Normal Pressure Hydrocephalus Clinical Presentation

  • Author: Arif I Dalvi, MD; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Oct 28, 2010
 

History

Patients present with a gradually progressive disorder. As noted above, the classic triad consists of abnormal gait, urinary incontinence, and dementia. The gait disturbance is typically the earliest feature noted and considered to be the most responsive to treatment. The primary feature is thought to resemble an apraxia of gait. True weakness or ataxia is typically not observed.

The gait of NPH is characterized as bradykinetic, broad based, magnetic, and shuffling. The urinary symptoms of NPH can present as urinary frequency, urgency, or frank incontinence. While incontinence can result from gait disturbance and dementia, in a study by Sakakibara and colleagues, 95% of patients had urodynamic parameters consistent with detrusor overactivity.[6]

The dementia of NPH is characterized by prominent memory loss and bradyphrenia. Frontal and subcortical deficits are particularly pronounced. Such deficits include forgetfulness, decreased attention, inertia, and bradyphrenia. The presence of cortical signs such as aphasia or agnosia should raise suspicion for an alternate pathology such as Alzheimer disease or vascular dementia. However, comorbid pathology is not uncommon with advancing age. In one study, more than 60% of patients with iNPH had cerebrovascular disease.[7] In another similar study, more than 75% had Alzheimer disease pathology at the time of shunt surgery.[8]

Patients commonly present with a gait disorder and dementia. On neurologic examination, pyramidal tract findings may be present in addition to the above findings.

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Causes

Normal pressure hydrocephalus may occur due to a variety of secondary causes but may be idiopathic in approximately 50% of patients. Secondary causes of NPH include head injury, subarachnoid hemorrhage, meningitis, and CNS tumor. Another potential cause could be previously compensated congenital hydrocephalus.[9]

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Contributor Information and Disclosures
Author

Arif I Dalvi, MD  Director, Movement Disorders Center, NorthShore University HealthSystem, Clinical Associate Professor of Neurology, University of Chicago Pritzker Medical School

Arif I Dalvi, MD is a member of the following medical societies: European Neurological Society and Movement Disorders Society

Disclosure: Nothing to disclose.

Coauthor(s)

Ashvini P Premkumar, MD  Associate Director, Movement Disorders Center, NorthShore University HealthSystem, Clinical Instructor of Neurology, University of Chicago Pritzker Medical School

Ashvini P Premkumar, MD is a member of the following medical societies: American Academy of Neurology and Movement Disorders Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Joseph F Hulihan, MD  Vice President, Medical Affairs, Ortho-McNeil Janssen Scientific Affairs, LLC

Joseph F Hulihan, MD is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society, American Headache Society, and American Medical Association

Disclosure: Johnson & Johnson Salary Employment; Johnson & Johnson Stock Employment

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Nestor Galvez-Jimenez, MD, MSc, MHA  Chairman, Department of Neurology, Program Director, Movement Disorders, Department of Neurology, Division of Medicine, Cleveland Clinic Florida

Nestor Galvez-Jimenez, MD, MSc, MHA is a member of the following medical societies: American Academy of Neurology, American College of Physicians, and Movement Disorders Society

Disclosure: Nothing to disclose.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Chief Editor

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

References
  1. Hakim S, Adams RD. The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics. J Neurol Sci. Jul-Aug 1965;2(4):307-27. [Medline].

  2. Brean A, Eide PK. Prevalence of probable idiopathic normal pressure hydrocephalus in a Norwegian population. Acta Neurol Scand. Jul 2008;118(1):48-53. [Medline].

  3. Hiraoka K, Meguro K, Mori E. Prevalence of idiopathic normal-pressure hydrocephalus in the elderly population of a Japanese rural community. Neurol Med Chir (Tokyo). May 2008;48(5):197-99; discussion 199-200. [Medline].

  4. Tanaka N, Yamaguchi S, Ishikawa H, Ishii H, Meguro K. Prevalence of possible idiopathic normal-pressure hydrocephalus in Japan: the Osaki-Tajiri project. Neuroepidemiology. 2009;32(3):171-5. [Medline].

  5. Marmarou A, Young HF, Aygok GA. Estimated incidence of normal pressure hydrocephalus and shunt outcome in patients residing in assisted-living and extended-care facilities. Neurosurg Focus. Apr 15 2007;22(4):E1. [Medline].

  6. Sakakibara R, Uchiyama T, Kanda T, Uchida Y, Kishi M, Hattori T. [Urinary dysfunction in idiopathic normal pressure hydrocephalus]. Brain Nerve. Mar 2008;60(3):233-9. [Medline].

  7. Bech-Azeddine R, Hogh P, Juhler M, Gjerris F, Waldemar G. Idiopathic normal-pressure hydrocephalus: clinical comorbidity correlated with cerebral biopsy findings and outcome of cerebrospinal fluid shunting. J Neurol Neurosurg Psychiatry. Feb 2007;78(2):157-61. [Medline].

  8. Golomb J, Wisoff J, Miller DC, et al. Alzheimer's disease comorbidity in normal pressure hydrocephalus: prevalence and shunt response. J Neurol Neurosurg Psychiatry. Jun 2000;68(6):778-81. [Medline].

  9. Graff-Radford NR, Godersky JC. Symptomatic congenital hydrocephalus in the elderly simulating normal pressure hydrocephalus. Neurology. Dec 1989;39(12):1596-600. [Medline].

  10. Sasaki M, Honda S, Yuasa T, Iwamura A, Shibata E, Ohba H. Narrow CSF space at high convexity and high midline areas in idiopathic normal pressure hydrocephalus detected by axial and coronal MRI. Neuroradiology. Feb 2008;50(2):117-22. [Medline].

  11. Gyldensted C. Measurements of the normal ventricular system and hemispheric sulci of 100 adults with computed tomography. Neuroradiology. Dec 31 1977;14(4):183-92. [Medline].

  12. Williams MA, Razumovsky AY, Hanley DF. Comparison of Pcsf monitoring and controlled CSF drainage diagnose normal pressure hydrocephalus. Acta Neurochir Suppl. 1998;71:328-30. [Medline].

  13. Governale LS, Fein N, Logsdon J, Black PM. Techniques and complications of external lumbar drainage for normal pressure hydrocephalus. Neurosurgery. Oct 2008;63(4 Suppl 2):379-84; discussion 384. [Medline].

  14. Marmarou A, Young HF, Aygok GA, et al. Diagnosis and management of idiopathic normal-pressure hydrocephalus: a prospective study in 151 patients. J Neurosurg. Jun 2005;102(6):987-97. [Medline].

  15. Murai R, Hashiguchi F, Kusuyama A, et al. Percutaneous stenting for malignant biliary stenosis. Surg Endosc. 1991;5(3):140-2. [Medline].

  16. Walchenbach R, Geiger E, Thomeer RT, Vanneste JA. The value of temporary external lumbar CSF drainage in predicting the outcome of shunting on normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry. Apr 2002;72(4):503-6. [Medline].

  17. Burnett MG, Sonnad SS, Stein SC. Screening tests for normal-pressure hydrocephalus: sensitivity, specificity, and cost. J Neurosurg. Dec 2006;105(6):823-9. [Medline].

  18. Stein SC, Burnett MG, Sonnad SS. Shunts in normal-pressure hydrocephalus: do we place too many or too few?. J Neurosurg. Dec 2006;105(6):815-22. [Medline].

  19. Hebb AO, Cusimano MD. Idiopathic normal pressure hydrocephalus: a systematic review of diagnosis and outcome. Neurosurgery. Nov 2001;49(5):1166-84; discussion 1184-6. [Medline].

  20. Vanneste J, Augustijn P, Davies GA, Dirven C, Tan WF. Normal-pressure hydrocephalus. Is cisternography still useful in selecting patients for a shunt?. Arch Neurol. Apr 1992;49(4):366-70. [Medline].

  21. Aimard G, Vighetto A, Gabet JY, Bret P, Henry E. [Acetazolamide: an alternative to shunting in normal pressure hydrocephalus? Preliminary results]. Rev Neurol (Paris). 1990;146(6-7):437-9. [Medline].

  22. Vanneste J, Augustijn P, Dirven C, Tan WF, Goedhart ZD. Shunting normal-pressure hydrocephalus: do the benefits outweigh the risks? A multicenter study and literature review. Neurology. Jan 1992;42(1):54-9. [Medline].

  23. Boon AJ, Tans JT, Delwel EJ, et al. Dutch Normal-Pressure Hydrocephalus Study: the role of cerebrovascular disease. J Neurosurg. Feb 1999;90(2):221-6. [Medline].

  24. Hamilton R, Patel S, Lee EB, Jackson EM, Lopinto J, Arnold SE. Lack of shunt response in suspected idiopathic normal pressure hydrocephalus with Alzheimer disease pathology. Ann Neurol. Oct 2010;68(4):535-40. [Medline].

  25. Pujari S, Kharkar S, Metellus P, Shuck J, Williams MA, Rigamonti D. Normal pressure hydrocephalus: long-term outcome after shunt surgery. J Neurol Neurosurg Psychiatry. Nov 2008;79(11):1282-6. [Medline].

  26. Hertel F, Zuchner M, Decker C, Schill S, Bosniak I, Bettag M. The Miethke dual switch valve: experience in 169 adult patients with different kinds of hydrocephalus: an open field study. Minim Invasive Neurosurg. Jun 2008;51(3):147-53. [Medline].

  27. Tisell M, Hellstrom P, Ahl-Borjesson G, Barrows G, Blomsterwall E, Tullberg M. Long-term outcome in 109 adult patients operated on for hydrocephalus. Br J Neurosurg. Aug 2006;20(4):214-21. [Medline].

  28. Tsakanikas D, Relkin N. Normal pressure hydrocephalus. Semin Neurol. Feb 2007;27(1):58-65. [Medline].

  29. 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. Dec 2005;252(12):1465-71. [Medline].

  30. Wikkelso C, Andersson H, Blomstrand C, Lindqvist G, Svendsen P. Normal pressure hydrocephalus. Predictive value of the cerebrospinal fluid tap-test. Acta Neurol Scand. Jun 1986;73(6):566-73. [Medline].

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T2-weighted MRI showing dilatation of ventricles out of proportion to sulcal atrophy in a patient with normal pressure hydrocephalus. The arrow points to transependymal flow.
CT head scan of a patient with normal pressure hydrocephalus showing dilated ventricles. The arrow points to a rounded frontal horn.
This image shows ventriculomegaly, which is typical in hydrocephalus ex vacuo.
This image shows cortical atrophy, which is the defining feature of hydrocephalus ex vacuo.
 
 
 
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