Normal Pressure Hydrocephalus

 

 Buy Article Permissions and Reprints

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.

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
  CrossrefPubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 4 Silverberg G D. Normal pressure hydrocephalus (NPH): ischaemia, CSF stagnation or both.  Brain. 2004;  127 947-948
  CrossrefPubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 7 Chakravarty A. Unifying concept for Alzheimer's disease, vascular dementia, and normal pressure hydrocephalus: a hypothesis.  Med Hypotheses. 2004;  63 827-833
  CrossrefPubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 10 Relkin N, Marmarou A, Klinge P, Bergsneider M, Black P M. Diagnosing idiopathic normal-pressure hydrocephalus.  Neurosurgery. 2005;  57(suppl) S4-S16
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 17 Merten T. [Neuropsychology of normal pressure hydrocephalus].  Nervenarzt. 1999;  70 496-503
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 21 Vanneste J AL, Hyman R. Klinische en neuropsychologische kenmerken van normal pressure hydrocephalus.  Ned Tijdschr Geneeskd. 1987;  131 1080-1084
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 29 Alexopoulos G S, Meyers B S, Young R C et al.. The vascular depression hypothesis.  Arch Gen Psychiatry. 1997;  54 915-922
  CrossrefPubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 41 Meier U, Miethke C. Predictors of outcome in patients with normal pressure hydrocephalus.  J Clin Neurosci. 2003;  10 453-459
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 43 Bergsneider M, Black P, Klinge P, Marmarou A, Relkin N. Surgical management of idiopathic normal-pressure hydrocephalus.  Neurosurgery. 2005;  57(suppl) S29-S39
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 45 Vanneste J A. Diagnosis and management of normal-pressure hydrocephalus.  J Neurol. 2000;  247 5-14
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 53 Tans J T, Boon A J. How to select patients with normal pressure hydrocephalus for shunting.  Acta Neurochir Suppl. 2002;  81 3-5
  PubMedGoogle Scholar
• 54 Meier U, Konig A, Miethke C. Predictors of outcome in patients with normal-pressure hydrocephalus.  Eur Neurol. 2004;  51 59-67
  PubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar
• 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
  CrossrefPubMedGoogle Scholar

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