ABSTRACT
Idiopathic normal pressure hydrocephalus (INPH) is a treatable neurological disorder
in older adults involving disturbances of gait/balance, control of micturition, and/or
cognition in combination with enlargement of the cerebral ventricles. Diagnosis can
be challenging due to its varied presentation and overlap with other disorders common
in the elderly. Evidence-based consensus guidelines for diagnosis and treatment of
INPH have been created that can assist in clinical management. Diagnosis requires
clinical documentation of one or more of the characteristic symptoms of INPH in combination
with a brain imaging study demonstrating nonobstructive ventricular enlargement disproportionate
to cerebral atrophy. Gait and balance disturbances are the most common presenting
findings in INPH and may occur alone or together with cognitive and urinary symptoms.
Adjunct tests, particularly those involving transient removal of cerebrospinal fluid
via lumbar puncture or lumbar drain, can serve the dual purpose of adding to diagnostic
certainty and assisting in prognostication about response to treatment. Prognostication
is important because neurosurgical treatment by placement of a ventricular shunt,
while effective, carries the risk of potentially significant morbidity. Outcome of
shunting in INPH is most often successful when patients are accurately diagnosed,
suitably evaluated for surgical candidacy, and managed carefully throughout the preoperative,
surgical, and postoperative periods.
KEYWORDS
Normal pressure hydrocephalus - communicating adult hydrocephalus - NPH - INPH - diagnosis
- treatment - outcome
REFERENCES
- 1
Adams R D, Fisher C M, Hakim S, Ojemann R G, Sweet W H.
Symptomatic occult hydrocephalus with “normal” cerebrospinal-fluid pressure: a treatable
syndrome.
N Engl J Med.
1965;
273
117-126
- 2
Bret P, Guyotat J, Chazal J.
Is normal pressure hydrocephalus a valid concept in 2002? A reappraisal in five questions
and proposal for a new designation of the syndrome as “chronic hydrocephalus.”
J Neurol Neurosurg Psychiatry.
2002;
73
9-12
- 3
Casmiro M, D'Alessandro R, Cacciatore F M, Daidone R, Calbucci F, Lugaresi E.
Risk factors for the syndrome of ventricular enlargement with gait apraxia (idiopathic
normal pressure hydrocephalus): a case-control study.
J Neurol Neurosurg Psychiatry.
1989;
52
847-852
- 4
Silverberg G D.
Normal pressure hydrocephalus (NPH): ischaemia, CSF stagnation or both.
Brain.
2004;
127
947-948
- 5
Momjian S, Owler B K, Czosnyka Z, Czosnyka M, Pena A, Pickard J D.
Pattern of white matter regional cerebral blood flow and autoregulation in normal
pressure hydrocephalus.
Brain.
2004;
127
965-972
- 6
Silverberg G D, Mayo M, Saul T, Rubenstein E, McGuire D.
Alzheimer's disease, normal pressure hydrocephalus, and senescent changes in CSF circulatory
physiology: a hypothesis.
Lancet Neurol.
2003;
2
506-511
- 7
Chakravarty A.
Unifying concept for Alzheimer's disease, vascular dementia, and normal pressure hydrocephalus:
a hypothesis.
Med Hypotheses.
2004;
63
827-833
- 8
Bateman G A.
Pulse wave encephalopathy: a spectrum hypothesis incorporating Alzheimer's disease,
vascular dementia and normal pressure hydrocephalus.
Med Hypotheses.
2004;
62
182-187
- 9
Marmarou A, Bergsneider M, Relkin N, Klinge P, Black P M.
Development of guidelines for idiopathic normal-pressure hydrocephalus: introduction.
Neurosurgery.
2005;
57(suppl)
S1-S3
- 10
Relkin N, Marmarou A, Klinge P, Bergsneider M, Black P M.
Diagnosing idiopathic normal-pressure hydrocephalus.
Neurosurgery.
2005;
57(suppl)
S4-S16
- 11
Meier U, Zeilinger F S, Kintzel D.
Signs, symptoms and course of normal pressure hydrocephalus in comparison with cerebral
atrophy.
Acta Neurochir (Wien).
1999;
141
1039-1048
- 12
Stolze H, Kuhtz-Buschbeck J P, Drucke H et al..
Gait analysis in idiopathic normal pressure hydrocephalus: which parameters respond
to the CSF tap test?.
Clin Neurophysiol.
2000;
111
1678-1686
- 13
Haan J, Jansen E N, Oostrom J, Roos R A.
Falling spells in normal pressure hydrocephalus: a favourable prognostic sign?.
Eur Neurol.
1987;
27
216-220
- 14
Blomsterwall E, Svantesson U, Carlsson U, Tullberg M, Wikkelso C.
Postural disturbance in patients with normal pressure hydrocephalus.
Acta Neurol Scand.
2000;
102
284-291
- 15
Krauss J K, Regel J P, Droste D W, Orszagh M, Borremans J, Vach W.
Movement disorders in adult hydrocephalus.
Mov Disord.
1997;
12
53-60
- 16
Stolze H, Kuhtz-Buschbeck J P, Drucke H, John K, Illert M, Deuschl G.
Comparative analysis of the gait disorder of normal pressure hydrocephalus and Parkinson's
disease.
J Neurol Neurosurg Psychiatry.
2001;
70
289-297
- 17
Merten T.
[Neuropsychology of normal pressure hydrocephalus].
Nervenarzt.
1999;
70
496-503
- 18
Iddon J L, Pickard J D, Cross J, Griffiths P D, Czosnyka M, Sahakian B J.
Specific patterns of cognitive impairment in patients with idiopathic normal pressure
hydrocephalus and Alzheimer's disease: a pilot study.
J Neurol Neurosurg Psychiatry.
1999;
67
723-732
- 19
Klinge P, Ruckert N, Schuhmann M, Dorner L, Brinker T, Samii M.
Neuropsychological testing to improve surgical management of patients with chronic
hydrocephalus after shunt treatment.
Acta Neurochir Suppl.
2002;
81
51-53
- 20
Boon A J, Tans J T, Delwel E J et al..
Dutch normal-pressure hydrocephalus study: prediction of outcome after shunting by
resistance to outflow of cerebrospinal fluid.
J Neurosurg.
1997;
87
687-693
- 21
Vanneste J AL, Hyman R.
Klinische en neuropsychologische kenmerken van normal pressure hydrocephalus.
Ned Tijdschr Geneeskd.
1987;
131
1080-1084
- 22
Schneider U, Malmadier A, Dengler R, Sollmann W P, Emrich H M.
Mood cycles associated with normal pressure hydrocephalus.
Am J Psychiatry.
1996;
153
1366-1367
- 23
Pinner G, Johnson H, Bouman W P, Isaacs J.
Psychiatric manifestations of normal-pressure hydrocephalus: a short review and unusual
case.
Int Psychogeriatr.
1997;
9
465-470
- 24
Holodny A I, Waxman R, George A E, Rusinek H, Kalnin A J, de Leon M.
MR differential diagnosis of normal-pressure hydrocephalus and Alzheimer disease:
significance of perihippocampal fissures.
AJNR Am J Neuroradiol.
1998;
19
813-819
- 25
Bradley Jr W G, Kortman K E, Burgoyne B.
Flowing cerebrospinal fluid in normal and hydrocephalic states: appearance on MR images.
Radiology.
1986;
159
611-616
- 26
Bradley Jr W G, Whittemore A R, Kortman K E et al..
Marked cerebrospinal fluid void: indicator of successful shunt in patients with suspected
normal-pressure hydrocephalus.
Radiology.
1991;
178
459-466
- 27
Ulug A M, Moore D F, Bojko A S, Zimmerman R D.
Clinical use of diffusion-tensor imaging for diseases causing neuronal and axonal
damage.
AJNR Am J Neuroradiol.
1999;
20
1044-1048
- 28
Chun T, Filippi C G, Relkin R, Zimmerman R D.
Diffusion changes in normal pressure hydrocephalus.
Proc Intl Soc Magn Reson Med.
2000;
8
797
- 29
Alexopoulos G S, Meyers B S, Young R C et al..
The vascular depression hypothesis.
Arch Gen Psychiatry.
1997;
54
915-922
- 30
Damasceno B P, Carelli E F, Honorato D C, Facure J.
The predictive value of cerebrospinal fluid tap-test in normal pressure hydrocephalus.
Arq Neuropsiquiatr.
1997;
55
179-185
- 31
Malm J, Kristensen B, Karlsson T, Fagerlund M, Elfverson J, Ekstedt J.
The predictive value of cerebrospinal fluid dynamic tests in patients with idiopathic
adult hydrocephalus syndrome.
Arch Neurol.
1995;
52
783-789
- 32
Marmarou A, Bergsneider M, Klinge P, Relkin N, Black P M.
The value of supplemental prognostic tests for the preoperative assessment of idiopathic
normal-pressure hydrocephalus.
Neurosurgery.
2005;
57(suppl)
S17-S28
- 33
Dumarey N E, Massager N, Laureys S, Goldman S.
Voxel-based assessment of spinal tap test-induced regional cerebral blood flow changes
in normal pressure hydrocephalus.
Nucl Med Commun.
2005;
26
757-763
- 34
Walter C, Hertel F, Naumann E, Morsdorf M.
Alteration of cerebral perfusion in patients with idiopathic normal pressure hydrocephalus
measured by 3D perfusion weighted magnetic resonance imaging.
J Neurol.
2005;
252
1465-1471
- 35
Boon A J, Tans J T, Delwel E J et al..
Dutch normal-pressure hydrocephalus study: randomized comparison of low-and medium
pressure shunts.
J Neurosurg.
1998;
88
490-495
- 36
Farace E, Shaffrey M E.
Value of neuropsychological information for improved understanding of the patient
with normal-pressure hydrocephalus.
J Neurosurg.
2005;
102
971-973
- 37
Klinge P, Ruckert N, Schuhmann M, Dorner L, Brinker T, Samii M.
Neuropsychological testing to improve surgical management of patients with chronic
hydrocephalus after shunt treatment.
Acta Neurochir Suppl.
2002;
81
51-53
- 38
Marmarou A, Young H F, Aygok G A et al..
Diagnosis and management of idiopathic normal-pressure hydrocephalus: a prospective
study in 151 patients.
J Neurosurg.
2005;
102
987-997
- 39
Chen I H, Huang C I, Liu H C, Chen K K.
Effectiveness of shunting in patients with normal pressure hydrocephalus predicted
by temporary, controlled-resistance, continuous lumbar drainage: a pilot study.
J Neurol Neurosurg Psychiatry.
1994;
57
1430-1432
- 40
Walchenbach R, Geiger E, Thomeer R T, Vanneste J A.
The value of temporary external lumbar CSF drainage in predicting the outcome of shunting
on normal pressure hydrocephalus.
J Neurol Neurosurg Psychiatry.
2002;
72
503-506
- 41
Meier U, Miethke C.
Predictors of outcome in patients with normal pressure hydrocephalus.
J Clin Neurosci.
2003;
10
453-459
- 42
Marmarou A, Foda M A, Bandoh K et al..
Posttraumatic ventriculomegaly: hydrocephalus or atrophy? A new approach for diagnosis
using CSF dynamics.
J Neurosurg.
1996;
85
1026-1035
- 43
Bergsneider M, Black P, Klinge P, Marmarou A, Relkin N.
Surgical management of idiopathic normal-pressure hydrocephalus.
Neurosurgery.
2005;
57(suppl)
S29-S39
- 44
Vanneste J, Augustijn P, Dirven C, Tan W F, Goedhart Z D.
Shunting normal-pressure hydrocephalus: do the benefits outweigh the risks? A multicenter
study and literature review.
Neurology.
1992;
42
54-59
- 45
Vanneste J A.
Diagnosis and management of normal-pressure hydrocephalus.
J Neurol.
2000;
247
5-14
- 46
Freter S, Bergman H, Gold S, Chertkow H, Clarfield A M.
Prevalence of potentially reversible dementias and actual reversibility in a memory
clinic cohort.
CMAJ.
1998;
159
657-662
- 47
Thomas G, McGirt M J, Woodworth G et al..
Baseline neuropsychological profile and cognitive response to cerebrospinal fluid
shunting for idiopathic normal pressure hydrocephalus.
Dement Geriatr Cogn Disord.
2005;
20
163-168
- 48
Duinkerke A, Williams M A, Rigamonti D, Hillis A E.
Cognitive recovery in idiopathic normal pressure hydrocephalus after shunt.
Cogn Behav Neurol.
2004;
17
179-184
- 49
Kaye J A, Grady C L, Haxby J V, Moore A, Friedland R P.
Plasticity in the aging brain: reversibility of anatomic, metabolic, and cognitive
deficits in normal-pressure hydrocephalus following shunt surgery.
Arch Neurol.
1990;
47
1336-1341
- 50
Klinge P, Marmarou A, Bergsneider M, Relkin N, Black P.
Outcome of shunting in idiopathic normal-pressure hydrocephalus and the value of outcome
assessment in shunted patients.
Neurosurgery.
2005;
57(suppl)
S40-S52
- 51
Savolainen S, Paljarvi L, Vapalahti M.
Prevalence of Alzheimer's disease in patients investigated for presumed normal pressure
hydrocephalus: a clinical and neuropathological study.
Acta Neurochir (Wien).
1999;
141
849-853
- 52
Boon A J, Tans J T, Delwel E J et al..
Dutch normal-pressure hydrocephalus study: the role of cerebrovascular disease.
J Neurosurg.
1999;
90
221-226
- 53
Tans J T, Boon A J.
How to select patients with normal pressure hydrocephalus for shunting.
Acta Neurochir Suppl.
2002;
81
3-5
- 54
Meier U, Konig A, Miethke C.
Predictors of outcome in patients with normal-pressure hydrocephalus.
Eur Neurol.
2004;
51
59-67
- 55
Klinge P, Gerkmann I, Samii A, Brinker T.
Incomplete reversibility of selective hippocampal response after early and delayed
shunting in adult kaolin-hydrocephalus: implications for early shunt treatment.
Cerebrospinal Fluid Res [serial online].
2004;
1(suppl 1)
S33
Available at: http://www.cerebrorpindfhridreserch.com/content/1/S1/S33
- 56
Bekkelund S I, Marthinsen T A, Harr T.
Reversible dementia in idiopathic normal pressure hydrocephalus: a case report.
Scand J Prim Health Care.
1999;
17
22-24
Norman RelkinM.D. Ph.D.
Director, Memory Disorders Program, Department of Neurology and Neuroscience, New
York Presbyterian Hospital-Weill Cornell Medical Center
428 East 72nd Street, Suite 500, New York, NY 10021