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REVIEW

CLINICAL PRACTICE

Clinical Features of Idiopathic Normal

Pressure Hydrocephalus: Critical Review
of Objective Findings
Brent Bluett, DO,1a Elissa Ash, MD,2a Amtul Farheen, MD,3,4 Alfonso Fasano, MD, PhD,5,6,7,8 Joachim K. Krauss, MD, PhD,9
Alessio Maranzano, MD,10,11 Massimiliano Passaretti, MD,12 David F. Tang-Wai, MD,6,7,8,13 Jay Van Gerpen, MD,14
Araceli Alonso-Canovas, MD, PhD,15 Jinyoung Youn, MD, PhD,16 Jan Malm, MD, PhD,17 and Davide Martino, MD, PhD,18,*
for the International Parkinson and Movement Disorder Society Normal Pressure Hydrocephalus Study Group

ABSTRACT: Background:
Background Idiopathic normal pressure hydrocephalus (iNPH) is characterized by the classic
clinical triad of gait, cognitive, and urinary dysfunction, albeit incomplete in a relevant proportion of patients.
The clinical findings and evolution of these symptoms have been variably defined in the literature.
Objectives To evaluate how the phenomenology has been defined, assessed, and reported, we performed a
Objectives:
critical review of the existing literature discussing the phenomenology of iNPH. The review also identified the
instrumental tests most frequently used and the evolution of clinical and radiologic findings.
Methods The review was divided into 3 sections based on gait, cognitive, and urinary dysfunction. Each
Methods:
section performed a literature search using the terms “idiopathic normal pressure hydrocephalus” (iNPH), with
additional search terms used by each section separately. The number of articles screened, duplicates, those
meeting the inclusion criteria, and the number of articles excluded were recorded. Findings were subsequently
tallied and analyzed.
Results A total of 1716 articles with the aforementioned search criteria were identified by the 3 groups. A total
Results:
of 81 full-text articles were reviewed after the elimination of duplicates, articles that did not discuss
phenomenological findings or instrumental testing of participants with iNPH prior to surgery, and articles with
fewer than 10 participants.
Conclusions “Wide-based gait” was the most common gait dysfunction identified. Cognitive testing varied
Conclusions:
significantly across articles, and ultimately a specific cognitive profile was not identified. Urodynamic testing
found detrusor overactivity and “overactive bladder” as the most common symptom of urinary dysfunction.

Idiopathic normal pressure hydrocephalus (iNPH) is an increasingly in cerebrospinal fluid (CSF) dynamics and possible vascular changes,
recognized disorder that primarily affects the elderly population. First which lead to ventricular enlargement (ventriculomegaly)—the neuro-
formally identified in 1965, its pathophysiology involves disturbances radiological hallmark of the disorder—but typically without increased

1
Central California Movement Disorders, Pismo Beach, California, USA; 2Department of Neurology, Faculty of Medicine, Tel Aviv Sourasky Medical Center, Tel
Aviv, Israel; 3Department of Neurology, Lebanon VA Medical Center, Lebanon, Pennsylvania, USA; 4Department of Neurology, Penn State Hershey Medical Center,
Hershey, Pennsylvania, USA; 5Edmond J. Safra Program in Parkinson’s Disease, Morton and Gloria Shulman Movement Disorders Clinic, Toronto Western Hospital,
University Health Network, Toronto, Ontario, Canada; 6Krembil Brain Institute, University Health Network, Toronto, Ontario, Canada; 7Howard Cohen Normal
Pressure Hydrocephalus Program, University Health Network, Toronto Western Hospital, Toronto, Ontario, Canada; 8Department of Medicine, Division of Neurology,
University of Toronto, Toronto, Ontario, Canada; 9Department of Neurosurgery, Medical School Hannover, Hannover, Germany; 10Department of Neurology, Istituto
Auxologico Italiano IRCCS (Istituto di Ricovero e Cura a Carattere Scientifico), Milan, Italy; 11Department of Pathophysiology and Transplantation, University of
Milan, Milan, Italy; 12Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy; 13University Health Network Memory Clinic, Toronto
Western Hospital, Toronto, Ontario, Canada; 14Department of Neurology, University of Alabama at Birmingham, Huntsville, Alabama, USA; 15Movement Disorders
Unit. Neurology Department, Hospital Universitario Ram
on y Cajal, Madrid, Spain; 16Department of Neurology, Samsung Medical Center, Sungkyunkwan University
School of Medicine, Seoul, South Korea; 17Department of Clinical Neuroscience, Umeå University, Umeå, Sweden; 18Department of Clinical Neurosciences and
Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada
*Correspondence to: Dr. Davide Martino, Department of Clinical Neurosciences, Cumming School of Medicine, University of Calgary Health Sci-
ences Centre, 3330 Hospital Drive NW, Calgary, AB, Canada T2N 4N1; E-mail: davide.martino@ucalgary.ca
Keywords: normal pressure hydrocephalus, phenomenology, gait, cognition, urination.
a
These authors are equally contributed to the manuscript.
Received 9 May 2022; revised 7 September 2022; accepted 23 September 2022.
Published online 00 Month 2022 in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/mdc3.13608

MOVEMENT DISORDERS CLINICAL PRACTICE 2022. doi: 10.1002/mdc3.13608

1
© 2022 International Parkinson and Movement Disorder Society.

REVIEW
CSF pressure.1,2 Clinically, the presence of gait disturbance with vari-
able cognitive impairment and/or urinary dysfunction characterizes the
clinical triad of iNPH, although rarely do patients with iNPH present
with all 3 symptoms. When untreated, these symptoms typically dete-
riorate over time, causing serious physical and cognitive disability.3
Several sets of guidelines for the diagnosis and management of
iNPH have been published,4–7 including American–European guide-
lines4 and 3 editions developed by the Japanese Society of Normal
Pressure Hydrocephalus.5–7 The American–European guidelines pro-
pose criteria for probable, possible, and unlikely iNPH based on both
clinical and brain imaging features. To diagnose probable iNPH based
on American–European guidelines, the clinical features required are
the presence of gait/balance disturbance and at least 1 of either cogni-
tive impairment and/or urinary incontinence/urgency. The diagnosis
of probable iNPH (ie, before definite diagnosis that follows objective
improvement after CSF shunt surgery) based on the third edition of
the Japanese guidelines is more involved and requires any 2 features of
the clinical triad of iNPH, a CSF opening pressure of ≤200 mm H2O,
normal CSF routine tests, and 1 neuroimaging feature or improve-
ment of symptoms after CSF tap and/or drainage test.
The creation of the different revised versions of these guidelines is
based on systematic reviews of the literature compounded by the
application of a specific classification of levels of evidence and recom-
mendation grading. However, the description of the clinical features
and the optimal or acceptable assessment methods used to screen for
their presence are not supported by a high quality of evidence. As a
result, the grading of the recommendations issued in the guidelines
expresses an “expert opinion” level of consensus that is not paired to
a universally accepted assessment procedure. A comprehensive quan-
titative and qualitative summary of gait, cognitive, and urinary fea-
tures of patients screened positive for the diagnosis of iNPH based on
the literature systematic reviews is not included in the commentary
of the diagnostic algorithm presented by the Japanese guidelines.
Moreover, when defining the key clinical features, such as gait
disturbance, the criteria combine more “objective” terminology (eg,
small-step gait) to vague or colloquial terminology (eg, magnetic gait),
hindering the reproducibility of assessment and screening outcome.
Finally, most clinical studies on iNPH applying both diagnostic
screening and severity rating have relied on the application of scales
such as the iNPH Grading Scale. However, this instrument uses, for
each of the 3 components of the clinical triad, anchor points that
are based on functional impairment rather than phenomenology.
To provide a comprehensive analysis of the clinical characteristics
that have been documented by clinical studies involving patients
diagnosed with iNPH, we performed a critical review of the litera-
ture that summarized the objective clinical findings in the 3 cardinal
domains of iNPH, incorporating both those obtained through objec-
tive neurological examination and those obtained through instru-
mental or ancillary investigations routinely used in clinical practice.
Methods
To conduct this critical review, we were guided by the standard-
ized Preferred Reporting Items for Systematic Reviews and
2 MOVEMENT DISORDERS CLINICAL PRACTICE 2022. doi: 10.1002/mdc3.13608
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REVIEW OF NPH PHENOMENOLOGY
Meta-Analyses guidelines in the selection of relevant articles.
The study was conceived and conducted within the International
Parkinson and Movement Disorder Society Normal Pressure
Hydrocephalus Study Group. Based on the clinical triad of
iNPH, we divided the review process into 3 sections: gait dys-
function, cognitive impairment, and urinary dysfunction. Each
section was based on an independent literature search of the
PubMed and EMBASE databases, always incorporating the term
“idiopathic normal pressure hydrocephalus.” In addition to the
latter, section-specific terms were included in the search. Specifi-
cally, for the gait dysfunction section, we also used the terms
“gait” and “gait dysfunction”; for the cognitive impairment sec-
tion, we used the terms “cognition,” “cognitive testing,”
“dementia,” “neuropsychology,” Montreal Cognitive Assessment
(“MoCA”), and Mini-Mental State Examination (“MMSE”); and
for the urinary dysfunction section, we used the terms “urinary
dysfunction,” “urination,” and “urinary incontinence.”
Our review included only articles describing clinical findings
related to at least 1 of the triad symptoms presented prior to any
therapeutic intervention and documented through objective
neurological examination or instrumental testing included in
routine clinical practice. For the section synthesizing gait in this
review, we were interested in assessing the frequency of specific
phenomena characterizing abnormalities of gait that could be
screened through objective neurological examination rather than
in quantitative scores of clinical or instrumental tests used to
measure gait parameters. To this aim, 2 authors (B.B. and D.M.)
proposed an initial list of phenomena judged to be clinically relevant
in iNPH. The list underwent revision through several iterations
involving the whole group, eventually leading to the selection of
15 gait-related phenomena. For our review, we included exclu-
sively articles that reported the prevalence of these 15 clinical gait
features (listed in Table 1), irrespective of the set of criteria used to
diagnose iNPH. Data subjectively reported by patients or other
informants were not included in our data synthesis. Review articles
or articles on iNPH including <10 participants (<15 for the cogni-
tive section) or written in a language other than English were
excluded. Publication time was limited to the 2000 to 2020 period
for the cognitive section to minimize heterogeneity of cognitive
assessment across different studies. For the other 2 sections, the sea-
rch start date corresponded to database inception. Duplicate articles
were identified and removed. The number of articles screened after
removal of duplicates was recorded for each group. Full-text articles
that met the aforementioned inclusion criteria were assessed for eli-
gibility, and those that did not meet the criteria were excluded.
Figure 1 summarizes the search results, including the number of
screened and selected articles and reasons for exclusion.
Results
Gait Dysfunction
A total of 26 full-text articles were reviewed in their entirety for
this section, and their salient findings are summarized in
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BLUETT B. ET AL. REVIEW

TABLE 1 Summary of prevalence of individual abnormal gait characteristics detected through objective neurological examination in studies of patients
with idiopathic normal pressure hydrocephalus
Number of
Studies Reporting
Prevalence of the Prevalence Prevalence
Gait Feature Gait Feature Range, % Median, %

Widened stance or broad-based gait 10 43–100 71

Reduced stride length 9 55–100 97
Shuffling gait 8 39–100 70
Impaired tandem gait 7 93–100 97
Bradykinesia/hypokinesia 7 15–100 65
Freezing of gait 6 30–56 41
Start hesitation 6 25–100 30
Postural instability 5 46–100 53
Turning en bloc 4 33–90 41
Impaired Romberg stance 4 53–100 97
Festination of gait 3 20–27 27
Impaired tandem stance 2 100 100
Irregular step length or cadence 0 – –
Abnormal arm swing 0 – –
Truncal flexion when walking 0 – –

Studies idenfied on PubMed or EMBASE

n = 1722?

Gait dysfuncon Cognive dysfuncon Urinary dysfuncon

n = 744 n = 763 n = 209

Studies not including Studies excluded because arcles not Studies not including instrumental
phenomenological gait findings wrien in English language, inclusion tesng or phenomenological
n = 223 of secondary causes of NPH, studies findings of urinary dysfuncon in
with less than 10 subjects in the trial, iNPH, and studies with less than ten
arcles reporng only cognive parcipants
rating based on the iNPH rang scale n = 203
Studies excluded due to small
sample size (less than 10 but that did not use independent
parcipants) screening or assessment
instruments, and arcles that were
n = 189 Studies excluded because urinary
not available for review n = 707
dysfuncon aer shunt placement
Studies excluded due to lack of n=1
original data (review or guidelines)
n = 127

Studies excluded due to unrelated

topic
n = 105

Studies excluded due to not-English

arcles
n = 74

Studies included in this review Studies included in this review Studies included in this review
n = 26 n = 50? n=5

FIG. 1. Flowchart of the selection process for the included studies in this critical literature review using Preferred Reporting Items for
Systematic Reviews and Meta-Analyses guidelines. iNPH, idiopathic normal pressure hydrocephalus; NPH, normal pressure
hydrocephalus.

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REVIEW REVIEW OF NPH PHENOMENOLOGY

TABLE 2 Instrumental testing used to evaluate cognitive dysfunction in studies of patients with idiopathic normal pressure hydrocephalus
Domain Tests

Frontal executive Frontal Assessment Battery, WAIS-III-Digit Span, Trail Making Test A and/or B, verbal (letter) fluency
tests (Controlled Oral Word Association Test), Digit Span Forward and Backward, Digit Symbol Substitution Test,
and Stroop Color-Word Test
Memory Wechsler Memory Scale–R visual, Rey Auditory-Verbal Learning Test, Galveston Orientation and Amnesia
Test, Benton Visual Retention Test–Revised, Wechsler Memory Scale–Revised Logical Memory Test, Guild
Paragraph Recall Test, Selective Reminding Test of the Brief Repeatable Battery, ADAS Word Recall and
Recognition, Memory Impairment Screen, CogNIT Battery, Bingley’s Visual Memory Test, CERAD, and
Rivermead Behavioral Memory Test
Language Boston Diagnostic Aphasia Examination, Category fluency, Boston Naming Test, Token Test, and Western
Aphasia Battery
Visuospatial Rey-Osterrieth Complex Figure (with a delayed recall component), Line-Tracing Test, Two-Dimensional
Rotation Test, clock draw, WAIS-III-Block Design, and Raven’s Colored Matrices
Praxis Boston Diagnostic Aphasia Examination—Praxis section
Perceptual/speed Purdue Pegboard Test, Mefferd and Moran Perceptual Speed Test, The Grooved Pegboard, finger tapping test,
tests and Identical Forms Test
Deductive reason Raven’s Colored Matrices
Abbreviations: WAIS-III, Wechsler Adult Intelligence Scale–III; ADAS, Alzheimer’s Disease Assessment Scale; CERAD, Consortium to Establish a Registry for
Alzheimer’s Disease.

Table S1. The level of confidence of the diagnosis of iNPH was
not specified in 22 of the 26 articles: in the remaining 4 studies,
3 indicated the diagnosis of all included patients as probable
iNPH8–10 and 1 as “definitive” iNPH.11
There was great heterogeneity across the 26 included studies in
terms of sample size (range, 15–429; median, 39 subjects) and in the
number and type of clinical gait features screened for through
objective neurological examination. None of the screened features
was reported in more than 10 studies. Features for which prevalence
was reported by 7 or more studies were “widened stance or broad-
based gait,” “reduced stride length,” “shuffling gait,” “impaired tan-
dem gait,” and “bradykinesia” or “hypokinesia” (Table 1). A total
of 6 studies reported the prevalence of “freezing of gait” and/or
“start hesitation,” whereas 4 studies reported prevalence of “turning
en bloc.” Other gait features (postural instability, festination of gait,
impaired Romberg stance, and impaired tandem stance) were
reported by a range of 2 to 5 studies.
A large variability was observed also in the range of prevalence
estimates reported by the selected studies for almost all of the gait
features explored (Table 1). The feature that yielded the narrowest
prevalence range was “impaired tandem gait,” for which estimates
ranged between 93% and 100% of patients. Of note, the gait fea-
tures included in the definitions of gait disturbance incorporated in
the diagnostic criteria also yielded wide prevalence ranges (43%–
100% for widened stance or broad-based gait, 39%–100% for shuf-
fling gait, and 33%–90% for turning en bloc).
Cognitive Dysfunction
Our search for this section identified 763 articles. Of these,
707 were excluded (articles not written in the English language,
inclusion of secondary causes of normal pressure hydrocephalus,
studies with fewer than 15 subjects in the trial, articles reporting
only cognitive rating based on the iNPH rating scale but that did
not use independent screening or assessment instruments, and
articles that were not available for review). A total of 52 full-text
articles were therefore reviewed in their entirety, and their salient
findings are summarized in Table S2.
A varied range of cognitive testing approaches was used in these
studies—from a single general cognitive screening test, such as the
MMSE or the MoCA, to comprehensive batteries of neuropsycho-
logical testing. The commonly used screening test was the MMSE
(n = 42 studies). Other screening tests used included the MoCA
(n = 5 studies), Addenbrooke’s Cognitive Examination (n = 2
studies), Repeatable Battery for the Assessment of Neuropsycholog-
ical Status (n = 1 study), and the Modified MMSE (n = 1 study).
The full list of neuropsychological tests used in the selected
studies is provided in Table 2. There were 4 patterns of cognitive
testing that were performed: screening test only (n = 14); screen-
ing test with additional frontal-executive testing (n = 8); screen-
ing test with additional frontal-executive and memory tests
(n = 4), and testing of other cognitive domains, with or without
the addition of general screening instruments, frontal-executive
testing, or memory testing (n = 26). In the latter subset, frontal-
executive domains were the most frequently explored.
The degree of cognitive impairment detected varied across the
selected studies and ranged from mild impairment to frank
dementia, likely because of variability in the duration and sever-
ity of symptoms of patients with iNPH within and across articles
and to the fact that cognitive impairment is neither necessary nor
always detected in patients with this diagnosis. With the excep-
tion of 1 study that identified a significant impairment at baseline

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BLUETT B. ET AL. REVIEW

TABLE 3 Instrumental testing used to evaluate urinary dysfunction in studies of patients with idiopathic normal pressure hydrocephalus
Most Common Percentage of
Number of Instrumental Participants with Total Number
Instrumental Testing Articles Test Finding Available Results of Participants

Urodynamics 4 Detrusor overactivity 89.0 273

Functional neuroimaging 1 Right frontal hypoperfusion Not reported 97

of attention, executive functions, memory, and visuoperceptual/
visuospatial abilities compared with healthy individuals,12 none
of the selected studies demonstrated or reported a well-defined
cognitive dysfunction profile on the basis of screening or neuro-
psychological assessment at baseline, that is, prior to shunt sur-
gery. In line with this, there was no narrative clinical description
of the investigated sample of patients with iNPH that attempted
to describe a specific cognitive syndrome associated with this
diagnosis. Finally, most studies applying neuropsychological test-
ing reported only of cognitive domain improvement following
intervention, which is beyond the scope of our systematic
review.
Urinary Dysfunction
Our search for this section identified 209 articles. Of these,
203 were excluded that did not discuss instrumental testing or
phenomenological findings of urinary dysfunction in iNPH or
included <10 participants, 1 article was not in English, and 1 arti-
cle discussed only urinary dysfunction after shunt placement.
Therefore, 4 full-text articles were included for our synthesis.
Of the 4 articles, 3 evaluated lower urinary tract symptoms in
iNPH, whereas the other article evaluated neurogenic causes of
urinary dysfunction in iNPH using neuroimaging (Table 3). The
3 studies evaluating lower urinary tract symptoms in iNPH per-
formed urodynamic testing to evaluate urinary dysfunction.
Urodynamic testing in these 3 studies found that detrusor over-
activity accounted for the majority of cases of urinary dysfunc-
tion (“overactive bladder” associated with subjective report of
nocturia and/or urge incontinence) in the setting of iNPH.13–15
The only article that evaluated neuroimaging correlates of
detrusor overactivity in iNPH documented hypoperfusion of the
right frontal gyrus, suggesting that this might lead to disinhibition
of the micturition reflex.16
contributing to the variability in the frequency and severity of
clinical features reported here.
Gait disturbance is typically considered the presenting symp-
tom of iNPH, is classically used to define a positive tap test, and
is typically the most responsive symptom to shunt surgery.3 Our
review identified, as the most common findings on routine
objective examination of gait in iNPH, widened stance or
broad-based gait, reduced stride length, shuffling gait, impaired
tandem gait, and bradykinesia/hypokinesia. Freezing of gait, start
hesitation, turning en bloc, and postural instability are also
observed but at a lower frequency. Instrumental testing of gait
has been performed using a gait mat, motion sensors, and optoki-
netic systems, although their role in clinical practice is still
unclear, and therefore these objective findings were not included
in our literature synthesis.
The American/European and Japanese diagnostic criteria of
iNPH have defined gait abnormality based on a systematic litera-
ture review.4–7 Our search strategy retrieved only 26 articles that
screened for specific objective pathological gait features, with the
earliest published in 1980. It is plausible to assume that the diag-
nostic qualifiers of gait disturbance are present in the majority or
totality of patients in the excluded 223 studies that did not screen
for specific features but used consensus criteria. The wide preva-
lence ranges reported by the 26 studies that provide prevalence
data on specific abnormal gait features may have different expla-
nations. First, gait disorders may be absent in some patients
receiving a diagnosis of iNPH, particularly if the condition is at
an early stage. The lack of information on the level of diagnostic
confidence (probable vs. possible) in 22 of the 26 selected studies
does not allow us to ascertain how the prevalence of gait features
was influenced by diagnostic confidence. Variability in study
design (controlled vs. uncontrolled, retrospective vs. prospective)
may have also contributed to the variable prevalence ranges,
although the small number of studies prevents us from drawing
firm conclusions on the impact of study design. The wide preva-
lence ranges might also be attributed to insufficient or inconsis-

Discussion tent application of diagnostic criteria for iNPH in clinical

Our critical review highlights a marked variability of clinical
findings related to the gait and cognition domains of the clinical
triad of iNPH as detected through routine objective neurological
examination (gait) and cognitive screening or neuropsychometric
assessment (cognition). Albeit yielding a consistent finding of
detrusor hyperactivity, objective findings related to the urinary
domain of the triad are limited by the very small number of
studies that could be included in our synthesis. The stage of
development of the condition is likely to be partially
research, suboptimal diagnostic accuracy or reliability of the diag-
nostic criteria in screening for gait abnormalities of iNPH, or a
combination of both. Finally, gait problems may be also attrib-
uted to intercurrent conditions unrelated to iNPH, such as mus-
culoskeletal comorbidities or peripheral neuropathies.
The composition of cognitive testing batteries varies signifi-
cantly across different studies of patients with iNPH, with basic
screening tests, such as the MMSE, used most frequently,
followed by specific neuropsychological tests tapping on the
frontal executive and memory domains. Several reports

MOVEMENT DISORDERS CLINICAL PRACTICE 2022. doi: 10.1002/mdc3.13608 5


REVIEW
highlighted frontal dysexecutive changes—declines in attention,
information processing and psychomotor speed, and other exec-
utive functions—as common and early cognitive abnormalities
associated with iNPH.12,17,18 However, this notion does not
appear to be corroborated by large observational studies, either
cross-sectional or prospective, that screened cognition broadly
using independently validated screening or assessment tools on
sufficiently sized clinical samples and compared patients with
iNPH to age-matched individuals from the general
population.19–23 Our review showed that the available studies
either reported postinterventional changes on preselected cogni-
tive domains or described small clinical samples using uncon-
trolled study designs or compared patients with iNPH to
neurodegenerative dementias such as Alzheimer’s disease
(AD).24–28 Despite the lack of a well-defined cognitive profile,
our literature review suggests the possible presence of impair-
ments in other cognitive domains including memory domains
and visuospatial and visuo-constructional skills that are propor-
tionally less investigated in patients with iNPH.29–32 Moreover,
the association between cognitive impairment and coexisting
neuropsychiatric features such as apathy might be important to
improve our understanding of the cognitive phenotype of this
condition33,34 given their link to dysfunctional connectivity
between frontal regions, limbic cortical regions, and subcortical
deep gray matter structures.35–37
It has also been suggested that differences in patterns of early
cognitive decline in iNPH may be useful to differentiate it from
the early pattern of decline in AD.38 In particular, patients with
iNPH are believed to show a more consistent impairment in
attention and processing speed, whereas patients with AD show
more consistent deficits in memory and orientation and nam-
ing.25,38 In our review, only 2 studies confirmed this
finding,12,25 even if with a certain degree of cognitive overlap
between the 2 diagnoses.12
Only 4 articles that specifically evaluated urinary dysfunction
in iNPH using routine objective assessment procedures met the
inclusion criteria of this review. However, these articles consis-
tently used urodynamic testing reporting detrusor overactivity in
the majority of participants with iNPH. This abnormality was
associated with “overactive bladder” manifesting with subjec-
tively reported urinary urgency, increase in urinary frequency,
nocturia, or frank urge incontinence.
Our critical review carries the major limitations of the
included studies. The lack of prospective assessment of patients
in the available literature limits our understanding of the most
common sequence of objective abnormalities in the natural his-
tory of iNPH before therapeutic intervention. Another potential
limitation is the inherent variability of this population of patients,
although reviewed studies adopted the diagnostic criteria avail-
able at the time of recruitment. Yet, these guidelines are slightly
different, also depending on the version. All patients in the
reviewed articles had evidence of enlarged ventricles, and the vast
majority featured at least 2 symptoms of the classic triad, that is,
gait dysfunction, cognitive dysfunction, and/or urinary inconti-
nence. Most studies also required a normal opening CSF pres-
sure, whereas only some required a positive tap test. In addition,
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REVIEW OF NPH PHENOMENOLOGY
some studies had patients with iNPH and another neurodegener-
ative disorder, most commonly AD, Parkinson’s disease, or vas-
cular cognitive impairment.
Gait and balance impairment remain the hallmark of the
iNPH clinical triad. Nevertheless, the observed heterogeneity in
prevalence of different gait descriptors should motivate joint
efforts from different categories of clinical experts, in particular
neurosurgeons and movement disorders neurologists. The
observed suboptimal consistency in recognizing a specific profile
of gait disturbances of iNPH may result, among other factors,
from the still recurrent use of vague terminology to describe gait
dysfunction in this condition, which includes terms that may lead
to arbitrary interpretation (eg, “magnetic gait”) or have become
obsolete because they are mechanistically inaccurate (eg, “gait
apraxia”). Objective examination of gait in routine clinical prac-
tice, even before instrumental gait laboratory-supported assess-
ment, should be more comprehensive and also focus on the
evaluation of phenomena so far reported inconsistently in iNPH,
for example, freezing of gait, start/turn hesitation, and fes-
tination. To better determine the phenomenology of gait dys-
function in iNPH, the standardization of objective and
laboratory assessment should also be pursued. New phenomeno-
logical and laboratory-supported observational studies should be
used to update the existing diagnostic guidelines, promoting the
use of clinical qualifiers showing greater specificity, sensitivity,
and interobserver reliability. When objective measures are not
available, clinical evaluation of gait by at least 2 raters would
more accurately define phenomenological findings. In addition,
future studies should also evaluate the presence of postural
abnormalities that are anecdotally reported in iNPH but that
have probably been overlooked in the available studies, for
example, camptocormia in patients with festination and the “Pisa
syndrome.” Another aspect that deserves consideration is the
development of the gait disorder and balance problems over
time. The occurrence of comorbid or secondary parkinsonism
(bradykinesia in particular) should be also addressed in more
depth as this seems to be more common than initially
believed.39,40 Finally, the findings on objective physical examina-
tion of gait should be adequately correlated to laboratory gait
analysis findings. This might inform on how to select patients
who require a more in-depth gait study as well as on the clinical
meaningfulness and impact on treatment of laboratory gait analy-
sis in patients with iNPH.
To determine the cognitive profile of iNPH, and if cognitive
domains other than frontal dysexecutive can also occur, a consis-
tent cognitive battery that examines all cognitive domains should
be provided to each patient with suspected iNPH. The battery
should include a general screening test, such as the MoCA, and
additional tests that include at least 1 in-depth assessment into
verbal memory/recall, frontal test, language, and visuospatial
domains. This comprehensive approach could be simplified in
the future, when a more defined cognitive phenotype
(or spectrum) of iNPH will be demonstrated, thus justifying the
use of a narrower cognitive assessment to confirm diagnosis and
monitor evolution and treatment response. In addition, the
timing of onset of cognitive dysfunction should be more

BLUETT B. ET AL.
accurately recorded in relation to other clinical symptoms of
iNPH, as it may be helpful to differentiate iNPH from AD and
other neurodegenerative cognitive disorders that may initially
present similarly.
Unlike neuropsychometry and laboratory gait analysis,
urodynamic testing is a more invasive procedure that may not be
a suitable screening method to confirm urinary dysfunction in
suspected iNPH. However, more research is needed to correlate
urodynamic findings to the subjective report of urinary symp-
toms as well as to rating scales assessing the severity and complex-
ity of urinary dysfunction in iNPH. This type of study will
contribute enormously to optimize the accuracy of subjective or
noninstrumental evaluation of urinary symptoms as well as physi-
cians’ confidence in diagnosing urinary dysfunction in iNPH.
Overall, there is a need to extend the application of compre-
hensive clinimetric rating instruments encompassing the nonin-
vasive assessment of all the domains in this triad with acceptable
reliability and high convergent validity with more costly and/or
invasive instrumental tests. The iNPH grading scale is the main
obvious candidate instrument to play this role in routine clinical
practice, but a more robust validation of this scale with the
objective measures evaluated in our systematic review is
warranted. This validation process will also clarify whether the
existing criteria used to diagnose the clinical triad of iNPH can
be confirmed or whether they should be integrated with objec-
tive instrumental testing. Finally, the time course of progression
of symptoms in iNPH has not been thoroughly investigated and
needs to be defined. Disease progression reflects the great vari-
ability in the assessment, diagnosis, and definition of gait, cogni-
tive, and urinary dysfunction in iNPH. Ongoing longitudinal
studies of the clinical features of iNPH will help more accurately
delineate the precise definition of each symptom. Despite the
variability in findings, based on the data gathered by our critical
review, a possible clinical definition of iNPH is a progressive neuro-
logical condition characterized by broad-based shuffling gait, early frontal
dysexecutive syndrome, and urge incontinence due to detrusor hyperactiv-
ity. How this definition would contribute to diagnosing iNPH
and reshape the future diagnostic guidelines is presently
unknown, and further studies are certainly warranted.
Acknowledgment
The authors acknowledge the members of the International
Parkinson and Movement Disorders Society Normal Pressure
Hydrocephalus Study Group.
Author Roles
(1) Research Project: A. Conception, B. Organization,
C. Execution; (2) Statistical Analysis: A. Design, B. Execution,
C. Review and Critique; (3) Manuscript: A. Writing of the First
Draft, B. Review and Critique.
B.B.: 1B, 1C, 2A, 2B, 3A
MOVEMENT DISORDERS CLINICAL PRACTICE 2022. doi: 10.1002/mdc3.13608
23301619, 0, Downloaded from https://movementdisorders.onlinelibrary.wiley.com/doi/10.1002/mdc3.13608 by Western Univ,Western Libraries Monograph Acquisitions, Wiley Online Library on [27/11/2022]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
REVIEW
E.A.: 1C, 2C, 3B
A. Farheen: 1C, 2C, 3B
A. Fasano: 1A, 2A, 3B
J.K.K.: 1A, 2A, 3B
A.M.: 1C, 2B, 3B
M.P.: 1C, 2B, 3B
D.F.T.-W.: 1B, 1C, 2B, 2C, 3A, 3B
J.V.G.: 1C, 2C, 3B
A.A.-C.: 1C, 2C, 3B
J.Y.: 1C, 2C, 3B
J.M.: 2C, 3B
D.M.: 1A, 1B, 1C, 2B, 2C, 3B
Disclosures
Ethical Compliance Statement: This review is a systemic
review and did not recruit any subjects. Therefore, this study was
done without an approval of institutional review board or
patient’s consent. We confirm that we have read the Journal’s
position on issues involved in ethical publication and affirm that
this work is consistent with those guidelines.
Funding Sources and Conflicts of Interest: The authors have
no funding sources or conflict of interest specific to this manu-
script to report.
Financial Disclosures for the Previous 12 Months: The
authors have no financial disclosure specific to this manuscript to
report. ■
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Supporting Information
Supporting information may be found in the online version of
this article.
Table S1. Summary of studies reporting quantitative data on
objective findings of physical examination related to gait and
stance in patients with normal pressure hydrocephalus (NPH).
Table S2. Summary of studies reporting objective findings of
neuropsychological assessment in patients with idiopathic normal
pressure hydrocephalus.