Abstract
Normal pressure hydrocephalus (NPH) is an important differential diagnosis of neurodegenerative diseases. The prevalence of dementia is increasing in line with the worldwide increase in life expectancy. NPH can be divided into idiopathic (iNPH) and secondary (sNPH) which is important in terms of clinical symptoms, future progress, and the outcome of possible treatment. The full clinical triad is not prevalent in all of the cases and the pathophysiology of iNPH remains unclear. Diagnosis is based on the evaluation of clinical symptoms (Hakim’s triad) combined with an MRI assessment, evaluation of CSF dynamic parameters by different methods such as a tap test, lumbar infusion test (LIT), and external lumbar drainage (ELD). Despite the development of diagnostic techniques and strategies in management, NPH remains to be a challenge for the specialists despite more than 50 years of research. However, results of this research have brought new opportunities in the diagnosis, therapy, and quality of life as well as survival time of NPH patients with improved symptoms. The aim of this article is to present the pathophysiological hypotheses of NPH and an overview of the diagnostic techniques used for the evaluation of NPH patients.
Similar content being viewed by others
References
Adachi M, Kawanami T, Ohshima F, Kato T (2006) Upper midbrain profile sign and cingulate sulcus sign: MRI findings on sagittal images in idiopathic normal-pressure hydrocephalus, Alzheimer’s disease, and progressive supranuclear palsy. Radiat Med 24:568–572. https://doi.org/10.1007/s11604-006-0074-6
Akai K, Uchigasaki S, Tanaka U, Komatsu A (2008) Normal pressure hydrocephalus: neuropathological study. Pathol Int 37:97–110. https://doi.org/10.1111/j.1440-1827.1987.tb03137.x
Albeck MJ, Børgesen SE, Gjerris F, Schmidt JF, Sørensen PS (1991) Intracranial pressure and cerebrospinal fluid outflow conductance in healthy subjects. J Neurosurg:597–600. https://doi.org/10.3171/jns.1991.74.4.0597
Al-Zain FT, Rademacher G, Meier U, Mutze S, Lemcke J (2008) The role of cerebrospinal fluid flow study using phase contrast MR imaging in diagnosing idiopathic normal pressure hydrocephalus. Acta Neurochir Suppl. https://doi.org/10.1007/978-3-211-85578-2_24
Ammar A, Abbas F, Al Issawi W, Fakhro F, Batarfi L, Hendam A, Hasen M, El Shawarby M, Al Jehani H (2017) Idiopathic normal-pressure hydrocephalus syndrome: is it understood? The comprehensive idiopathic normal-pressure hydrocephalus theory (CiNPHT). In: Hydrocephalus: what do we know? and what do we still not know? pp 67–82. https://doi.org/10.1007/978-3-319-61304-8_5
Arighi A, Di Cristofori A, Fenoglio C, Borsa S, D’Anca M, Fumagalli GG, Locatelli M, Carrabba G, Pietroboni AM, Ghezzi L, Carandini T, Colombi A, Scarioni M, De Riz MA, Serpente M, Rampini PM, Scarpini E, Galimberti D (2019) Cerebrospinal fluid level of Aquaporin4: a new window on glymphatic system involvement in neurodegenerative disease? J Alzheimers Dis 69:663–669. https://doi.org/10.3233/JAD-190119
Bateman GA (2004) Idiopathic intracranial hypertension: priapism of the brain? Med Hypotheses 63:549–552. https://doi.org/10.1016/j.mehy.2004.03.014
Bateman GA, Brown KM (2012) The measurement of CSF flow through the aqueduct in normal and hydrocephalic children: from where does it come, to where does it go? Childs Nerv Syst 28:55–63. https://doi.org/10.1007/s00381-011-1617-4
Bech-Azeddine R, Høgh P, Juhler M, Gjerris F, Waldemar G (2007) Idiopathic normal-pressure hydrocephalus: clinical comorbidity correlated with cerebral biopsy findings and outcome of cerebrospinal fluid shunting. J Neurol Neurosurg Psychiatry 78:157–161. https://doi.org/10.1136/jnnp.2006.095117
Benveniste RJ, Sur S (2018) Delayed symptom progression after ventriculoperitoneal shunt placement for normal pressure hydrocephalus. J Neurol Sci 393:105–109. https://doi.org/10.1016/j.jns.2018.08.002
Blomsterwall E, Svantesson U, Carlsson U, Tullberg M, Wikkelsö C (2000) Postural disturbance in patients with normal pressure hydrocephalus. Acta Neurol Scand 102:284–291. https://doi.org/10.1034/j.1600-0404.2000.102005284.x
Børgesen SE, Gjerris F (1987) Relationships between intracranial pressure, ventricular size, and resistance to CSF outflow. J Neurosurg 67:535–539. https://doi.org/10.3171/jns.1987.67.4.0535
Børgesen SE, Gjerris F, Sørensen SC (1979) Cerebrospinal fluid conductance and compliance of the craniospinal space in normal-pressure hydrocephalus. J Neurosurg 51:521–525. https://doi.org/10.3171/jns.1979.51.4.0521
Børgesen SE, Gyldensted C, Gjerris F, Lester J (1980) Computed tomography and pneumoencephalography compared to conductance to outflow of CSF in normal pressure hydrocephalus. Neuroradiology 20:17–22. https://doi.org/10.1007/bf00346856
Brean A, Eide PK (2008) Assessment of idiopathic normal pressure patients in neurological practice: the role of lumbar infusion testing for referral of patients to neurosurgery. Eur J Neurol 15:605–612. https://doi.org/10.1111/j.1468-1331.2008.02134.x
Brecknell JE, Brown JI (2004) Is idiopathic normal pressure hydrocephalus an independent entity? Acta Neurochir 146:1003–1006; discussion 1006-1007. https://doi.org/10.1007/s00701-004-0332-2
Cabral D, Beach TG, Vedders L, Sue LI, Jacobson S, Myers K, Sabbagh MN (2011) Frequency of Alzheimer’s disease pathology at autopsy in patients with clinical normal pressure hydrocephalus. Alzheimers Dement 7:509–513. https://doi.org/10.1016/j.jalz.2010.12.008
Cheng W (2013) Analysis of related factors affecting prognosis of shunt surgery in patients with secondary normal pressure hydrocephalus. Chin J Traumatol:221–224. https://doi.org/10.3760/cma.j.issn.1008-1275.2013.04.007
Chikly B, Quaghebeur J (2013) Reassessing cerebrospinal fluid (CSF) hydrodynamics: a literature review presenting a novel hypothesis for CSF physiology. J Bodyw Mov Ther 17:344–354. https://doi.org/10.1016/j.jbmt.2013.02.002
Chotai S, Medel R, Herial N, Medhkour A (2014) External lumbar drain: a pragmatic test for prediction of shunt outcomes in idiopathic normal pressure hydrocephalus. Surg Neurol Int 5:12. https://doi.org/10.4103/2152-7806.125860
Craven CL, Toma AK, Mostafa T, Patel N, Watkins LD (2016) The predictive value of DESH for shunt responsiveness in idiopathic normal pressure hydrocephalus. J Clin Neurosci 34:294–298. https://doi.org/10.1016/j.jocn.2016.09.004
Czepko R, Cieslicki K (2016) Repeated assessment of suspected normal pressure hydrocephalus in non-shunted cases. A prospective study based on the constant rate lumbar infusion test. Acta Neurochir 158:855–863. https://doi.org/10.1007/s00701-016-2732-5
Czosnyka Z, Czosnyka M (2017) Long-term monitoring of intracranial pressure in normal pressure hydrocephalus and other CSF disorders. Acta Neurochir 159:1979–1980. https://doi.org/10.1007/s00701-017-3282-1
Damasceno BP (2015) Neuroimaging in normal pressure hydrocephalus. Dement Neuropsychologia 9:350–355. https://doi.org/10.1590/1980-57642015DN94000350
Daou B, Klinge P, Tjoumakaris S, Rosenwasser RH, Jabbour P (2016) Revisiting secondary normal pressure hydrocephalus: does it exist? A review. Neurosurg Focus 41. https://doi.org/10.3171/2016.6.FOCUS16189
Dennhardt J, LeDoux MS (2010) Huntington disease in a nonagenarian mistakenly diagnosed as normal pressure hydrocephalus. J Clin Neurosci 17:1066–1067. https://doi.org/10.1016/j.jocn.2009.11.011
Devito EE, Pickard JD, Salmond CH, Iddon JL, Loveday C, Sahakian BJ (2005) The neuropsychology of normal pressure hydrocephalus (NPH). Br J Neurosurg 19:217–224. https://doi.org/10.1080/02688690500201838
Eide PK, Ringstad G (2019) Delayed clearance of cerebrospinal fluid tracer from entorhinal cortex in idiopathic normal pressure hydrocephalus: a glymphatic magnetic resonance imaging study. J Cereb Blood Flow Metab 39:1355–1368. https://doi.org/10.1177/0271678X18760974
Evans WA (1942) An encephalographic ratio for estimating ventricular enlargement and cerebral atrophy. Arch Neurol Psychiatr 47:931. https://doi.org/10.1001/archneurpsyc.1942.02290060069004
Freimann FB, Streitberger KJ, Klatt D, Lin K, McLaughlin J, Braun J, Sprung C, Sack I (2012) Alteration of brain viscoelasticity after shunt treatment in normal pressure hydrocephalus. Neuroradiology 54:189–196. https://doi.org/10.1007/s00234-011-0871-1
Gallagher R, Marquez J, Osmotherly P (2018) Gait and balance measures can identify change from a cerebrospinal fluid tap test in idiopathic normal pressure hydrocephalus. Arch Phys Med Rehabil 99:2244–2250. https://doi.org/10.1016/j.apmr.2018.03.018
Gallagher R, Marquez J, Osmotherly P (2019) Clinimetric properties and minimal clinically important differences for a battery of gait, balance, and cognitive examinations for the tap test in idiopathic normal pressure hydrocephalus. Neurosurgery 84:E378–E384. https://doi.org/10.1093/neuros/nyy286
Ghosh S, Lippa C (2014) Diagnosis and prognosis in idiopathic normal pressure hydrocephalus. Am J Alzheimers Dis Other Dement 29:583–589. https://doi.org/10.1177/1533317514523485
Giordan E, Palandri G, Lanzino G, Murad MH, Elder BD (2018) Outcomes and complications of different surgical treatments for idiopathic normal pressure hydrocephalus: a systematic review and meta-analysis. J Neurosurg:1–13. https://doi.org/10.3171/2018.5.JNS1875
Governale LS, Fein N, Logsdon J, Black PM (2008) Techniques and complications of external lumbar drainage for normal pressure hydrocephalus. Neurosurgery 63:ONS379–ONS384. https://doi.org/10.1227/01.NEU.0000327023.18220.88
Grahnke K, Jusue-Torres I, Szujewski C, Joyce C, Schneck M, Prabhu VC, Anderson DE (2018) The quest for predicting sustained shunt response in normal-pressure hydrocephalus: an analysis of the callosal angle’s utility. World Neurosurg 115:e717–e722. https://doi.org/10.1016/j.wneu.2018.04.150
Greitz D, Hannerz J, Rahn T, Bolander H, Ericsson A (1994) MR imaging of cerebrospinal fluid dynamics in health and disease. On the vascular pathogenesis of communicating hydrocephalus and benign intracranial hypertension. Acta Radiologica:204–211. https://doi.org/10.1177/028418519403500302
Hakim S, Adams RD (1965) The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure observations on cerebrospinal fluid hydrodynamics. J Neurol Sci:307–327. https://doi.org/10.1016/0022-510X(65)90016-X
Hasan-Olive MM, Enger R, Hansson HA, Nagelhus EA, Eide PK (2019) Loss of perivascular aquaporin-4 in idiopathic normal pressure hydrocephalus. Glia 67:91–100. https://doi.org/10.1002/glia.23528
Hashimoto M, Ishikawa M, Mori E, Kuwana N (2010) Diagnosis of idiopathic normal pressure hydrocephalus is supported by MRI-based scheme: a prospective cohort study. Cerebrospinal Fluid Res 7:7–11. https://doi.org/10.1186/1743-8454-7-18
Hattingen E, Jurcoane A, Melber J, Blasel S, Zanella FE, Neumann-Haefelin T, Singer OC (2010) Diffusion tensor imaging in patients with adult chronic idiopathic hydrocephalus. Neurosurgery 66:917–924. https://doi.org/10.1227/01.NEU.0000367801.35654.EC
Hebb AO, Cusimano MD (2001) Idiopathic normal pressure hydrocephalus: a systematic review of diagnosis and outcome. Neurosurgery 49:1166–1186
Hellström P, Klinge P, Tans J, Wikkelsø C (2012) A new scale for assessment of severity and outcome in iNPH. Acta Neurol Scand 126:229–237. https://doi.org/10.1111/j.1600-0404.2012.01677.x
Hoff J, Barber R (1974) Transcerebral mantle pressure in normal pressure hydrocephalus. Arch Neurol 31:101–105. https://doi.org/10.1001/archneur.1974.00490380049005
Iddon JL, Pickard JD, Cross JJL, Griffiths PD, Czosnyka M, Sahakian BJ (1999) Specific patterns of cognitive impairment in patients with idiopathic normal pressure hydrocephalus and Alzheimer’s disease: a pilot study. J Neurol Neurosurg Psychiatry 67:723–732. https://doi.org/10.1136/jnnp.67.6.723
Iseki C, Kawanami T, Nagasawa H, Wada M, Koyama S, Kikuchi K, Arawaka S, Kurita K, Daimon M, Mori E, Kato T (2009) 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 277:54–57. https://doi.org/10.1016/j.jns.2008.10.004
Ishii K, Kanda T, Harada A, Miyamoto N, Kawaguchi T, Shimada K, Ohkawa S, Uemura T, Yoshikawa T, Mori E (2008) Clinical impact of the callosal angle in the diagnosis of idiopathic normal pressure hydrocephalus. Eur Radiol 18:2678–2683. https://doi.org/10.1007/s00330-008-1044-4
Ishikawa M, Oowaki H, Takezawa M, Takenaka T, Yamada S, Yamamoto K, Okamoto S (2016) Disproportionately enlarged subarachnoid space hydrocephalus in idiopathic normal-pressure hydrocephalus and its implication in pathogenesis. In: Ang B-T (ed) Intracranial Pressure and Brain Monitoring XV, vol 122. Springer International Publishing, Cham, pp 287–290
Jeppsson A, Zetterberg H, Blennow K, Wikkelso C (2013) Idiopathic normal-pressure hydrocephalus: pathophysiology and diagnosis by CSF biomarkers. Neurology 80:1385–1392. https://doi.org/10.1212/WNL.0b013e31828c2fda
Kahlon B, Sundbärg G, Rehncrona S (2002) Comparison between the lumbar infusion and CSF tap tests to predict outcome after shunt surgery in suspected normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry 73:721–726. https://doi.org/10.1136/jnnp.73.6.721
Keong NCH, Pena A, Price SJ, Czosnyka M, Czosnyka Z, Pickard JD (2016) Imaging normal pressure hydrocephalus: theories, techniques, and challenges. Neurosurg Focus 41. https://doi.org/10.3171/2016.7.FOCUS16194
Kiefer M, Unterberg A (2012) The differential diagnosis and treatment of normal-pressure hydrocephalus. Deutsches Arzteblatt Int 109:15–26. https://doi.org/10.3238/arztebl.2012.0015
Kim MJ, Seo SW, Lee KM, Kim ST, Lee JI, Nam DH, Na DL (2011) Differential diagnosis of idiopathic normal pressure hydrocephalus from other dementias using diffusion tensor imaging. AJNR Am J Neuroradiol 32:1496–1503. https://doi.org/10.3174/ajnr.A2531
Kim DJ, Kim H, Kim YT, Yoon BC, Czosnyka Z, Park KW, Czosnyka M (2015) Thresholds of resistance to CSF outflow in predicting shunt responsiveness. Neurol Res 37:332–340. https://doi.org/10.1179/1743132814Y.0000000454
Kito Y, Kazui H, Kubo Y, Yoshida T, Takaya M, Wada T, Nomura K, Hashimoto M, Ohkawa S, Miyake H, Ishikawa M, Takeda M (2009) Neuropsychiatric symptoms in patients with idiopathic normal pressure hydrocephalus. Behav Neurol 21:165–174. https://doi.org/10.3233/BEN-2009-0233
Klausner AP, Steers WD (2011) The neurogenic bladder: an update with management strategies for primary care physicians. Med Clin North Am 95:111–120. https://doi.org/10.1016/j.mcna.2010.08.027
Klinge P, Marmarou A, Bergsneider M, Relkin N, Black PML (2005) INPH guidelines, part V: outcome of shunting in idiopathic normal-pressure hydrocephalus and the value of outcome assessment in shunted patients. Neurosurgery 57:S2-40-S42-52. https://doi.org/10.1227/01.NEU.0000168187.01077.2F
Kogan MI, Zachoval R, Ozyurt C, Schafer T, Christensen N (2014) Epidemiology and impact of urinary incontinence, overactive bladder, and other lower urinary tract symptoms: results of the EPIC survey in Russia, Czech Republic, and Turkey. Curr Med Res Opin 30:2119–2130. https://doi.org/10.1185/03007995.2014.934794
Kondziella D, Sonnewald U, Tullberg M, Wikkelso C (2008) Brain metabolism in adult chronic hydrocephalus. J Neurochem 106:1515–1524. https://doi.org/10.1111/j.1471-4159.2008.05422.x
Krishnamurthy S, Li J (2014) New concepts in the pathogenesis of hydrocephalus. Transl Pediatr 3:185–194. https://doi.org/10.3978/j.issn.2224-4336.2014.07.02
Krzastek SC, Bruch WM, Robinson SP, Young HF, Klausner AP (2017) Characterization of lower urinary tract symptoms in patients with idiopathic normal pressure hydrocephalus. Neurourol Urodyn 36:1167–1173. https://doi.org/10.1002/nau.23084
Krzastek SC, Robinson SP, Young HF, Klausner AP (2017) Improvement in lower urinary tract symptoms across multiple domains following ventriculoperitoneal shunting for idiopathic normal pressure hydrocephalus. Neurourol Urodyn 36:2056–2063. https://doi.org/10.1002/nau.23235
Lee L (2013) Riding the wave of ependymal cilia: Genetic susceptibility to hydrocephalus in primary ciliary dyskinesia: ependymal cilia and PCD. J Neurosci Res 91:1117–1132. https://doi.org/10.1002/jnr.23238
Lehnert BE, Rahbar H, Relyea-Chew A, Lewis DH, Richardson ML, Fink JR (2011) Detection of ventricular shunt malfunction in the ED: relative utility of radiography, CT, and nuclear imaging. Emerg Radiol 18:299–305. https://doi.org/10.1007/s10140-011-0955-6
Leinonen V, Koivisto AM, Savolainen S, Rummukainen J, Sutela A, Vanninen R, Jaaskelainen JE, Soininen H, Alafuzoff I (2012) Post-mortem findings in 10 patients with presumed normal-pressure hydrocephalus and review of the literature. Neuropathol Appl Neurobiol 38:72–86. https://doi.org/10.1111/j.1365-2990.2011.01195.x
Lemcke J, Meier U (2010) Improved outcome in shunted iNPH with a combination of a codman hakim programmable valve and an aesculap-miethke shuntassistant. Zentralbl Neurochir 71:113–116. https://doi.org/10.1055/s-0029-1241179
Lemcke J, Meier U (2012) Idiopathic normal pressure hydrocephalus (iNPH) and co-morbidity: an outcome analysis of 134 patients. Acta Neurochirl Suppl 114. https://doi.org/10.1007/978-3-7091-0956-4_50
Lenfeldt N, Larsson A, Nyberg L, Birgander R, Eklund A, Malm J (2011) Diffusion tensor imaging reveals supplementary lesions to frontal white matter in idiopathic normal pressure hydrocephalus. Neurosurgery 68:1586–1593; discussion 1593. https://doi.org/10.1227/NEU.0b013e31820f3401
Li X, Miyajima M, Mineki R, Taka H, Murayama K, Arai H (2006) Analysis of potential diagnostic biomarkers in cerebrospinal fluid of idiopathic normal pressure hydrocephalus by proteomics. Acta Neurochir 148:859–864; discussion 864. https://doi.org/10.1007/s00701-006-0787-4
Mahr CV, Dengl M, Nestler U, Reiss-Zimmermann M, Eichner G, Preuß M, Meixensberger J (2016) Idiopathic normal pressure hydrocephalus: diagnostic and predictive value of clinical testing, lumbar drainage, and CSF dynamics. J Neurosurg 125:591–597. https://doi.org/10.3171/2015.8.JNS151112
Malm J, Graff-Radford NR, Ishikawa M, Kristensen B, Leinonen V, Mori E, Owler BK, Tullberg M, Williams MA, Relkin NR (2013) 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 10:22. https://doi.org/10.1186/2045-8118-10-22
Marmarou A, Abd-Elfattah Foda MA, Bandoh K, Yoshihara M, Yamamoto T, Tsuji O, Zasler N, Ward JD, Young HF (1996) Posttraumatic ventriculomegaly: hydrocephalus or atrophy? A new approach for diagnosis using CSF dynamics. J Neurosurg 85:1026–1035. https://doi.org/10.3171/jns.1996.85.6.1026
Marmarou A, Bergsneider M, Klinge P, Relkin N, Black PM (2005) The value of supplemental prognostic tests for the preoperative assessment of idiopathic normal-pressure hydrocephalus. Neurosurgery 57:S17–S28; discussion ii-v. https://doi.org/10.1227/01.neu.0000168184.01002.60
Marmarou A, Young HF, Aygok GA, Sawauchi S, Tsuji O, Yamamoto T, Dunbar J (2005) Diagnosis and management of idiopathic normal-pressure hydrocephalus: a prospective study in 151 patients. J Neurosurg 102:987–997. https://doi.org/10.3171/jns.2005.102.6.0987
Martín-Láez R, Caballero-Arzapalo H, López-Menéndez LÁ, Arango-Lasprilla JC, Vázquez-Barquero A (2015) Epidemiology of idiopathic normal pressure hydrocephalus: a systematic review of the literature. World Neurosurgery 84:2002–2009. https://doi.org/10.1016/j.wneu.2015.07.005
Mascalchi M, Arnetoli G, Inzitari D, Pozzo GD, Lolli F, Caramella D (1993) Cine-mr imaging of aqueductal csf flow in normal pressure hydrocephalus syndrome before and after csf shunt. Acta Radiol:586–592. https://doi.org/10.3109/02841859309175413
Matsumae M, Sato O, Hirayama A, Hayashi N, Takizawa K, Atsumi H, Sorimachi T (2016) Research into the physiology of cerebrospinal fluid reaches a new horizon: intimate exchange between cerebrospinal fluid and interstitial fluid may contribute to maintenance of homeostasis in the central nervous system. Neurol Med Chir (Tokyo) 56:416–441. https://doi.org/10.2176/nmc.ra.2016-0020
Meier U, Bartels P (2001) The importance of the intrathecal infusion test in the diagnostic of normal-pressure hydrocephalus. Eur Neurol 46:178–186. https://doi.org/10.1159/000050801
Meier U, Lemcke J (2010) Co-morbidity as a predictor of outcome in patients with idiopathic normal-pressure hydrocephalus. Acta Neurochir Suppl 106:127–130
Meier U, Mutze S (2004) Correlation between decreased ventricular size and positive clinical outcome following shunt placement in patients with normal-pressure hydrocephalus. J Neurosurg 100:1036–1040. https://doi.org/10.3171/jns.2004.100.6.1036
Mirzayan MJ, Luetjens G, Borremans JJ, Regel JP, Krauss JK (2010) Extended long-term (> 5 years) outcome of cerebrospinal fluid shunting in idiopathic normal pressure hydrocephalus. Neurosurgery 67:295–301. https://doi.org/10.1227/01.NEU.0000371972.74630.EC
Missori P, Scollato A, Formisano R, Curra A, Mina C, Marianetti M, Polli FM, Peschillo S, Paolini S, Frati A, Miscusi M (2009) Restoration of sexual activity in patients with chronic hydrocephalus after shunt placement. Acta Neurochir 151:1241–1244. https://doi.org/10.1007/s00701-009-0331-4
Mori E, Ishikawa M, Kato T, Kazui H, Miyake H, Miyajima M, Nakajima M, Hashimoto M, Kuriyama N, Tokuda T, Ishii K, Kaijima M, Hirata Y, Saito M, Arai H (2012) Guidelines for management of idiopathic normal pressure hydrocephalus: second edition. Neurol Med Chir 52:775–809. https://doi.org/10.2176/nmc.52.775
Morimoto Y, Yoshida S, Kinoshita A, Satoh C, Mishima H, Yamaguchi N, Matsuda K, Sakaguchi M, Tanaka T, Komohara Y, Imamura A, Ozawa H, Nakashima M, Kurotaki N, Kishino T, K-i Y, Ono S (2019) Nonsense mutation in CFAP43 causes normal-pressure hydrocephalus with ciliary abnormalities. Neurology 92:e2364–e2374. https://doi.org/10.1212/WNL.0000000000007505
Ogino A, Kazui H, Miyoshi N, Hashimoto M, Ohkawa S, Tokunaga H, Ikejiri Y, Takeda M (2006) Cognitive impairment in patients with idiopathic normal pressure hydrocephalus. Dement Geriatr Cogn Disord 21:113–119. https://doi.org/10.1159/000090510
Oi S, Di Rocco C (2006) Proposal of “evolution theory in cerebrospinal fluid dynamics” and minor pathway hydrocephalus in developing immature brain. Childs Nerv Syst 22:662–669. https://doi.org/10.1007/s00381-005-0020-4
Organization WH (2016) World Health Statistics 2016
Pfanner T, Henri-Bhargava A, Borchert S (2018) Cerebrospinal fluid biomarkers as predictors of shunt response in idiopathic normal pressure hydrocephalus: a systematic review. Can J Neurol Sci 45:3–10. https://doi.org/10.1017/cjn.2017.251
Pirouzmand F, Tator CH, Rutka J (2001) Management of hydrocephalus associated with vestibular schwannoma and other cerebellopontine angle tumors. Neurosurgery 48:1246–1254
Poca MA, Solana E, Martínez-Ricarte FR, Romero M, Gándara D, Sahuquillo J (2012) Idiopathic normal pressure hydrocephalus: results of a prospective cohort of 236 shunted patients. Acta Neurochir Suppl 114. https://doi.org/10.1007/978-3-7091-0956-4_49
Preuss M, Hoffmann KT, Reiss-Zimmermann M, Hirsch W, Merkenschlager A, Meixensberger J, Dengl M (2013) Updated physiology and pathophysiology of CSF circulation--the pulsatile vector theory. Childs Nerv Syst 29:1811–1825. https://doi.org/10.1007/s00381-013-2219-0
Prince M, Bryce R, Albanese E, Wimo A, Ribeiro W, Ferri CP (2013) The global prevalence of dementia: a systematic review and metaanalysis. Alzheimers Dement 9:63–75 e62. https://doi.org/10.1016/j.jalz.2012.11.007
Pujari S, Kharkar S, Metellus P, Shuck J, Williams MA, Rigamonti D (2008) Normal pressure hydrocephalus: Long-term outcome after shunt surgery. J Neurol Neurosurg Psychiatry 79:1282–1286. https://doi.org/10.1136/jnnp.2007.123620
Raimondi AJ (1994) A unifying theory for the definition and classification of hydrocephalus. Childs Nerv Syst 10:2–12. https://doi.org/10.1007/BF00313578
Rasmussen MK, Mestre H, Nedergaard M (2018) The glymphatic pathway in neurological disorders. Lancet Neurol 17:1016–1024. https://doi.org/10.1016/s1474-4422(18)30318-1
Ravdin LD, Katzen HL, Jackson AE, Tsakanikas D, Assuras S, Relkin NR (2008) Features of gait most responsive to tap test in normal pressure hydrocephalus. Clin Neurol Neurosurg 110:455–461. https://doi.org/10.1016/j.clineuro.2008.02.003
Reddy GK, Bollam P, Caldito G (2014) Long-term outcomes of ventriculoperitoneal shunt surgery in patients with hydrocephalus. World Neurosurg 81:404–410. https://doi.org/10.1016/j.wneu.2013.01.096
Rekate HL, Nadkarni TD, Wallace D (2008) The importance of the cortical subarachnoid space in understanding hydrocephalus. J Neurosurg Pediatr:1–11. https://doi.org/10.3171/PED/2008/2/7/001
Relkin N, Marmarou A, Klinge P, Bergsneider M, Black PM (2005) Diagnosing idiopathic normal-pressure hydrocephalus. Neurosurgery 57:S2-4–S2-16. https://doi.org/10.1227/01.NEU.0000168185.29659.C5
Rigamonti D (2014) Adult hydrocephalus. Cambridge University Press, Cambridge
Ringstad G, Vatnehol SAS, Eide PK (2017) Glymphatic MRI in idiopathic normal pressure hydrocephalus. Brain 140:2691–2705. https://doi.org/10.1093/brain/awx191
Rodis I, Mahr CV, Fehrenbach MK, Meixensberger J, Merkenschlager A, Bernhard MK, Schob S, Thome U, Wachowiak R, Hirsch FW, Nestler U, Preuss M (2016) Hydrocephalus in aqueductal stenosis—a retrospective outcome analysis and proposal of subtype classification. Childs Nerv Syst 32:617–627. https://doi.org/10.1007/s00381-016-3029-y
Román GC, Jackson RE, Fung SH, Zhang YJ, Verma AK (2019) Sleep-disordered breathing and idiopathic normal-pressure hydrocephalus: recent pathophysiological advances. Curr Neurol Neurosci Rep 19:39. https://doi.org/10.1007/s11910-019-0952-9
Sakakibara R, Kanda T, Sekido T, Uchiyama T, Awa Y, Ito T, Liu Z, Yamamoto T, Yamanishi T, Yuasa T, Shirai K, Hattori T (2008) Mechanism of bladder dysfunction in idiopathic normal pressure hydrocephalus. Neurourol Urodyn 27:507–510. https://doi.org/10.1002/nau.20547
Schirinzi T, Sancesario GM, Ialongo C, Imbriani P, Madeo G, Toniolo S, Martorana A, Pisani A (2015) A clinical and biochemical analysis in the differential diagnosis of idiopathic normal pressure hydrocephalus. Front Neurol 6:86. https://doi.org/10.3389/fneur.2015.00086
Schirinzi T, Sancesario GM, Di Lazzaro G, D’Elia A, Imbriani P, Scalise S, Pisani A (2018) Cerebrospinal fluid biomarkers profile of idiopathic normal pressure hydrocephalus. J Neural Transm (Vienna) 125:673–679. https://doi.org/10.1007/s00702-018-1842-z
Sharma AK, Gaikwad S, Gupta V, Garg A, Mishra NK (2008) Measurement of peak CSF flow velocity at cerebral aqueduct, before and after lumbar CSF drainage, by use of phase-contrast MRI: utility in the management of idiopathic normal pressure hydrocephalus. Clin Neurol Neurosurg 110:363–368. https://doi.org/10.1016/j.clineuro.2007.12.021
Sosvorova L, Hill M, Mohapl M, Vitku J, Hampl R (2015) Steroid hormones in prediction of normal pressure hydrocephalus. J Steroid Biochem Mol Biol 152:124–132. https://doi.org/10.1016/j.jsbmb.2015.05.004
Stolze H (2001) Comparative analysis of the gait disorder of normal pressure hydrocephalus and Parkinson’s disease. J Neurol Neurosurg Psychiatry 70:289–297. https://doi.org/10.1136/jnnp.70.3.289
Thomas G, McGirt MJ, Woodworth GF, Heidler J, Rigamonti D, Hillis AE, Williams MA (2005) Baseline neuropsychological profile and cognitive response to cerebrospinal fluid shunting for idiopathic normal pressure hydrocephalus. Dement Geriatr Cogn Disord 20:163–168. https://doi.org/10.1159/000087092
Thomsen AM, Børgesen SE, Bruhn P, Gjerris F (1986) Prognosis of dementia in normal-pressure hydrocephalus after a shunt operation: prognosis of Dementia NPH. Ann Neurol 20:304–310. https://doi.org/10.1002/ana.410200306
Toma AK, Holl E, Kitchen ND, Watkins LD (2011) Evans’ index revisited: the need for an alternative in normal pressure hydrocephalus. Neurosurgery 68:939–944. https://doi.org/10.1227/NEU.0b013e318208f5e0
Toma AK, Stapleton S, Papadopoulos MC, Kitchen ND, Watkins LD (2011) Natural history of idiopathic normal-pressure hydrocephalus. Neurosurg Rev 34:433–438. https://doi.org/10.1007/s10143-011-0316-7
Toma AK, Papadopoulos MC, Stapleton S, Kitchen ND, Watkins LD (2013) Systematic review of the outcome of shunt surgery in idiopathic normal-pressure hydrocephalus. Acta Neurochir 155:1977–1980. https://doi.org/10.1007/s00701-013-1835-5
Tsakanikas D, Relkin N (2007) Normal pressure hydrocephalus. Semin Neurol 27:58–65. https://doi.org/10.1055/s-2006-956756
Tullberg M, Persson J, Petersen J, Hellström P, Wikkelsø C, Lundgren-Nilsson Å (2018) Shunt surgery in idiopathic normal pressure hydrocephalus is cost-effective—a cost utility analysis. Acta Neurochir 160:509–518. https://doi.org/10.1007/s00701-017-3394-7
van Harten B, Courant MN, Scheltens P, Weinstein HC (2004) Validation of the HIV Dementia Scale in an elderly cohort of patients with subcortical cognitive impairment caused by subcortical ischaemic vascular disease or a normal pressure hydrocephalus. Dement Geriatr Cogn Disord 18:109–114. https://doi.org/10.1159/000077818
Virhammar J, Laurell K, Cesarini KG, Larsson E-M (2014) The callosal angle measured on MRI as a predictor of outcome in idiopathic normal-pressure hydrocephalus. J Neurosurg 120:178–184. https://doi.org/10.3171/2013.8.JNS13575
Virhammar J, Laurell K, Cesarini KG, Larsson E-M (2014) Preoperative prognostic value of MRI findings in 108 patients with idiopathic normal pressure hydrocephalus. Am J Neuroradiol 35:2311–2318. https://doi.org/10.3174/ajnr.A4046
Walchenbach R, Geiger E, Thomeer RTWM, Vanneste JAL (2002) The value of temporary external lumbar CSF drainage in predicting the outcome of shunting on normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry 72:503–506. https://doi.org/10.1136/jnnp.72.4.503
Wikkelsø C, Andersson H, Blomstrand C, Lindqvist G, Svendsen P (1986) Predictive value of the cerebrospinal fluid tap-test. Acta Neurol Scand 73:566–573. https://doi.org/10.1111/j.1600-0404.1986.tb04601.x
Wikkelsø C, Hellström P, Klinge PM, Tans JTJ (2013) 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 84:562–568. https://doi.org/10.1136/jnnp-2012-303314
Williams MA, Malm J (2016) Diagnosis and treatment of idiopathic normal pressure hydrocephalus. Normal Press Hydroceph 21
Williams MA, Thomas G, De Lateur B, Imteyaz H, Rose JG, Shore WS, Kharkar S, Rigamonti D (2008) Objective assessment of gait in normal-pressure hydrocephalus. Am J Phys Med Rehabil 87:39–45. https://doi.org/10.1097/PHM.0b013e31815b6461
Yamada S, Ishikawa M, Miyajima M, Nakajima M, Atsuchi M, Kimura T, Tokuda T, Kazui H, Mori E (2017) Timed up and go test at tap test and shunt surgery in idiopathic normal pressure hydrocephalus. Neurol Clin Pract 7:98–108. https://doi.org/10.1212/CPJ.0000000000000334
Zemack G, Romner B, Pickard JD, Sindou MP, Drake JM, Milhorat TH (2002) Adjustable valves in normal-pressure hydrocephalus: a retrospective study of 218 patients. Neurosurgery 51:1392–1402. https://doi.org/10.1097/00006123-200212000-00009
Funding
This study is supported by an institutional grant from the Czech Ministry of Defence MO1012.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Ethical approval
For this type of study formal consent is not required.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Skalický, P., Mládek, A., Vlasák, A. et al. Normal pressure hydrocephalus—an overview of pathophysiological mechanisms and diagnostic procedures. Neurosurg Rev 43, 1451–1464 (2020). https://doi.org/10.1007/s10143-019-01201-5
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10143-019-01201-5