Normal Pressure Hydrocephalus Follow-up
- Author: Michael J Schneck, MD, MBA; Chief Editor: Selim R Benbadis, MD more...
The overall prognosis of NPH remains poor both due to a lack of improvement in some patients following surgery as well as a significant complication rate. In a study by Vanneste et al, one of the more comprehensive studies described above, marked improvement was noted in only 21% of patients following shunt surgery. Complication rate was approximately 28% with death or severe residual morbidity in 7% of patients, further emphasizing the importance of careful patient selection. Concomitant cerebrovascular disease is a recognized negative prognostic factor.
In a small prospective study, Hamilton et al measured the impact of cortical Alzheimer disease pathology on shunt responsiveness in 37 individuals treated for idiopathic NPH. Clinical measures, including neuropsychometrics and gait, were correlated with amyloid β (Aβ) plaques, neuritic plaques, and neurofibrillary tangles observed in cortical biopsies obtained during shunt insertion. Patients with no tau and Aβ pathology and mild tau and Aβ pathology improved on the neuropsychometric and gait evaluations. In contrast, patients with moderate-to-severe pathology did not show improvement on any study measure. However, the relatively small numbers in the study, presence of contradictory studies, and absence of a widely accepted biomarker for Alzheimer disease make it difficult to use this finding while evaluating patients with NPH.
In patients who develop recurrent symptoms after initial improvement, shunt malfunction should be suspected and an evaluation for mechanical failure should be pursued. In some of these cases, catheter migration may have occurred, which is a correctable cause of shunt malfunction. In one case series, shunt revision was required in more than half of treated patients over a 6-year period, with improvement in most of these patients.
The incidence of shunt complications is estimated in 30-40% of patients. These include anesthetic complications, intracranial hemorrhage from placement of the ventricular catheter, infection, CSF hypotensive headaches, subdural hematomas, shunt occlusion, and catheter breakage. Rapid reduction in ventricular size following the shunt favors complications such as subdural hematoma, which may occur in 2-17% of patients. Dual-switch valves and programmable valves may reduce the incidence of this complication.
For excellent patient education resources, visit eMedicineHealth's Brain and Nervous System Center. Also, see eMedicineHealth's patient education article Normal Pressure Hydrocephalus.
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].
Hakim S, Adams RD. The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics. J Neurol Sci. 1965 Jul-Aug. 2(4):307-27. [Medline].
Brean A, Eide PK. Prevalence of probable idiopathic normal pressure hydrocephalus in a Norwegian population. Acta Neurol Scand. 2008 Jul. 118(1):48-53. [Medline].
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). 2008 May. 48(5):197-99; discussion 199-200. [Medline].
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].
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. 2007 Apr 15. 22(4):E1. [Medline].
Sakakibara R, Uchiyama T, Kanda T, Uchida Y, Kishi M, Hattori T. [Urinary dysfunction in idiopathic normal pressure hydrocephalus]. Brain Nerve. 2008 Mar. 60(3):233-9. [Medline].
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. 2007 Feb. 78(2):157-61. [Medline].
Golomb J, Wisoff J, Miller DC, et al. Alzheimer's disease comorbidity in normal pressure hydrocephalus: prevalence and shunt response. J Neurol Neurosurg Psychiatry. 2000 Jun. 68(6):778-81. [Medline].
Graff-Radford NR, Godersky JC. Symptomatic congenital hydrocephalus in the elderly simulating normal pressure hydrocephalus. Neurology. 1989 Dec. 39(12):1596-600. [Medline].
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. 2008 Feb. 50(2):117-22. [Medline].
Gyldensted C. Measurements of the normal ventricular system and hemispheric sulci of 100 adults with computed tomography. Neuroradiology. 1977 Dec 31. 14(4):183-92. [Medline].
Singer OC, Melber J, Hattingen E, Jurcoane A, Keil F, Neumann-Haefelin T, et al. MR volumetric changes after diagnostic CSF removal in normal pressure hydrocephalus. J Neurol. 2012 May 17. [Medline].
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].
Governale LS, Fein N, Logsdon J, Black PM. Techniques and complications of external lumbar drainage for normal pressure hydrocephalus. Neurosurgery. 2008 Oct. 63(4 Suppl 2):379-84; discussion 384. [Medline].
Marmarou A, Young HF, Aygok GA, et al. Diagnosis and management of idiopathic normal-pressure hydrocephalus: a prospective study in 151 patients. J Neurosurg. 2005 Jun. 102(6):987-97. [Medline].
Murai R, Hashiguchi F, Kusuyama A, et al. Percutaneous stenting for malignant biliary stenosis. Surg Endosc. 1991. 5(3):140-2. [Medline].
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. 2002 Apr. 72(4):503-6. [Medline].
Burnett MG, Sonnad SS, Stein SC. Screening tests for normal-pressure hydrocephalus: sensitivity, specificity, and cost. J Neurosurg. 2006 Dec. 105(6):823-9. [Medline].
Stein SC, Burnett MG, Sonnad SS. Shunts in normal-pressure hydrocephalus: do we place too many or too few?. J Neurosurg. 2006 Dec. 105(6):815-22. [Medline].
Hebb AO, Cusimano MD. Idiopathic normal pressure hydrocephalus: a systematic review of diagnosis and outcome. Neurosurgery. 2001 Nov. 49(5):1166-84; discussion 1184-6. [Medline].
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. 1992 Apr. 49(4):366-70. [Medline].
Rangel-Castilla L, Barber S, Zhang YJ. The role of endoscopic third ventriculostomy in the treatment of communicating hydrocephalus. World Neurosurg. 2012 Mar. 77(3-4):555-60. [Medline].
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. 1992 Jan. 42(1):54-9. [Medline].
Boon AJ, Tans JT, Delwel EJ, et al. Dutch Normal-Pressure Hydrocephalus Study: the role of cerebrovascular disease. J Neurosurg. 1999 Feb. 90(2):221-6. [Medline].
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. 2010 Oct. 68(4):535-40. [Medline].
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. 2008 Nov. 79(11):1282-6. [Medline].
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. 2008 Jun. 51(3):147-53. [Medline].
Brooks M. CSF Protein a Diagnostic Marker for Idiopathic NPH? Medscape Medical News. July 05, 2013. Available at http://www.medscape.com/viewarticle/807381. Accessed: July 16, 2013.
Nishida N, Nagata N, Toda H, Ishikawa M, Urade Y, Iwasaki K. L-PGDS could be a surrogate marker of frontal lobe dysfunction in idiopathic NPH [abstract 1014]. Available at http://www.mdsabstracts.com/abstract.asp?MeetingID=798&id=107057. Accessed: July 16, 2013.
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. 2006 Aug. 20(4):214-21. [Medline].
Tsakanikas D, Relkin N. Normal pressure hydrocephalus. Semin Neurol. 2007 Feb. 27(1):58-65. [Medline].
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 Dec. 252(12):1465-71. [Medline].
Wikkelso C, Andersson H, Blomstrand C, Lindqvist G, Svendsen P. Normal pressure hydrocephalus. Predictive value of the cerebrospinal fluid tap-test. Acta Neurol Scand. 1986 Jun. 73(6):566-73. [Medline].