J Neurosurg 61:501-509, 1984

The syndrome of normal-pressure hydrocephalus

JOHN VASSILOUTHIS, M.D.
Neurosurgical Department, Army Veterans Administration Hospital (417 NITS), Athens, Greece
~" A series of 40 patients with the syndrome of normal-pressure hydrocephalus is presented. Diagnosis was
based on clinical and computerized tomography (CT) scan criteria and was followed by the insertion of a
ventriculoperitoneal shunt in every instance. All patients responded favorably to treatment (four of them had
a fair outcome), and this response was maintained. There was one postoperative death, but no other serious
complications. This study demonstrates that it is possible to diagnose the syndrome of normal-pressure
hydrocephalus on the basis of clinical and CT scan criteria without any other invasive investigations. Such
patients should respond favorably to an adequately functioning shunting system.
KEY WORDS
normal-pressure hydrocephalus
ventriculoperitoneal shunt
9 hydrocephalus
9

HE term "normal-pressure hydrocephalus" (NPH)

is used to describe a clinical syndrome mainly
comprising gait disturbance, dementia, and urinary incontinence, and is associated with dilatation of
the ventricular system of the brain and normal cerebrospinal fluid (CSF) pressure at lumbar puncture. 27 Since
the initial reports from the Massachussets General Hospital, 1,2.46,47numerous series of patients apparently suffering from this syndrome have been described in the
literature. 3-6'8'9"11'22'24"32-34'36'41'53'56'58'64 In these studies,
attention has mainly concentrated on establishing diagnostic criteria and prognostic factors that would reliably predict a favorable outcome following a ventricular
shunting procedure. 4'6'8'11,22,34,36,53,56,63,64
In this report, we present the results of treatment of
40 patients with the syndrome of NPH. The questions
we have attempted to answer include: Are there any
criteria on the basis of which we can select with certainty at least some of the patients with the syndrome?
Are there any patients with the syndrome who do not
respond favorably to a shunting procedure? and Why
do they not respond?

Clinical Material and M e t h o d s

The study concerns 40 patients in whom the diagnosis of NPH was based on clinical and computerized
tomography (CT) criteria. All the patients were hospitalized in either the Neurosurgical or the Neurological
Departments of the Army General Hospital or the
Army Veterans Administration Hospital between January, 1979, and August, 1983. For treatment and incluJ. Neurosurg. / Volume 61/September, 1984

9 computerized tomography

sion in the study, patients must have had a clinical

history and symptomatology suggestive of the syndrome and the demonstration of a dilated unobstructed
ventricular system on the CT scan, together with some
additional features which excluded dilatation ex vacuo
of the ventricular system. Lumbar puncture with measurement of the CSF pressure was not considered necessary and was therefore performed according to the
needs of each patient. Apart from routine investigations, no other tests were performed before treatment
was undertaken.
In this series of patients we did not consider separating patients with a known cause of their communicating hydrocephalus from those with an unknown
etiology (idiopathic). The reasons for this are discussed below.
Patient Population
Almost half of the patients were selected from a pool
of patients with otherwise unexplained gait disturbance
and dementia. The remainder were mostly neurosurgical patients under care or being followed for other
intracranial disorders. There were 24 patients with communicating hydrocephalus of known etiology, and 16
in whom no cause for the hydrocephalus could be
identified from either the investigations or the patient's
medical history, based on information given mainly by
the relatives (Table 1).
Gait disturbance was present in every patient and
ranged from a mild deficit in balance to complete
inability to walk or even stand. Excluding the four
501

J. Vassilouthis
TABLE 1
Clinicalfeatures in 40 patients with the NPH syndrome*
Etiology

No. of
Cases

Age R a n g e

Duration of
Symptoms
(range)

Urinary
DeterioraGait Distioni" Mutism
Dementia Incontiturbance
nence

SAH
10
20-63
3 wks-10 mos
4
2
6~
craniotomy
6
42-64
3 wks-2 yrs
2
0
6
CNS infection
3
15-58
2 mos- 1 yr
0
0
3
head injury
5
58-75
6 mos-10 yrs
1
1
4:~
unknown (idiopathic)
16
52-80
1-4 yrs
0
0
16
total cases
40
7
3
35~;
* NPH = normal-pressurehydrocephalus;SAH = subarachnoidhemorrhage;CNS -- central nervoussystem.
t Deteriorationof preexistingdeficitsor failureto make a good recovery.
The remainingpatients werebedridden.

patients with subarachnoid hemorrhage (SAH) who

were bedridden at diagnosis, in every instance the onset
of the disability was insidious and required some time
to become apparent to the patient or his relatives. This
period of time was shorter in the group with known
etiology (several weeks to 1 year, with one exception)
than in the idiopathic group (1 to 4 years). In four
patients in the idiopathic group, inability to walk was
the only presenting symptom, save for a mild memory
deficit considered "normal" for the patient's age.
Frank dementia (akinetic mutism in three cases) was
the predominant feature in eight patients. Impairment
of memory, mainly short-term, slowing of thought, and
lack of attention or initiative were the most common
forms of disturbance in the remaining 24 cases. In four
patients the dementia antedated the disturbance of gait
by several months to years; in fact it dominated the
clinical picture. One of these patients had suffered
severe ischemic damage to the hypothalamus and the
medial aspects of the frontal lobes following rupture of
a giant anterior communicating artery aneurysm, another had a severe closed head injury, residual aphasia,
hemiparesis, and personality changes, and two patients
were in the idiopathic group. These patients are discussed separately, as they form the group of "fair"
responders to a shunting procedure.
Urinary incontinence was present in almost half the
patients (19 out of 40), and in every instance it followed
disturbance of gait and mentation. Most often it took
the form of lack of concern, but in a few patients it
appeared as urgency of micturition.
Other symptoms or signs, such as headache, dizziness, visual disturbances, or pyramidal or extrapyramidal signs, were less frequent and are not considered in
this study. In three patients (two with SAH and one
with a history of head injury), the diagnosis of the
syndrome was suspected when their preexisting neurological deficits began to deteriorate. Interestingly, their
CT scans demonstrated, in addition to ventricular dilatation, the presence of areas of low density roughly
corresponding to the underlying ischemic white matter
which disappeared following treatment. 59 Finally, fail502

8
5
3
4
12
32

7
2
1
3
6
19

ure to make a satisfactory recovery following SAH or

surgery for intracranial tumor (meningioma or pituitary
adenoma) led to the correct diagnosis "n four other
patients.
Computerized Tomography Findings
Most of the CT scans were obtained on an Ohio
Nuclear "Delta" head scanner and displayed on a 160
x 160 matrix. In the eight most recent cases, a Pfizer
AS and EO450 body scanner was also used.* Four CT
scan criteria were used in this study, based on our
previous experience6~ and the experience of others.6.11,21,26,44,48,56These criteria were the presence on the
CT scan of: 1) dilatation of the unobstructed ventricular
system; 2) obliteration of the cerebral sulci; 3) areas of
periventricular low density; and 4) "rounding" of the
frontal horns of the lateral ventricles. We considered
these characteristics as indicating that the coexisting
ventricular enlargement was not an ex vacuo variant.
Subsequently, only patients satisfying at least one of
the first two criteria, in addition to the ventricular
dilatation, were offered treatment and included in
the study.
Dilatation of the unobstructed ventricular system
was present in every patient and was considered a
prerequisite for treatment and inclusion in the study.
Only patients with more than moderate ventricular
enlargement were selected, in order to overcome arguments regarding the actual size of the ventricles. 66 Thus,
only patients in whom the ratio of the width of the
lateral ventricles at the level of the foramina of Monro
to the transverse inner diameter of the skull at the same
level was above 1:5 were included. 6~
Obliteration of the cerebral sulci (Figs. 1, 2, and 3),
as seen in the uppermost CT cuts, was present in 3 l
cases (Table 2); thus, no patients were excluded from
the study on the basis of the absence of this criterion.
* Delta scanner 25 manufactured by Ohio Nuclear, Inc.,
2910 Aurora Road, Solon, Ohio. Pfizer AS and EO450 body
scanner manufactured by Pfizer Medical Systems, Inc., 9052
Annapolis Road, Columbia, Maryland.
J. Neurosurg. / Volume 61/September, 1984

Normal-pressure hydrocephalus

FIG. 1. The chief computerized tomography characteristics in a 70-year-old woman with normal-pressure
hydrocephalus syndrome, a and b: Marked ventricular dilatation and "rounding" of the frontal horns of the
lateral ventricles are seen. c: Periventricular areas of low density are indicated by arrows, d: The cerebral
sulci are obliterated (arrows).

FIG. 2. Obliteration of the cerebral sulci (arrows) in a 72-year-old man with ventricular enlargement. Note
the considerable dilatation of the subarachnoid spaces at the lower levels (including the Sylvian fissures).

FIG. 3. Left Pair: Obliteration of the cerebral sulci in a 58-year-old man with the normal-pressure
hydrocephalus syndrome (small arrow). Note the widening of the Sylvian fissures (large arrow). Right Pair."
Reappearance of the cerebral sulci 1 year following treatment (small arrows). Note the diminution in the size
of the Sylvian fissures (large arrow).

The width of the cerebral sulci and its predictive value

regarding response to treatment have been discussed
previously. 6J1'22'26'36'56 In our study, obliteration of the
sulci was sometimes observed in combination with
dilatation of the subarachnoid spaces at lower levels,
including the Sylvian fissures (Figs. 2 and 3). This

J. Neurosurg. / Volume 61/September, 1984

finding was considered to indicate obstruction of the

subarachnoid space of the convexity (Table 2). In a few
cases, which were followed postoperatively with CT
scanning, reappearance of the cerebral sulci was documented (Fig. 3), and the validity of this criterion was
reinforced.
503

J. Vassilouthis
TABLE 2
Computerized tomography characteristics in 40 patients with the NPH syndrome*
Etiology

SAH
craniotomy

CNS infection
head injury
unknown (idiopathic)
total cases

No. of Cases

> Moderate
Ventricular
Enlargement

Obliteration
of Cerebral
Sulci

10
6

10
6

8 (2)?
4 ( 1)?

3
5
16
40

3
5
16
40

3
4 (2)?
12 (4),
31 (9)?

Periventricular
Areas of Low "Rounding" of
Density
Frontal Horns

8
5

3
3
8
27

Cortical
Atrophy

7
5

3
4
9
28

1
1

2
4
8

* NPH = normal-pressurehydrocephalus;SAH = subarachnoidhemorrhage;CNS = centralnervoussystem.

t Numbers in parenthesesindicate caseswith dilatation of the subarachnoidspacesat lowerlevels(Sylvianfissuresincluded).

In one SAH patient, in one head-injured patient, and

in two in the idiopathic group, cerebral sulci were
clearly visible in the uppermost cuts, but in the remaining cases no firm judgment could be made for technical
reasons (including movement artifacts or no available
uppermost CT cuts). The combination of the clinical
picture and the presence of areas of periventricular low
density in these last patients was, however, considered
sufficient for inclusion of the patients in the study (Fig.
4). This feature was taken to indicate transependymal
movement and absorption of CSF, which is known to
take place in communicating hydrocephalus. 13'25'3~176
Granholm 21 has shown that periventricular areas of low
density are present in cases of hydrocephalus and disappear following shunting. This was subsequently confirmed by others. 43'44Overall, 27 patients exhibited periventricular low-density areas on CT scanning, and this
was taken as an absolute indication for treatment in
every patient (Figs. 1 and 4). Interestingly, this feature
was seen in four of our patients with clearly visible
cortical sulci (Fig. 4), suggesting that obliteration of the
subarachnoid space in these areas is not necessarily

present in every patient with this syndrome, and that it

is at least partially dependent on the actual size of the
cerebral gyri.
Finally, "rounding" of the frontal horns of the lateral
ventricles (Fig. 1) was demonstrated in 28 patients and
was considered as equivalent to the pneumoencephalographic (PEG) finding that the greater impact of
ventricular dilatation is on the anterior portions of the
ventricular system. 21'37 This feature did not serve as an
absolute indication for treatment in this series.
An interesting group of eight patients, half of whom
were in the idiopathic category, showed CT features
suggestive of cortical atrophy. This as a rule was better
seen on CT cuts lower than the uppermost ones; for
example, cuts through the upper part of the lateral
ventricular bodies. No attempt was made to distinguish
these appearances etiologically, as this was beyond the
scope of this study. It is emphasized, however, that in
every patient of this group the combination of clinical
and additional CT scan features such as those mentioned above suggested that the coexistent ventricular
dilatation was at least partially due to obstruction of

FIG. 4. Areas of periventricular low density (arrows) in a 75-year-old patient with ventricular dilatation 6
months following head injury. The cerebral sulci are clearly visible.
504

J. Neurosurg. / Volume 61/September, 1984

Normal-pressurehydrocephalus
the CSF bulk flow, and required shunting. This was
shown to be the case in every patient of this group,
even in the three patients who showed only a fair
response to treatment.
Management
Following diagnosis and routine preoperative investigations (chest radiograph, electrocardiogram, blood
biochemical analysis, blood count and hematocrit, and
urinalysis), all the patients were given prophylactic flucloxacillin (Floxapen) in a dose of 500 mg three times
daily for 1 day pre- and 2 days postoperatively. Surgery
was performed under general anesthesia. A ventriculoperitoneal shunting system was installed in every patient, incorporating a medium-pressure Holter valve
and a standard Rickham reservoir.t The standard surgical technique was used, with special care taken that
no part of the device came in contact with the patient's
skin. Operative time ranged from 50 to 70 minutes.
Patients were encouraged to leave their bed in the 2nd
postoperative day and were usually discharged home
between the 8th and the 10th day. Follow-up examination was performed at 3 and 6 months, at 1 year, and
thereafter every year.
Results

Response to treatment was graded according to the

criteria proposed by Black,6 which are as follows:
Excellent: resumed pre-illness activity without deficit
Good: resumed pre-illness activity with moderate
deficit
Fair: improved but no return to previous work
Poor: no change or worse.
Table 3 summarizes the results of treatment. Thirty
patients achieved an excellent result. Of the remaining
10, six were judged as good responders in that they
obtained complete resolution of gait disturbance but
not the memory deficit. They were nevertheless able to
return to their previous activities. Four patients showed
only a fair response to treatment. These patients deserve
special mention. The first patient was a 23-year-old
man who suffered two SAH's within 1 month from
rupture of a giant anterior communicating aneurysm.
This was clipped elsewhere. Following surgery, he remained inert and apathetic for a few months and then
began to improve, but reached an unsatisfactory plateau
in 1 year. Memory and emotional dysfunction, together
with mild disturbance of gait, were the predominant
symptoms. There was no urinary incontinence. His CT
scan showed ischemic changes of the medial aspects of
the frontal lobes, more than moderate ventricular dilatation, and areas of periventricular low density. Following treatment, he demonstrated definite improvement

t Medium-pressure Holter valve and Rickham reservoir,

supplied by Codman and Shurtleff, Inc., Randolph, Massachusetts.
J. Neurosurg. / Volume 61 /September, 1984

TABLE 3
Results of surgery in 40 cases of NPH*
Etiology
SAH
craniotomy
CNS infection
head injury
unknown (idiopathic)
total cases

NO.casesOfExcellent Good
10
6
3
5
16
40

9
5
3
3
10
30

0
1
0
1
4
6

Follow-Up
Fair Period (yrs)
1
0
0
1
2
4

1-4
1-2
1-4
89
89189

* N P H = normal-pressure hydrocephalus;SAH = subarachnoid

hemorrhage;CNS = centralnervoussystem.

of his gait but no other significant change. A C T scan

6 months following surgery showed disappearance of
the periventricular lucencies but no change in the size
of the ventricles.
The second patient with only fair response was a 73year-old man with a severe closed head injury and a
left parietal extradural hematoma. When seen 6 months
after the accident he was mute and bedridden with right
spastic hemiparesis. He was incontinent of urine and
feces. A C T scan showed ventricular dilatation with
periventricular lucencies and visible cerebral sulci (Fig.
4). A few days following surgery he began to converse
and take a few steps with support, but 6 months after
surgery he remains dysphasic with right hemiparesis,
and at times he is incontinent of urine. The third
patient was a 72-year-old man, who presented with a 3year history of gait disturbance leading to inability to
walk or stand and frank dementia of 2 years' duration.
He was also incontinent of urine. A CT scan demonstrated some cortical atrophy, ventricular enlargement,
and definite obliteration of the cerebral sulci, together
with dilatation of the subarachnoid spaces at lower
levels (Fig. 2). One year after surgery, this patient walks
normally; he is continent of urine but continues to have
severe memory deficit.
The last patient in this group, a 73-year-old man,
was referred with a history of gait disturbance of 10
months' duration and a longer history of short-term
memory deficit and mild dementia. His medical history
included coronary artery bypass surgery for myocardial
ischemia and two mild ischemic strokes of the right
cerebral hemisphere. A C T scan showed ischemic lesions and cortical atrophy in both hemispheres and
ventricular dilatation combined with obliteration of the
cerebral sulci. Soon after surgery he was able to walk
almost normally (and he remains able to do so); however, his dementia remains unchanged 6 months postoperatively.
There were four additional patients (two with SAH
and two in the idiopathic group) who showed an initial
satisfactory response to surgery only to return to their
preoperative level in 1 to 2 days. In every instance,
palpation of the valve showed that it emptied and filled
505

J. Vassilouthis
properly, and the patients' postoperative CT scans
showed no change in the size of the ventricles and no
additional features. Pumping of the valve was then
started three times daily (30 pumpings each time) as
inadequate function of the system was suspected. This
produced a definite improvement in the patients' condition within 48 hours. Improvement was dramatic in
two cases. Eventually, three of these patients achieved
an excellent and one a good response to treatment.
Pumping, performed by instructed relatives, was successfully discontinued in two patients in 3 months, and
is continuing in the rest (6 months and 1 year later,
respectively).
There was one postoperative death in this series. This
was in an 80-year-old man with a 4-year history of gait
disturbance leading to inability to stand or walk; he had
no other symptoms. He responded dramatically to the
shunting procedure, and was able to walk on the 3rd
postoperative day. He was discharged home on the 9th
postoperative day, but died from bronchopneumonia
in another hospital 5 weeks later.
Obstruction of the proximal end of the shunt system
was diagnosed in one patient and the ventricular catheter was revised. Four patients developed postoperative
urinary tract infections or bronchopneumonia, and
were treated successfully with the appropriate antibiotics. There were no cases of shunt infection or postoperative subdural hematomas.
Discussion
Eighteen years since its initial description, 2,27 the
syndrome of normal-pressure hydrocephalus continues
to present questions, mainly regarding reliable diagnostic criteria and prognostic factors associated with a
shunting procedure. 6'22'36'53'56 There is little disagreement about the clinical symptoms that constitute
the syndrome. Disturbance of gait, dementia, and urinary incontinence are regarded as the predominant
signs. 1,2'16'27The demonstration of a dilated ventricular
system on neuroradiological studies is the cardinal feature, but it is now well appreciated that the CSF pressure
is not normal in these patients, at least not continuously.9.~1.22,28,48,54,55The demonstration of a definite and
at times dramatic improvement of a patient's symptoms following a shunting procedure 1'2'27'46 has stimulated tremendous interest in this treatable cause of
dementia. ~0
The initial enthusiasm5~has gradually subsided, leaving behind a voluminous amount of information. Numerous studies dealing with the syndrome and proposing criteria for selection of patients appropriate for
treatment have appeared in the literature. These include
PEG f e a t u r e s , 23'24'36'37'56'58'64 iodine- 13 I-labeled human
serum albumin (RIHSA) scanning patterns following
intrathecal administration, 3'29'37'56'58 lumbar45'48 or ventriculolumbar38 subarachnoid infusion tests, and evaluation of the patients' mental function before and after
withdrawal of small amounts of CSF. 17'63 Measure506

ments of cerebral blood ~]OW,39 CT features, 6"I1"22'26"36

and patterns of continuously measured and recorded
intracranial pressure (ICP) 9'11'22"28'48'54'55 have been proposed as predictors of a response to treatment. It is
generally agreed that continuous ICP monitoring offers
one of the most reliable means for selection of patients
likely to respond to treatment.48 Since the pioneering
work of Symon, et al., 55 many other investigators have
demonstrated that the ICP in these patients is not
normal, thus refuting the title of the syndrome. They
also showed that the presence in the record of the socalled "B-waves" is of important prognostic significance. T M
More recently, B0rgesen, et aL, 8 found
that measurement of the conductance to outflow of the
CSF is more useful in predicting the outcome to treatment than measurement of the ICP.
Obstruction of the bulk flow of the CSF in the
subarachnoid space distal to the outlet foramina of the
fourth ventricle has been proposed as the initial defect
in this syndrome. This eventually leads to ventricular
dilatation, which is responsible for the clinical picture. 2"23'27'38 Several pathoanatomical reports have
substantiated this proposition, demonstrating leptomeningeal fibrosis mainly in the basal cisterns or the
subarachnoid space of the convexities of the hemispheres with or without changes of the arachnoid granulations in patients with known etiology (SAH, central
nervous system infection, head trauma, intracranial
surgery) or unknown (idiopathic) cause of their hydrocephalus. 12'13'29'49'5~ Additional findings include ventricular ependymal disruption, periventricular white
matter edema, subependymal glial reaction, and periventricular loss of myelin staining, 13 features that are
considered to be caused by disturbance of the CSF flow
and ventricular enlargement. As transependymal and
possibly transchoroidal absorption of CSF is enhanced,4~ the initial intracranial hypertension subsides but the ventricles continue to enlarge, leading to
destruction of periventricular nerve fibers.61 How ventricular enlargement continues in the presence of a fall
in CSF pressure is difficult to explain. Hakim and
Adams27 offered the proposition that, since the total
force on the ventricular wall is related to both area and
pressure, if the area of the ventricles is increased, a
smaller pressure is required to maintain their dilated
state. This theory has since been taken further by other
authorsJ 5'2~ It is also interesting to note that experimental obliteration of the subarachnoid space leads to
ipsilateral ventricular dilatation,25 which lends support
to the proposition that obliteration of the subarachnoid
space of the convexities contributes to an increase in
cerebral mantle pressure between the ventricles and the
subdural space. 25'3~
It is well known that some patients are found at
postmortem examination to have changes due to both
Alzheimer's disease and leptomeningeal fibrosis, causing obstruction of the subarachnoid space. 13 Postmortem studies of patients with the NPH syndrome showing extensive hypertensive cerebrovascular disease with
J. Neurosurg. / Volume 61/September, 1984

Normal-pressurehydrocephalus
multiple small infarcts but without gross leptomeningeal changes should be viewed with caution; well-organized subdural membranes were present in two of the
three reported cases and in the third the diagnosis of
the syndrome was not c e r t a i n . 14'35
In clinical practice, when a patient presents with gait
disturbance and memory deficit (with or without urinary incontinence), a decision must be made whether
a radiologically demonstrated ventricular dilatation is
clinically significant and therefore requires shunting, or
whether it is only the result of wasting of cerebral
substance (dilatation e x vacuo). The existing body of
evidence does not offer reliable criteria on which to
base with certainty the distinction between these entities. 6'7'48 The basic question remains: Do any cases of
NPH syndrome not respond to treatment? And if, yes,
Why do they not respond? Almost all investigative
efforts have been concentrated in "identifying the winners;" that is, the patients who will respond favorably
to a shunting procedure.I~ As a result, conclusions have
been based on the response to treatment of groups of
patients with ventricular dilatation fulfilling various
criteria. The validity of these criteria was subsequently
evaluated in retrospect according to the observed response of the patient to the shunting procedure. It is
obvious that in this process two variables are at work.
First, the clinical material may not be pure. It is inevitable that some patients with dilatation e x vacuo and/
or some with both the syndrome and additional cerebral
pathology (such as Alzheimer's disease) escape recognition and are included in the studies. These cases
should not have been included in the evaluation process
because if they had been recognized at the beginning
they would not have been treated by shunting. The
second factor involved the effectiveness of treatment.
In most of the studies, this was judged on the supposition that a properly selected and adequately functioning
shunt system had been installed. It is surprising how
few studies have addressed the question of evaluation
of shunt function, particularly in cases of idiopathic
NPH that failed to respond to treatment. 4'36'56'65
Separation of the group of patients with NPH of
unknown etiology (idiopathic group) by many authors 6'22'26'32'59 from those with known cause, as if different pathological mechanisms are involved in the two
groups, has not in our opinion contributed to the clarification of the issues. If there are different mechanisms
involved, then we must accept that we are dealing with
different entities. In this respect, it is of interest to note
the results of a recent study which showed similar
patterns of ICP recordings in two groups of hydrocephalic patients, one with known etiology and another
of the idiopathic type. 11 It could, of course, be claimed
that patients of the first group, as a rule, respond
favorably to treatment, 56 and this is not so in the
idiopathic group. It is, however, also true that the
diagnosis of the syndrome is usually certain from the
medical history in the first group and, when response
to treatment is not satisfactory, a vigorous search for
J. Neurosurg. / Volume 61/September, 1984

the possible factors follows. This usually identifies an

inadequately functioning shunt, which is subsequently
revised.
In our study we attempted to investigate the possibility of using relatively simple criteria as a basis for
diagnosing the NPH syndrome, avoiding invasive techniques. The clinical criteria used are in accordance with
those reported previously. 6'16'22'24'36'47'53'56Gait disturbance was present in every patient (that is, no patient
without this symptom was considered for treatment).
As a rule, gait disturbance heralded the other symptoms, 16,18and improved in every patient following treatment. Dementia was the dominant symptom in eight
patients and was present in 32. Following treatment it
definitely improved in 22 patients, was slightly ameliorated in six, and remained unchanged in four (all cases
with underlying additional cerebral pathology). Urinary
incontinence, when present, resolved following treatment. The selection of CT scan criteria was based on
published evidence, 6'11.22.26.36,44,60and on the belief that
the CT scan appearance would represent existing pathoanatomical changes, as reported by previous investigators.12'13"25'29'49'50'57
The criteria used in this study can be criticized as
strict. However, considering the fact that all the patients
diagnosed as suffering from the NPH syndrome on the
basis of these criteria responded favorably to shunting,
we conclude that they are at least valid in selecting with
certainty patients who do have the syndrome. Our
suggested indications for treatment are given, and the
opportunity to study the response to them in a homogeneous group is offered.
With regard to treatment, we used a medium-pressure valve system in all patients in an attempt to avoid
the development of subdural hematomas, which are
more common when low-pressure systems a r e used. 33
We also used the same system to standardize treatment
throughout the series. The finding that four of the
patients eventually required pumping of their valves in
order to achieve and maintain a satisfactory result
implies that in these cases a low-pressure or high-flow
system was probably required. It also suggests that in
these patients every effort should be undertaken to
assure adequate function of the shunting system before
failure of the treatment is accepted.
Four patients showed a "fair" response to treatment
in that they only achieved improvement of gait disturbance. We believe that these patients represent examples
of cases where the NPH syndrome is superimposed on
preexisting cerebral pathology (ischemia, trauma, possible Alzheimer's disease, and multi-infarct dementia,
respectively). ~1'~9'36'56 Surgical treatment is only expected to alleviate symptoms caused by the NPH syndrome without influencing the underlying pathology,
and in our opinion this is what occurred in these
patients. Thus, the second conclusion that emerges
from this study is that all patients with the NPH syndrome respond or should respond to the placement of
an adequately functioning shunting device.
507

J. Vassilouthis
This study suggests that it is possible to base the
diagnosis of the syndrome of N P H on clinical and CT
criteria. Favorable response to treatment in patients so
selected can be obtained without unacceptably high
mortality or morbidity rates.

Addendum
Since submission of this manuscript, a relevant report has appeared (Salibi NA, Lourie GL, Lourie H: A
variant of normal-pressure hydrocephalus simulating
Pick's disease on computerized tomography. Report of
two cases. J Neurosurg 59:902-904, 1983). In this
paper, dilatation of the Sylvian fissures, mimicking
atrophy of Pick's disease, was shown to be a feature in
two cases of the N P H syndrome, both of which responded favorably to shunting.

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Manuscript received December 5, 1983.

Accepted in final form March 26, 1984.
Address reprint requests to: John Vassilouthis, M.D.,
8 Alopekis Street, Kolonaki, Athens, Greece.

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