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Current Update on Treatment Strategies for Idiopathic Normal Pressure Hydrocephalus

  • Dementia (J Pillai, Section Editor)
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Abstract

Purpose of review

Idiopathic normal pressure hydrocephalus (iNPH) is a surgically treatable neurological disorder of the elderly population that is characterized by abnormal ventricular enlargement due to cerebrospinal fluid (CSF) accumulation and gait disturbance, cognitive impairment, or urinary incontinence. The objective of this review is to present the current diagnostic and treatment approaches for iNPH and to discuss some of the postoperative modalities that complement positive surgical outcomes.

Recent findings

Although historically reported patient outcomes following iNPH surgery were dismal and highly variable, recent advances in terms of better understanding of the iNPH disease process, better standardization of iNPH diagnostic and treatment processes arising from the adoption of clinical guidelines for diagnosis, treatment and in research methodologies, and availability of long-term follow-up data, have helped reduce the variations to a much improved 73 to 96% reported good outcomes.

Summary

With careful evaluation, good patient selection, and advanced surgical techniques, iNPH can be surgically treated to return patients close to their pre-iNPH functional status. Institution of an interdisciplinary effort to rehabilitate patients following surgery may help augment their recovery.

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References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. Rekate HL. A contemporary definition and classification of hydrocephalus. Semin Pediatr Neurol. 2009;16(1):9–15.

    PubMed  Google Scholar 

  2. Kahle KT, Kulkarni AV, Limbrick DD Jr, Warf BC. Hydrocephalus in children. Lancet. 2016;387(10020):788–99.

    PubMed  Google Scholar 

  3. Eide PK. The pathophysiology of chronic noncommunicating hydrocephalus: lessons from continuous intracranial pressure monitoring and ventricular infusion testing. J Neurosurg. 2018;129(1):220–33.

    PubMed  Google Scholar 

  4. Hakim S. Algunas observaciones sobre la presion del L.C.R. Sindrome. Hidrocefalico en el Adulto con "Presion Normal" del L.C.R.(Presentacion de un Nuevo Sindrome). Bogota: Javeriana University; 1964.

    Google Scholar 

  5. 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;2(4):307–27.

    PubMed  CAS  Google Scholar 

  6. Williams MA, Relkin NR. Diagnosis and management of idiopathic normal-pressure hydrocephalus. Neurol Clin Pract. 2013;3(5):375–85.

    PubMed  PubMed Central  Google Scholar 

  7. Malm J, Eklund A. Idiopathic normal pressure hydrocephalus. Pract Neurol. 2006;6(1):14–27.

    Google Scholar 

  8. Williams MA, Nagel SJ, Luciano MG, Relkin N, Zwimpfer TJ, Katzen H, et al. The clinical spectrum of hydrocephalus in adults: report of the first 517 patients of the adult hydrocephalus clinical research network registry. J Neurosurg. 2019:1–12.

  9. Relkin N, Marmarou A, Klinge P, Bergsneider M, Black PM. INPH guidelines, part II: diagnosing idio-pathic normal-pressure hydrocephalus. Neurosurgery. 2005;57(3 SUPPL):S2-4–S2-16.

    Google Scholar 

  10. Mori E, Ishikawa M, Kato T, Kazui H, Miyake H, Miyajima M, et al. Guidelines for management of idiopathic normal pressure hydrocephalus: second edition. Neurol Med Chir (Tokyo). 2012;52(11):775–809.

    Google Scholar 

  11. Kuriyama N, Miyajima M, Nakajima M, Kurosawa M, Fukushima W, Watanabe Y, et al. Nationwide hospital-based survey of idiopathic normal pressure hydrocephalus in Japan: epidemiological and clinical characteristics. Brain Behav. 2017;7(3):e00635.

    PubMed  PubMed Central  Google Scholar 

  12. Jaraj D, Rabiei K, Marlow T, Jensen C, Skoog I, Wikkelso C. Prevalence of idiopathic normal-pressure hydrocephalus. Neurology. 2014;82(16):1449–54.

    PubMed  PubMed Central  Google Scholar 

  13. Isaacs AM, Riva-Cambrin J, Yavin D, Hockley A, Pringsheim TM, Jette N, et al. Age-specific global epidemiology of hydrocephalus: systematic review, metanalysis and global birth surveillance. PLoS One. 2018;13(10):e0204926. Isaacs, et al reported a comprehensive systematic review and meta-analysis that defines the prevalence of iNPH as 175/100,000. The prevalence increases with age to 5,900/100,000 for those >85 years old.

    PubMed  PubMed Central  Google Scholar 

  14. Williams MA, McAllister JP, Walker ML, Kranz DA, Bergsneider M, Del Bigio MR, et al. Priorities for hydrocephalus research: report from a National Institutes of Health-sponsored workshop. J Neurosurg. 2007;107(5 Suppl):345–57.

    PubMed  Google Scholar 

  15. Graff-Radford NR, Godersky JC. Symptomatic congenital hydrocephalus in the elderly simulating normal pressure hydrocephalus. Neurology. 1989;39(12):1596–600.

    PubMed  CAS  Google Scholar 

  16. Krauss JK, Regel JP, Vach W, Droste DW, Borremans JJ, Mergner T. Vascular risk factors and arteriosclerotic disease in idiopathic normal-pressure hydrocephalus of the elderly. Stroke. 1996;27(1):24–9.

    PubMed  CAS  Google Scholar 

  17. Jaraj D, Agerskov S, Rabiei K, Marlow T, Jensen C, Guo X, et al. Vascular factors in suspected normal pressure hydrocephalus: a population-based study. Neurology. 2016;86(7):592–9.

    PubMed  PubMed Central  Google Scholar 

  18. Bech RA, Waldemar G, Gjerris F, Klinken L, Juhler M. Shunting effects in patients with idiopathic normal pressure hydrocephalus; correlation with cerebral and leptomeningeal biopsy findings. Acta Neurochir. 1999;141(6):633–9.

    PubMed  CAS  Google Scholar 

  19. Bech RA, Juhler M, Waldemar G, Klinken L, Gjerris F. Frontal brain and leptomeningeal biopsy specimens correlated with cerebrospinal fluid outflow resistance and B-wave activity in patients suspected of normal-pressure hydrocephalus. Neurosurgery. 1997;40(3):497–502.

    PubMed  CAS  Google Scholar 

  20. Malm J, Graff-Radford NR, Ishikawa M, Kristensen B, Leinonen V, Mori E, et al. Influence of comorbidities in idiopathic normal pressure hydrocephalus - research and clinical care. A report of the ISHCSF task force on comorbidities in INPH. Fluids Barriers CNS. 2013;10(1):22.

    PubMed  PubMed Central  Google Scholar 

  21. Mocco J, Tomey MI, Komotar RJ, Mack WJ, Frucht SJ, Goodman RR, et al. Ventriculoperitoneal shunting of idiopathic normal pressure hydrocephalus increases midbrain size: a potential mechanism for gait improvement. Neurosurgery. 2006;59(4):847–50 discussion 50-1.

    PubMed  CAS  Google Scholar 

  22. Del Bigio MR, Di Curzio DL. Nonsurgical therapy for hydrocephalus: a comprehensive and critical review. Fluids Barriers CNS. 2016;13(1):3.

    PubMed  PubMed Central  Google Scholar 

  23. Williams MA, Malm J. Diagnosis and treatment of idiopathic normal pressure hydrocephalus. Continuum (Minneap Minn). 2016;22(2 Dementia):579–99.

    PubMed  Google Scholar 

  24. Marmarou A, Bergsneider M, Relkin N, Klinge P, Black PM. Development of guidelines for idiopathic normal-pressure hydrocephalus: introduction. Neurosurgery. 2005;57(3 Suppl):S1–3; discussion ii-v.

    Google Scholar 

  25. Nutt JG. Higher-level gait disorders: an open frontier. Mov Disord. 2013;28(11):1560–5.

    PubMed  Google Scholar 

  26. Nutt JG, Marsden CD, Thompson PD. Human walking and higher-level gait disorders, particularly in the elderly. Neurology. 1993;43(2):268–79.

    PubMed  CAS  Google Scholar 

  27. Boon AJ, Tans JT, Delwel EJ, Egeler-Peerdeman SM, Hanlo PW, Wurzer HA, et al. Dutch normal-pressure hydrocephalus study: prediction of outcome after shunting by resistance to outflow of cerebrospinal fluid. J Neurosurg. 1997;87(5):687–93.

    PubMed  CAS  Google Scholar 

  28. Tinetti ME. Performance-oriented assessment of mobility problems in elderly patients. J Am Geriatr Soc. 1986;34(2):119–26.

    PubMed  CAS  Google Scholar 

  29. Podsiadlo D, Richardson S. The timed "up & go": a test of basic functional mobility for frail elderly persons. J Am Geriatr Soc. 1991;39(2):142–8.

    PubMed  CAS  Google Scholar 

  30. Graham JE, Ostir GV, Fisher SR, Ottenbacher KJ. Assessing walking speed in clinical research: a systematic review. J Eval Clin Pract. 2008;14(4):552–62.

    PubMed  PubMed Central  Google Scholar 

  31. Relkin N, Marmarou A, Klinge P, Bergsneider M, Black PM. Diagnosing idiopathic normal-pressure hydrocephalus. Neurosurgery. 2005;57(3 Suppl):S4–16; discussion ii-v.

    Google Scholar 

  32. Picascia M, Zangaglia R, Bernini S, Minafra B, Sinforiani E, Pacchetti C. A review of cognitive impairment and differential diagnosis in idiopathic normal pressure hydrocephalus. Funct Neurol. 2015;30(4):217–29.

    PubMed  Google Scholar 

  33. Saito M, Nishio Y, Kanno S, Uchiyama M, Hayashi A, Takagi M, et al. Cognitive profile of idiopathic normal pressure hydrocephalus. Dement Geriatr Cogn Dis Extra. 2011;1(1):202–11.

    PubMed  PubMed Central  Google Scholar 

  34. Nasreddine ZS, Phillips NA, Bedirian V, Charbonneau S, Whitehead V, Collin I, et al. The Montreal Cognitive Assessment, MoCA: a brief screening tool for mild cognitive impairment. J Am Geriatr Soc. 2005;53(4):695–9.

    Google Scholar 

  35. Israelsson H, Allard P, Eklund A, Malm J. Symptoms of depression are common in patients with idiopathic normal pressure hydrocephalus: the INPH-CRasH study. Neurosurgery. 2016;78(2):161–8.

    PubMed  Google Scholar 

  36. Klausner AP, Ellen EF, Collins CW, Marmarou A, Young HF, Boling PA, et al. Characterization of urinary incontinence in patients with normal pressure hydrocephalus (NPH). Journal of Urology. 2008;179(4):353-.

    Google Scholar 

  37. Virhammar J, Laurell K, Cesarini KG, Larsson EM. The callosal angle measured on MRI as a predictor of outcome in idiopathic normal-pressure hydrocephalus. J Neurosurg. 2014;120(1):178–84.

    PubMed  Google Scholar 

  38. Reinard K, Basheer A, Phillips S, Snyder A, Agarwal A, Jafari-Khouzani K, et al. Simple and reproducible linear measurements to determine ventricular enlargement in adults. Surg Neurol Int. 2015;6:59.

    PubMed  PubMed Central  Google Scholar 

  39. Jaraj D, Rabiei K, Marlow T, Jensen C, Skoog I, Wikkelso C. Estimated ventricle size using Evans index: reference values from a population-based sample. Eur J Neurol. 2017;24(3):468–74.

    PubMed  CAS  Google Scholar 

  40. Hashimoto M, Ishikawa M, Mori E, Kuwana N, Study of Ioni. Diagnosis of idiopathic normal pressure hydrocephalus is supported by MRI-based scheme: a prospective cohort study. Cerebrospinal Fluid Res. 2010;7(1):18.

  41. Kazui H, Miyajima M, Mori E, Ishikawa M, Investigators S-. Lumboperitoneal shunt surgery for idiopathic normal pressure hydrocephalus (SINPHONI-2): an open-label randomised trial. Lancet Neurol. 2015;14(6):585–94. Kazui, et al reported a prospective multicenter randomized clinical trial (RCT) of 93 patients with iNPH and DESH. 44/93 had shunt surgery delayed by 3 months. 65% of shunted patients improved at 3 months compared with 5% of delayed patients. Improvement was equivalent for both groups at 12 months. This RCT used clinical and radiological criteria without a tap test and clearly demonstrated benefit from shunt surgery. The overall lower treatment outcome success reflects using clinical and radiological criteria without a tap test which decreased the sensitivity of the patient selection process.

  42. Ishikawa M, Oowaki H, Takezawa M, Takenaka T, Yamada S, Yamamoto K, et al. Disproportionately enlarged subarachnoid space hydrocephalus in idiopathic normal-pressure hydrocephalus and its implication in pathogenesis. Acta Neurochir Suppl. 2016;122:287–90.

    PubMed  Google Scholar 

  43. Ahmed AK, Luciano M, Moghekar A, Shin J, Aygun N, Sair HI, et al. Does the presence or absence of DESH predict outcomes in adult hydrocephalus? AJNR Am J Neuroradiol. 2018;39(11):2022–6.

    PubMed  CAS  Google Scholar 

  44. Graff-Radford NR, Godersky JC. Idiopathic normal pressure hydrocephalus and systemic hypertension. Neurology. 1987;37(5):868–71.

    PubMed  CAS  Google Scholar 

  45. Casmiro M, D'Alessandro R, Cacciatore FM, 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(7):847–52.

    PubMed  PubMed Central  CAS  Google Scholar 

  46. Krauss JK, Droste DW, Vach W, Regel JP, Orszagh M, Borremans JJ, et al. Cerebrospinal fluid shunting in idiopathic normal-pressure hydrocephalus of the elderly: effect of periventricular and deep white matter lesions. Neurosurgery. 1996;39(2):292–9 discussion 9-300.

    PubMed  CAS  Google Scholar 

  47. Jacobs L. Diabetes mellitus in normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry. 1977;40(4):331–5.

    PubMed  PubMed Central  CAS  Google Scholar 

  48. Andren K, Wikkelso C, Sundstrom N, Agerskov S, Israelsson H, Laurell K, et al. Long-term effects of complications and vascular comorbidity in idiopathic normal pressure hydrocephalus: a quality registry study. J Neurol. 2018;265(1):178–86.

    PubMed  Google Scholar 

  49. Israelsson H, Carlberg B, Wikkelsö C, Laurell K, Kahlon B, Eklund A, et al. Cardiovascular risk factors are associated with idiopathic normal pressure hydrocephalus. Fluids and Barriers of the CNS. 2015;12(Suppl 1):O41.

    PubMed Central  Google Scholar 

  50. Vaes B, Pasquet A, Wallemacq P, Rezzoug N, Mekouar H, Olivier PA, et al. The BELFRAIL (BFC80+) study: a population-based prospective cohort study of the very elderly in Belgium. BMC Geriatr. 2010;10:39.

    PubMed  PubMed Central  Google Scholar 

  51. Isaacs AM, Hamilton MG, Williams MA. Hydrocephalus in the elderly: Diagnosis of idiopathic normal pressure hydrocephalus. Brain and Spine Surgery in the Elderly2017. p. 455–467.

  52. Isaacs AM, Hamilton MG, Williams MA. Idiopathic normal pressure hydrocephalus. In: Limbrick Jr DD, Leonard JR, editors. Cerebrospinal fluid disorders : lifelong implications. Cham: Springer International Publishing; 2019. p. 219–35.

    Google Scholar 

  53. Robert-Ebadi H, Le Gal G, Righini M. Use of anticoagulants in elderly patients: practical recommendations. Clin Interv Aging. 2009;4:165–77.

    PubMed  PubMed Central  CAS  Google Scholar 

  54. Spyropoulos AC, Merli G. Management of venous thromboembolism in the elderly. Drugs Aging. 2006;23(8):651–71.

    PubMed  CAS  Google Scholar 

  55. Hamilton MG, Golfinos J, Pineo G, Couldwell WT. Handbook of bleeding and coagulation for neurosurgery. 1st New York: Thieme; 2015.

  56. Goodwin CR, Kharkar S, Wang P, Pujari S, Rigamonti D, Williams MA. Evaluation and treatment of patients with suspected normal pressure hydrocephalus on long-term warfarin anticoagulation therapy. Neurosurgery. 2007;60(3):497–501 discussion 2.

    PubMed  Google Scholar 

  57. Lip GYHaDJD. Perioperative management of patients receiving anticoagulants In: UpToDate, Post TW (Ed). Accessed on June 5, 2016 ed: UpToDate; 2016.

  58. Ishikawa M. Guideline Committe for Idiopathic Normal Pressure Hydrocephalus JSoNPH. Clinical guidelines for idiopathic normal pressure hydrocephalus. Neurol Med Chir (Tokyo). 2004;44(4):222–3.

    Google Scholar 

  59. Ishikawa M, Hashimoto M, Kuwana N, Mori E, Miyake H, Wachi A, et al. Guidelines for management of idiopathic normal pressure hydrocephalus: Guidelines from the Guidelines committee of idiopathic normal pressure hydrocephalus, the Japanese society of normal pressure hydrocephalus. Neurologia Medico-Chirurgica. 2008;48(SUPPL):S1–S23.

    PubMed  Google Scholar 

  60. Halperin JJ, Kurlan R, Schwalb JM, Cusimano MD, Gronseth G, Gloss D. Practice guideline: idiopathic normal pressure hydrocephalus: response to shunting and predictors of response: report of the guideline development, dissemination, and implementation Subcommittee of the American Academy of neurology. Neurology. 2015;85(23):2063–71.

    PubMed  PubMed Central  Google Scholar 

  61. Sackett DL. Rules of evidence and clinical recommendations on the use of antithrombotic agents. Chest. 1989;95(2):2S–4S.

    PubMed  CAS  Google Scholar 

  62. Tarnaris A, Williams MA. Idiopathic Normal pressure hydrocephalus: update and practical approach on diagnosis and management. Neurosurg Q. 2011;21(1):72–81.

    Google Scholar 

  63. Ishikawa M, Hashimoto M, Mori E, Kuwana N, Kazui H. The value of the cerebrospinal fluid tap test for predicting shunt effectiveness in idiopathic normal pressure hydrocephalus. Fluids Barriers CNS. 2012;9(1):1.

    PubMed  PubMed Central  Google Scholar 

  64. Ishikawa MaHMaKNaMEaMHaWAaTTaKHaKH. Guidelines for management of idiopathic normal pressure hydrocephalus. 2008.

    Google Scholar 

  65. Thoennissen J, Herkner H, Lang W, Domanovits H, Laggner AN, Mullner M. Does bed rest after cervical or lumbar puncture prevent headache? A systematic review and meta-analysis. Can Med Assoc J. 2001;165(10):1311–6.

    CAS  Google Scholar 

  66. Arevalo-Rodriguez I, Ciapponi A, Figuls MRI, Munoz L, Cosp XB. Posture and fluids for preventing post-dural puncture headache. Cochrane Database Syst Rev. 2016;3.

  67. Virhammar J, Cesarini KG, Laurell K. The CSF tap test in normal pressure hydrocephalus: evaluation time, reliability and the influence of pain. Eur J Neurol. 2012;19(2):271–6.

    PubMed  CAS  Google Scholar 

  68. Mahr CV, Dengl M, Nestler U, Reiss-Zimmermann M, Eichner G, Preuss M, et al. Idiopathic normal pressure hydrocephalus: diagnostic and predictive value of clinical testing, lumbar drainage, and CSF dynamics. J Neurosurg. 2016;125(3):591–7.

    PubMed  CAS  Google Scholar 

  69. Wikkelso C, Hellstrom P, Klinge PM, Tans JT. European i NPHMSG. The European iNPH multicentre study on the predictive values of resistance to CSF outflow and the CSF tap test in patients with idiopathic normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry. 2013;84(5):562–8.

    PubMed  Google Scholar 

  70. Tullberg M, Persson J, Petersen J, Hellstrom P, Wikkelso C, Lundgren-Nilsson A. Shunt surgery in idiopathic normal pressure hydrocephalus is cost-effective-a cost utility analysis. Acta Neurochir. 2018;160(3):509–18.

    PubMed  Google Scholar 

  71. Greenberg BM, Williams MA. Infectious complications of temporary spinal catheter insertion for diagnosis of adult hydrocephalus and idiopathic intracranial hypertension. Neurosurgery. 2008;62(2):431–5 discussion 5-6.

    PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  73. Kahlon B, Sundbarg G, Rehncrona S. Comparison between the lumbar infusion and CSF tap tests to predict outcome after shunt surgery in suspected normal pressure hydrocephalus. Journal of Neurology Neurosurgery and Psychiatry. 2002;73(6):721–6.

    CAS  Google Scholar 

  74. Hussey F, Schanzer B, Katzman R. A simple constant-infusion manometric test for measurement of CSF absorption. II Clinical studies Neurology. 1970;20(7):665–80.

    PubMed  CAS  Google Scholar 

  75. Eklund A, Smielewski P, Chambers I, Alperin N, Malm J, Czosnyka M, et al. Assessment of cerebrospinal fluid outflow resistance. Med Biol Eng Comput. 2007;45(8):719–35.

    PubMed  Google Scholar 

  76. Kahlon B, Sundbarg G, Rehncrona S. Lumbar infusion test in normal pressure hydrocephalus. Acta Neurol Scand. 2005;111(6):379–84.

    PubMed  CAS  Google Scholar 

  77. Qvarlander S, Lundkvist B, Koskinen LO, Malm J, Eklund A. Pulsatility in CSF dynamics: pathophysiology of idiopathic normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry. 2013;84(7):735–41.

    PubMed  Google Scholar 

  78. Malm J, Sundstrom N, Cesarini KG, Edsbagge M, Kristensen B, Leijon G, et al. Implementation of a new CSF dynamic device: a multicenter feasibility study in 562 patients. Acta Neurol Scand. 2012;125(3):199–205.

    PubMed  CAS  Google Scholar 

  79. Malm J, Jacobsson J, Birgander R, Eklund A. Reference values for CSF outflow resistance and intracranial pressure in healthy elderly. Neurology. 2011;76(10):903–9.

    PubMed  Google Scholar 

  80. Jacobsson J, Qvarlander S, Eklund A, Malm J. Comparison of the CSF dynamics between patients with idiopathic normal pressure hydrocephalus and healthy volunteers. J Neurosurg. 2018:1–6.

  81. Malm J, Kristensen B, Karlsson T, Fagerlund M, Elfverson J, Ekstedt J. The predictive value of cerebrospinal fluid dynamic tests in patients with th idiopathic adult hydrocephalus syndrome. Arch Neurol. 1995;52(8):783–9.

    PubMed  CAS  Google Scholar 

  82. Malm J, Kristensen B, Fagerlund M, Koskinen LO, Ekstedt J. Cerebrospinal fluid shunt dynamics in patients with idiopathic adult hydrocephalus syndrome. J Neurol Neurosurg Psychiatry. 1995;58(6):715–23.

    PubMed  PubMed Central  CAS  Google Scholar 

  83. Iseki C, Kawanami T, Nagasawa H, Wada M, Koyama S, Kikuchi K, et al. Asymptomatic ventriculomegaly with features of idiopathic normal pressure hydrocephalus on MRI (AVIM) in the elderly: a prospective study in a Japanese population. J Neurol Sci. 2009;277(1–2):54–7.

    PubMed  Google Scholar 

  84. Iseki C, Takahashi Y, Wada M, Kawanami T, Adachi M, Kato T. Incidence of idiopathic normal pressure hydrocephalus (iNPH): a 10-year follow-up study of a rural community in Japan. J Neurol Sci. 2014;339(1–2):108–12.

    PubMed  Google Scholar 

  85. Andren K, Wikkelso C, Tisell M, Hellstrom P. Natural course of idiopathic normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry. 2014;85(7):806–10.

    PubMed  Google Scholar 

  86. Toma AK, Stapleton S, Papadopoulos MC, Kitchen ND, Watkins LD. Natural history of idiopathic normal-pressure hydrocephalus. Neurosurg Rev. 2011;34(4):433–9.

    PubMed  Google Scholar 

  87. Razay G, Vreugdenhil A, Liddell J. A prospective study of ventriculo-peritoneal shunting for idiopathic normal pressure hydrocephalus. J Clin Neurosci. 2009;16(9):1180–3.

    PubMed  Google Scholar 

  88. Isaacs AM, Williams MA, Hamilton MG. Hydrocephalus in the elderly: Surgical management of idiopathic normal pressure hydrocephalus. Brain and Spine Surgery in the Elderly2017. p. 469–500.

  89. Serarslan Y, Yilmaz A, Cakir M, Guzel E, Akakin A, Guzel A, et al. Use of programmable versus nonprogrammable shunts in the management of normal pressure hydrocephalus: a multicenter retrospective study with cost-benefit analysis in Turkey. Medicine (Baltimore). 2017;96(39):e8185.

    Google Scholar 

  90. Meier U, Mutze S. Correlation between decreased ventricular size and positive clinical outcome following shunt placement in patients with normal-pressure hydrocephalus. J Neurosurg. 2004;100(6):1036–40.

    PubMed  Google Scholar 

  91. Huyette DR, Turnbow BJ, Kaufman C, Vaslow DF, Whiting BB, Oh MY. Accuracy of the freehand pass technique for ventriculostomy catheter placement: retrospective assessment using computed tomography scans. J Neurosurg. 2008;108(1):88–91.

    PubMed  Google Scholar 

  92. Garell PC, Mirsky R, Noh MD, Loftus CM, Hitchon PW, Grady MS, et al. Posterior ventricular catheter burr-hole localizer. Technical note. J Neurosurg. 1998;89(1):157–60.

    PubMed  CAS  Google Scholar 

  93. Zanaty M, Banu M, Flouty O, Grady S, Holland MT. Isaacs A, et al. A Cranial Midline Localizing Device. World Neurosurg: The Wishbone; 2019.

    Google Scholar 

  94. Ghajar JB. A guide for ventricular catheter placement. Technical note J Neurosurg. 1985;63(6):985–6.

    PubMed  CAS  Google Scholar 

  95. Azeem SS, Origitano TC. Ventricular catheter placement with a frameless neuronavigational system: a 1-year experience. Neurosurgery. 2007;60(4 Suppl 2):243–7 discussion 7-8.

    PubMed  Google Scholar 

  96. Jung N, Kim D. Effect of electromagnetic navigated ventriculoperitoneal shunt placement on failure rates. J Korean Neurosurg Soc. 2013;53(3):150–4.

    PubMed  PubMed Central  Google Scholar 

  97. Hayhurst C, Beems T, Jenkinson MD, Byrne P, Clark S, Kandasamy J, et al. Effect of electromagnetic-navigated shunt placement on failure rates: a prospective multicenter study. J Neurosurg. 2010;113(6):1273–8.

    PubMed  Google Scholar 

  98. Toma AK, Papadopoulos MC, Stapleton S, Kitchen ND, Watkins LD. Systematic review of the outcome of shunt surgery in idiopathic normal-pressure hydrocephalus. Acta Neurochir. 2013;155(10):1977–80.

    PubMed  Google Scholar 

  99. Miyajima M, Kazui H, Mori E, Ishikawa M. Sinphoni-investigators obot. One-year outcome in patients with idiopathic normal-pressure hydrocephalus: comparison of lumboperitoneal shunt to ventriculoperitoneal shunt. J Neurosurg. 2016;125(6):1483–92.

    PubMed  Google Scholar 

  100. Eide PK, Sorteberg W. Outcome of surgery for idiopathic normal pressure hydrocephalus: role of preoperative static and pulsatile intracranial pressure. World Neurosurg. 2016;86(C):186–93 e1.

    PubMed  Google Scholar 

  101. Golz L, Ruppert FH, Meier U, Lemcke J. Outcome of modern shunt therapy in patients with idiopathic normal pressure hydrocephalus 6 years postoperatively. J Neurosurg. 2014;121(4):771–5.

    PubMed  Google Scholar 

  102. Bergsneider M, Black PM, Klinge P, Marmarou A, Relkin N. Surgical management of idiopathic normal-pressure hydrocephalus. Neurosurgery. 2005;57(3 Suppl):S29–39; discussion ii-v.

    Google Scholar 

  103. Kubo Y, Kazui H, Yoshida T, Kito Y, Kimura N, Tokunaga H, et al. Validation of grading scale for evaluating symptoms of idiopathic normal-pressure hydrocephalus. Dement Geriatr Cogn Disord. 2008;25(1):37–45.

    PubMed  Google Scholar 

  104. Raysi Dehcordi S, De Tommasi C, Ricci A, Marzi S, Ruscitti C, Amicucci G, et al. Laparoscopy-assisted ventriculoperitoneal shunt surgery: personal experience and review of the literature. Neurosurg Rev. 2011;34(3):363–70 discussion 70-1.

    PubMed  Google Scholar 

  105. Schucht P, Banz V, Trochsler M, Iff S, Krahenbuhl AK, Reinert M, et al. Laparoscopically assisted ventriculoperitoneal shunt placement: a prospective randomized controlled trial. J Neurosurg. 2015;122(5):1058–67. Schucht, et al, reported a prospective multicenter RCT of 120 adult patients with hydrocephalus undergoing VP shunt surgery with randomization to laparoscopic (no distal shunt failures) or conventional mini-laparotomy (8% distal shunt failures) for peritoneal catheter insertion. Patients in the laparoscopic group had no distal shunt failures compared with 8% of the mini-laparotomy, although overall shunt failure was the same. This paper demonstrated the feasibility of RCTs to evaluate possible improvements for shunt surgery and a need to focus on all aspects of the operation.

  106. Isaacs AM, Ball C, Urbaneja G, Holubkov R, Hamilton MG. Laparoscopic-guided distal ventriculoperitoneal shunt insertion improves shunt outcome in adult patients: results of a cohort study with 222 patients experiencing 268 shunt operations. Fluids and Barriers of the CNS. 2018;15(Suppl 1):A63.

    Google Scholar 

  107. Svoboda SM, Park H, Naff N, Dorai Z, Williams MA, Youssef Y. Preventing distal catheter obstruction in laparoscopic ventriculoperitoneal shunt placement in adults: the "Falciform technique". J Laparoendosc Adv Surg Tech A. 2015;25(8):642–5.

    PubMed  Google Scholar 

  108. Epstein N, Epstein F, Trehan N. Percutaneous placement of the atrial end of a vascular shunt utilizing the Swan-Ganz introducer. Neurosurgery. 1981;9(5):564–5.

    PubMed  CAS  Google Scholar 

  109. Tomita T. Placement of a ventriculoatrial shunt using external jugular catheterization: technical note. Neurosurgery. 1984;14(1):74–5.

    PubMed  CAS  Google Scholar 

  110. Hung AL, Vivas-Buitrago T, Adam A, Lu J, Robison J, Elder BD, et al. Ventriculoatrial versus ventriculoperitoneal shunt complications in idiopathic normal pressure hydrocephalus. Clin Neurol Neurosurg. 2017;157:1–6.

    PubMed  Google Scholar 

  111. Girotti ME, Singh RR, Rodgers BM. The ventriculo-gallbladder shunt in the treatment of refractory hydrocephalus: a review of the current literature. Am Surg. 2009;75(8):734–7.

    PubMed  Google Scholar 

  112. Szajer J, Russo R, Mansberg R. Ventriculopleural shunt dysfunction due to a Loculated pleural collection demonstrated on SPECT/CT imaging. Clin Nucl Med. 2018;43(2):144–6.

    PubMed  Google Scholar 

  113. Willison CD, Kopitnik TA, Gustafson R, Kaufman HH. Ventriculopleural shunting used as a temporary diversion. Acta Neurochir. 1992;115(1–2):67–8.

    PubMed  CAS  Google Scholar 

  114. Chuang HL, Chang CN, Hsu JC. Minimally invasive procedure for ventriculoatrial shunt-combining a percutaneous approach with real-time transesophageal echocardiogram monitoring: report of six cases. Chang Gung Med J. 2002;25(1):62–6.

    PubMed  Google Scholar 

  115. McGovern RA, Kelly KM, Chan AK, Morrissey NJ, McKhann GM 2nd. Should ventriculoatrial shunting be the procedure of choice for normal-pressure hydrocephalus? J Neurosurg. 2014;120(6):1458–64.

    PubMed  Google Scholar 

  116. Hamilton MG, Fung A, Lam-Li D, Isaacs AM, Conly J. Development and application of a surgical site infection prevention bundle for shunt-related insertions and revisions. Fluids and Barriers of the CNS. 2018;15(Suppl 1):A54.

    Google Scholar 

  117. Machinis TG, Fountas KN, Hudson J, Robinson JS, Troup EC. Accurate placement of the distal end of a ventriculoatrial shunt with the aid of real-time transesophageal echocardiography. Technical note. J Neurosurg. 2006;105(1):153–6.

    PubMed  Google Scholar 

  118. Hamilton MG, Isaacs AM, Urbaneja G, Krahn D, Rogan K, Walker A, et al. Ventriculo-atrial shunt insertion using transesophageal echo: initial experience. Fluids and Barriers of the CNS. 2018;15(S1):A53.

    Google Scholar 

  119. Gangemi M, Maiuri F, Naddeo M, Godano U, Mascari C, Broggi G, et al. Endoscopic third ventriculostomy in idiopathic normal pressure hydrocephalus: an Italian multicenter study. Neurosurgery. 2008;63(1):62–7 discussion 7-9.

    PubMed  Google Scholar 

  120. Longatti PL, Fiorindi A, Martinuzzi A. Failure of endoscopic third ventriculostomy in the treatment of idiopathic normal pressure hydrocephalus. Minim Invasive Neurosurg. 2004;47(6):342–5.

    PubMed  CAS  Google Scholar 

  121. Tudor KI, Tudor M, McCleery J, Car J. Endoscopic third ventriculostomy (ETV) for idiopathic normal pressure hydrocephalus (iNPH). Cochrane Database Syst Rev. 2015;7(7):CD010033.

    Google Scholar 

  122. Benzel EC, Pelletier AL, Levy PG. Communicating hydrocephalus in adults: prediction of outcome after ventricular shunting procedures. Neurosurgery. 1990;26(4):655–60.

    PubMed  CAS  Google Scholar 

  123. Raftopoulos C, Massager N, Baleriaux D, Deleval J, Clarysse S, Brotchi J. Prospective analysis by computed tomography and long-term outcome of 23 adult patients with chronic idiopathic hydrocephalus. Neurosurgery. 1996;38(1):51–9.

    PubMed  CAS  Google Scholar 

  124. Kahlon B, Sjunnesson J, Rehncrona S. Long-term outcome in patients with suspected normal pressure hydrocephalus. Neurosurgery. 2007;60(2):327–32 discussion 32.

    PubMed  Google Scholar 

  125. Shaw R, Mahant N, Jacobson E, Owler B. A review of clinical outcomes for gait and other variables in the surgical treatment of idiopathic normal pressure hydrocephalus. Movement Disorders Clinical Practice. 2016;3(4):331–41.

    PubMed  PubMed Central  Google Scholar 

  126. 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;79(11):1282–6.

    PubMed  CAS  Google Scholar 

  127. Peterson KA, Housden CR, Killikelly C, DeVito EE, Keong NC, Savulich G, et al. Apathy, ventriculomegaly and neurocognitive improvement following shunt surgery in normal pressure hydrocephalus. Br J Neurosurg. 2016;30(1):38–42.

    PubMed  Google Scholar 

  128. Shaw R, Everingham E, Mahant N, Jacobson E, Owler B. Clinical outcomes in the surgical treatment of idiopathic normal pressure hydrocephalus. J Clin Neurosci. 2016;29:81–6.

    PubMed  Google Scholar 

  129. Klinge P, Hellstrom P, Tans J, Wikkelso C. European i NPHMSG. One-year outcome in the European multicentre study on iNPH. Acta Neurol Scand. 2012;126(3):145–53.

    PubMed  CAS  Google Scholar 

  130. Klinge P, Marmarou A, Bergsneider M, Relkin N, Black PM. Outcome of shunting in idiopathic normal-pressure hydrocephalus and the value of outcome assessment in shunted patients. Neurosurgery. 2005;57(3 Suppl):S40–52; discussion ii-v.

    Google Scholar 

  131. Marmarou A, Young HF, Aygok GA, Sawauchi S, Tsuji O, Yamamoto T, et al. Diagnosis and management of idiopathic normal-pressure hydrocephalus: a prospective study in 151 patients. J Neurosurg. 2005;102(6):987–97.

    PubMed  Google Scholar 

  132. Ferrazzoli D, Ortelli P, Zivi I, Cian V, Urso E, Ghilardi MF, et al. Efficacy of intensive multidisciplinary rehabilitation in Parkinson's disease: a randomised controlled study. J Neurol Neurosurg Psychiatry. 2018;89(8):828–35.

    PubMed  PubMed Central  Google Scholar 

  133. Pitkala KH, Raivio MM, Laakkonen ML, Tilvis RS, Kautiainen H, Strandberg TE. Exercise rehabilitation on home-dwelling patients with Alzheimer's disease--a randomized, controlled trial. Study protocol Trials. 2010;11:92.

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neuroscience, Washington University School of Medicine, St. Louis, MO, USA

    Albert M. Isaacs MD

  2. Division of Neurosurgery, Department of Clinical Neuroscience, University of Calgary, Calgary, Alberta, Canada

    Albert M. Isaacs MD & Mark G. Hamilton MD, CM, FRCSC, FAANS

  3. Adult and Transitional Hydrocephalus and CSF Disorders, Departments of Neurology and Neurological Surgery, University of Washington School of Medicine, Seattle, WA, USA

    Michael A. Williams MD, FAAN

  4. Adult Hydrocephalus Program, Department of Clinical Neuroscience, University of Calgary, Foothills Medical Centre - 12th Floor, Neurosurgery, 1403 – 29 Street NW, Calgary, Alberta, T2N 2T9, Canada

    Mark G. Hamilton MD, CM, FRCSC, FAANS

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Correspondence to Mark G. Hamilton MD, CM, FRCSC, FAANS.

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Conflict of Interest

Albert M. Isaacs receives financial support as a graduate student from the Vanier Canada Graduate Scholarship and, recently from the Killam Predoctoral Scholarship programs.

Michael A. Williams currently is a recipient of a research grant from NASA on research on intracranial pressure. In 2017, Dr. Williams received honoraria and paid travel costs from Medtronic Inc. for preparation of independently developed lectures on hydrocephalus, which was presented at Neurosciences Grand Rounds, University of University of Saskatchewan College of Medicine. Dr. Williams is the Chair of the Medical Advisor board of the Hydrocephalus Association (HA). Dr. Williams is a member of the Board of Directors of the HA.

Mark G. Hamilton is a member of the Board of Directors of the HA. Dr. Hamilton received honoraria from Integra (Canada) Inc. for preparation of an independently developed seminar on adult hydrocephalus. Dr. Hamilton is the Chair of the Adult Hydrocephalus Clinical Research Network (AHCRN), Vice-Chair of the Medical Advisor board of the Hydrocephalus Association (HA) and a member of the Board of Directors of Hydrocephalus Canada.

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Isaacs, A.M., Williams, M.A. & Hamilton, M.G. Current Update on Treatment Strategies for Idiopathic Normal Pressure Hydrocephalus. Curr Treat Options Neurol 21, 65 (2019). https://doi.org/10.1007/s11940-019-0604-z

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