, this is clinically manifested by recurrence of the original
INPH symptoms after a period of recovery, but
it should also be suspected for patients who have
no improvement after shunt surgery.34 Injection
of a radionuclide tracer into the shunt reservoir
can determine whether flow through the shunt
is partially or totally obstructed.35,36

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The INPH guidelines list complications that

include shunt malfunction (20%), subdural
hematoma (217%), seizure (311%), shunt
infection (36%) and intracerebral hematoma
(3%).6 In our recent series of 132 INPH patients,
33% had their shunts revised, 7% developed an
infection, 2% developed a subdural hematoma,
and 1% developed an intracerebral hematoma.14 Because our clinical approach includes
monitoring and investigation of the possibility
of shunt obstruction, our rate of shunt revision
might be higher than the rates reported in other
published series.
OUTCOMES

Variable improvement rates after CSF shunting

have been reported in the literature. This variation can be explained mainly by differing
methods of patient selection criteria and postoperative assessment, and variations in followup periods. The INPH guidelines reported
improvement rates of 3096%.5 A 2001 metaanalysis reported that 59% of patients improved
after shunting, and 29% experienced prolonged
improvement. Although all symptoms can
resolve following shunt surgery, gait is the
most likely to improve.3 We found that 75% of
patients had improvement in at least one INPH
symptom, and 46% had improvement in all
INPH symptoms at 18 months. Altogether, 93%
had gait improvement, but dementia and urinary
incontinence were only half as likely to improve.
The time to intervention is important: numerous
studies have demonstrated that a longer duration of INPH symptoms is associated with lower
likelihood of response to shunting.14,37,38
Of the three classic symptoms, cognitive
impairment is the least likely to improve following
treatment.3 Although variable rates of improvement have been reported,5,39 we and others have
observed significant cognitive improvement in
more that 50% of patients following shunting.4043
This is in contrast to the outcomes observed
with Alzheimers disease, in which fewer than
half of the patients exhibit a clinically significant
response to anticholinesterase therapy.44
Because none of the currently available prognostic tests have 100% sensitivity, there are
patients who will not improve after shunting.
If a head CT scan demonstrates no problems
requiring surgical intervention, evaluation of
shunt patency is indicated.34 If the shunt is
obstructed, it should be revised. If the shunt
is functioning adequately and the patient has

not improved clinically, either the patient

does not have NPH, or, alternatively, they
have comorbid conditions severe enough that
treatment of INPH is unable to improve the
patients symptomatology.
MODEL OF CNS DISEASE

INPH may represent a unique reversible form

of neuronal injury. The precise impact of this
disease on the CNS, and how the associated
neurological symptoms that might be present for
months or years can reverse in weeks or months,
is not fully understood. Future basic science and
clinical studies exploring hydrocephalus will
not only help us to understand the mechanisms
of neuronal injury and recovery in INPH, but
might also elucidate mechanisms that could be
applicable to other CNS disorders. The National
Institutes of Neurological Disorders and Stroke,
in collaboration with other NIH Institutes, sponsored a 2-day workshop on hydrocephalus in
September 2005 to promote awareness of and
interest in hydrocephalus research.
EPIDEMIOLOGY

Only a few epidemiological studies on INPH

are available, so the incidence and prevalence of
this disorder are difficult to determine. The incidence of INPH has been reported to be between
1.8 cases per 100,000 individuals and 2.2 cases
per 1,000,000 individuals.45,46 A door-to-door
survey of individuals older than 65 years of age
in two German villages reported that the prevalence of NPH was 0.41% in this age-group.47 It
has been reported that between 1.6% and 5.4%
of patients with dementia have NPH,48,49 and a
recent analysis of nondegenerative nonvascular
dementia from a registry in Rochester, MN,
found no cases of NPH from 1990 to 1994.
The authors concluded, however, that with a
population of 70,745, failure to find NPH was
not unexpected.50
CONCLUSIONS

INPH is a potentially treatable condition

consisting of gait disturbance, dementia and
urinary incontinence, and it should therefore
be included in the differential diagnosis of
elderly patients presenting with such symptoms.
Patients are categorized into those with probable,
possible and unlikely INPH on the basis of their
clinical history, physical examination, radiographic evaluation and supplemental testing.
The mainstay of treatment remains the surgical

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placement of a shunt, and in properly selected

patients shunting is associated with significant
symptom improvement. The recently published
international INPH guidelines providing the
framework for standardization of patient evaluation, diagnostic work-up and outcome assessment
are useful in guiding the clinical management of
INPH patients.

Supplementary information is available on the

Nature Clinical Practice Neurology website.

10
11

KEY POINTS

Idiopathic normal pressure hydrocephalus

(INPH) is characterized by gait disturbance,
cognitive deterioration and urinary
incontinence, and is associated with
ventricular enlargement in the absence of
elevated cerebrospinal fluid (CSF) pressure

INPH may represent a unique reversible form

of neuronal injury, the mechanism of which is
not well understood

Only a few epidemiological studies on INPH

are available, so the incidence and prevalence
of this disorder are difficult to determine

An essential part of the evaluation of patients

with suspected INPH is neuroimaging with
either CT or MRI to assess ventricular size

12

13

14

15

16

Because INPH is a disease of the elderly

population, a diverse differential diagnosis of
the individual symptoms should be considered,
including neurodegenerative diseases, vascular
etiologies and urological disorders
Treatment for INPH is surgical diversion of
CSF, which is accomplished by implanting a
shunt to drain CSF from either the intracranial
ventricular system or the lumbar subarachnoid
space to a distal site, where the CSF can
be reabsorbed

17

18

19

20

21

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Competing interests
D Rigamonti and MA
Williams have declared
associations with the
following companies/
organizations: Medtronic,
the Hydrocephalus
Association. See the
article online for full details
of the relationship. GL
Gallia declared he has no
competing interests.

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