Normal pressure hydrocephalus: an update

Carlos Eduardo Borges Passos-Neto; Cesar Castello Branco Lopes; Mauricio Silva Teixeira; Adalberto Studart Neto; Raphael Ribeiro Spera

doi:10.1590/0004-282X-ANP-2022-S118

Arquivos de Neuro-Psiquiatria, Table of Contents

CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2022; 80(S 05): 42-52
DOI: 10.1590/0004-282X-ANP-2022-S118

NEUROLOGY OF COGNITIVE AND BEHAVIORAL DISORDERS

Normal pressure hydrocephalus: an update

Hidrocefalia de pressão normal: uma atualização

Authors

Carlos Eduardo Borges Passos-Neto

¹Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Departamento de Neurologia, Grupo de Neurologia Cognitiva e do Comportamento, São Paulo - SP, Brazil.
Cesar Castello Branco Lopes

¹Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Departamento de Neurologia, Grupo de Neurologia Cognitiva e do Comportamento, São Paulo - SP, Brazil.
Mauricio Silva Teixeira

¹Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Departamento de Neurologia, Grupo de Neurologia Cognitiva e do Comportamento, São Paulo - SP, Brazil.
Adalberto Studart Neto

¹Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Departamento de Neurologia, Grupo de Neurologia Cognitiva e do Comportamento, São Paulo - SP, Brazil.
Raphael Ribeiro Spera

¹Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, Departamento de Neurologia, Grupo de Neurologia Cognitiva e do Comportamento, São Paulo - SP, Brazil.

Abstract

ABSTRACT

Normal pressure hydrocephalus (NPH) has been a topic of debate since its introduction in publications. More frequent in the elderly population, it is characterized by gait disturbance, urinary urge incontinence and cognitive decline. Therefore, it is a clinical-radiological entity with relatively common findings for the age group, which together may have greater specificity. Therefore, its diagnosis must be careful for an adequate selection of patients for treatment with ventricular shunt, since the symptoms are potentially reversible. The tap test has a high positive predictive value as a predictor of therapeutic response, but a negative test does not exclude the possibility of treatment. Scientific efforts in recent years have been directed towards a better understanding of NPH and this narrative review aims to compile recent data from the literature in a didactic way for clinical practice.

RESUMO

A hidrocefalia de pressão normal (HPN) é tema de debate desde sua introdução na literatura. Mais frequente na população idosa, caracteriza-se por distúrbio de marcha, urge-incontinência urinária e declínio cognitivo. Portanto, trata-se de uma entidade clínico-radiológica com achados relativamente comuns para a faixa etária, que em conjunto, podem ter maior especificidade. Sendo assim, seu diagnóstico deve ser criterioso para uma adequada seleção de pacientes para tratamento com a derivação ventricular, uma vez que os sintomas são potencialmente reversíveis. O tap test possui valor preditivo positivo alto preditor de resposta terapêutica, mas um teste negativo não exclui a possibilidade de tratamento. Esforços científicos nos últimos anos têm sido direcionados para melhor entendimento da HPN e essa revisão narrativa se propõe a compilar dados recentes da literatura de forma didática para a prática clínica.

Keywords:

Hydrocephalus, Normal Pressure - Dementia - Spinal Puncture

Palavras-chave:

Hidrocefalia de Pressão Normal - Demência - Punção Espinal

Full Text

References

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 1965; 2 (04) 307-327 https://doi.org/10.1016/0022-510x(65)90016-x
2 Adams RD, Fisher CM, Hakim S, Ojemann RG, Sweet WH. Symptomatic occult hydrocephalus with "normal" cerebrospinal-fluid pressure. A treatable syndrome. N Engl J Med 1965; 273: 117-126 https://doi.org/10.1056/NEJM196507152730301
3 Ishikawa M. Guideline Committe for Idiopathic Normal Pressure Hydrocephalus, Japanese Society of Normal Pressure Hydrocephalus. Clinical guidelines for idiopathic normal pressure hydrocephalus. Neurol Med Chir (Tokyo) 2004; 44 (04) 222-223 https://doi.org/10.2176/nmc.44.222
4 Yamada S, Ishikawa M, Iwamuro Y, Yamamoto K. Choroidal fissure acts as an overflow device in cerebrospinal fluid drainage: morphological comparison between idiopathic and secondary normal-pressure hydrocephalus. Sci Rep 2016; 6: 39070-39070 https://doi.org/10.1038/srep39070
5 Wallenstein MB, McKhann GM. Salomon Hakim and the discovery of normal-pressure hydrocephalus. Neurosurgery 2010; 67 (01) 155-159 https://doi.org/10.1227/01.NEU.0000370058.12120.0E
6 Espay AJ, Da Prat GA, Dwivedi AK, Rodriguez-Porcel F, Vaughan JE, Rosso M. et al. Deconstructing normal pressure hydrocephalus: ventriculomegaly as early sign of neurodegeneration. Ann Neurol 2017; 82 (04) 503-513 https://doi.org/10.1002/ana.25046
7 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 https://doi.org/10.2176/nmc.52.775
8 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) 2063-2071 https://doi.org/10.1212/WNL.2022s1182022s1182193
9 Relkin N, Marmarou A, Klinge P, Bergsneider M, Black PM. Diagnosing idiopathic normalpressure hydrocephalus. Neurosurgery 2005; 57 (Suppl. 03) S4-16 https://doi.org/10.1227/01.neu.0000168185.29659.c5
10 Verrees M, Selman WR. Management of normal pressure hydrocephalus. Am Fam Physician 2004; 70 (06) 1071-1078
11 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; 48 (05) 197-199 https://doi.org/10.2176/nmc.48.197
12 Marmarou A, Black P, Bergsneider M, Klinge P, Relkin N. International NPH Consultant Group. Guidelines for management of idiopathic normal pressure hydrocephalus: progress to date. Acta Neurochir Suppl 2005; 95: 237-240 https://doi.org/10.1007/3-211-32318-x_48
13 Ishikawa M, Hashimoto M, Kuwana N, Mori E, Miyake H, Wachi A. et al. Guidelines for management of idiopathic normal pressure hydrocephalus. Neurol Med Chir (Tokyo) 2008; 48: S1-23 https://doi.org/10.2176/nmc.48.S1
14 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: 171-175 https://doi.org/10.1159/000186501
15 Trenkwalder C, Schwarz J, Gebhard J, Ruland D, Trenkwalder P, Hense HW. et al. Starnberg trial on epidemiology of Parkinsonism and hypertension in the elderly. Prevalence of Parkinson's disease and related disorders assessed by a door-to-door survey of inhabitants older than 65 years. Arch Neurol 1995; 52 (10) 1017-1022 https://doi.org/10.1001/archneur.1995.00540340109020
16 Brean A, Eide PK. Prevalence of probable idiopathic normal pressure hydrocephalus in a Norwegian population. Acta Neurol Scand 2008; 118 (01) 48-53 https://doi.org/10.1111/j.1600-0404.2007.00982.x
17 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) 1282-1286 https://doi.org/10.1136/jnnp.2007.123620
18 Hakim CA, Hakim R, Hakim S. Normal-pressure hydrocephalus. Neurosurg Clin N Am 2001; 12 (04) 761-773
19 Bech-Azeddine R, Waldemar G, Knudsen GM, Høgh P, Bruhn P, Wildschiødtz G. et al. Idiopathic normal-pressure hydrocephalus: evaluation and findings in a multidisciplinary memory clinic. Eur J Neurol 2001; 8 (06) 601-611 https://doi.org/10.1046/j.1468-1331.2001.00291.x
20 Vale FAC, Miranda SJC. Clinical and demographic features of patients with dementia attended in a tertiary outpatient clinic. Arq Neuropsiquiatr 2002; 60 3-A 548-552 https://doi.org/10.1590/s0004-282x2002000400006
21 Nitrini R, Mathias SC, Caramelli P, Carrilho PE, Lefèvre BH, Porto CS. et al. Evaluation of 100 patients with dementia in São Paulo, Brazil: correlation with socioeconomic status and education. Alzheimer Dis Assoc Disord 1995; 9 (03) 146-151
22 Takada LT, Caramelli P, Radanovic M, Anghinah R, Hartmann APBJ, Guariglia CC. et al. Prevalence of potentially reversible dementias in a dementia outpatient clinic of a tertiary university-affiliated hospital in Brazil. Arq Neuropsiquiatr 2003; 61 (04) 925-929 https://doi.org/10.1590/s0004-282x2003000600007
23 Krefft TA, Graff-Radford NR, Lucas JA, Mortimer JA. Normal pressure hydrocephalus and large head size. Alzheimer Dis Assoc Disord 2004; 18 (01) 35-37 https://doi.org/10.1097/00002093-200401000-00007
24 Oliveira LM, Nitrini R, Román GC. Normal-pressure hydrocephalus: a critical review. Dement Neuropsychol 2019; 13 (02) 133-143 https://doi.org/10.1590/1980-57642018dn13-020001
25 Bae YJ, Choi BS, Kim J-M, Choi J-H, Cho SJ, Kim JH. Altered glymphatic system in idiopathic normal pressure hydrocephalus. Parkinsonism Relat Disord 2021; 82: P56-P60 https://doi.org/10.1016/j.parkreldis.2020.11.009
26 Campbel WW, Jong De. O exame neurológico. 7. Rio de Janeiro (RJ): Guanabara Koogan LTDA; 2014
27 Ropper A, Samuels M. Adams and Victor's principles of neurology. 10. McGrawHill; 2014: 1664-1664
28 Stolze H, Kuhtz-Buschbeck JP, Drücke H, Jöhnk 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 (03) 289-297 https://doi.org/10.1136/jnnp.70.3.289
29 Daroff RB, Jankovic J, Mazziotta J, Pomeroy S. Bradley's Neurology in Clinical Practice, volume 2. 7. Elsevier; 2015: 2348-2348
30 Papageorgiou SG, Kontaxis T, Bonakis A, Karahalios G, Kalfakis N, Vassilopoulos D. Rapidly progressive dementia: causes found in a Greek tertiary referral center in Athens. Alzheimer Dis Assoc Disord 2009; 23 (04) 337-346 https://doi.org/10.1097/WAD.0b013e31819e099b
31 Cagnin A, Simioni M, Tagliapietra M, Citton V, Pompanin S, Puppa AD. et al. A Simplified callosal angle measure best differentiates idiopathic-normal pressure hydrocephalus from neurodegenerative dementia. J Alzheimers Dis 2015; 46 (04) 1033-1038 https://doi.org/10.3233/JAD-150107
32 Virhammar J, Laurell K, Cesarini KG, Larsson E-M. The callosal angle measured on MRI as a predictor of outcome in idiopathic normal-pressure hydrocephalus. J Neurosurg 2014; 120 (01) 178-184 https://doi.org/10.3171/2013.8.JNS13575
33 Qvarlander S, Ambarki K, Wåhlin A, Jacobsson J, Birgander R, Malm J. et al. Cerebrospinal fluid and blood flow patterns in idiopathic normal pressure hydrocephalus. Acta Neurol Scand 2017; 135 (05) 576-584 https://doi.org/10.1111/ane.12636
34 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 P54-P57 https://doi.org/10.1016/j.jns.2008.10.004
35 Hussain M, Schott JM. Oxford textbook of cognitive neurology and dementia. 1. Oxford University Press; 2016: 481-481
36 Kizu O, Yamada K, Nishimura T. Proton chemical shift imaging in normal pressure hydrocephalus. AJNR Am J Neuroradiol 2001; 22 (09) 1659-1664
37 Lenfeldt N, Hauksson J, Birgander R, Eklund A, Malm J. Improvement after cerebrospinal fluid drainage is related to levels of N-acetyl-aspartate in idiopathic normal pressure hydrocephalus. Neurosurgery 2008; 62 (01) 135-141 https://doi.org/10.1227/01.NEU.0000311070.25992.05
38 del Mar MM, Pueyo R, Poca MA, Falcón C, Mataró M, Bargalló N. et al. Post-surgical changes in brain metabolism detected by magnetic resonance spectroscopy in normal pressure hydrocephalus: results of a pilot study. J Neurol Neurosurg Psychiatry 2007; 78 (07) 760-763 https://doi.org/10.1136/jnnp.2006.088450
39 Algin O, Hakyemez B, Parlak M. Proton MR spectroscopy and white matter hyperintensities in idiopathic normal pressure hydrocephalus and other dementias. Br J Radiol 2010; 83 993 747-752 https://doi.org/10.1259/bjr/43131041
40 Algin O, Hakyemez B, Ocakoğlu G, Parlak M. MR cisternography: is it useful in the diagnosis of normal-pressure hydrocephalus and the selection of “good shunt responders”?. Diagn Interv Radiol 2011; 17 (02) 105-111 https://doi.org/10.4261/1305-3825.DIR.3133-09.1
41 Larsson A, Ärlig Å, Bergh A-C, Bilting M, Jacobsson L, Stephensen H. et al. Quantitative SPECT cisternography in normal pressure hydrocephalus. Acta Neurol Scand 1994; 90 (03) 190-196 https://doi.org/10.1111/j.1600-0404.1994.tb02704.x
42 Thut DP, Kreychman A, Obando JA. 111In-DTPA cisternography with SPECT/CT for the evaluation of normal pressure hydrocephalus. J Nucl Med Technol 2014; 42 (01) 70-74 https://doi.org/10.2967/jnmt.113.128041
43 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; 49 (04) 366-370 https://doi.org/10.1001/archneur.1992.00530280046021
44 Kilic K, Czorny A, Auque J, Berkman Z. Predicting the outcome of shunt surgery in normal pressure hydrocephalus. J Clin Neurosci 2007; 14 (08) 729-736 https://doi.org/10.1016/j.jocn.2006.03.028
45 Mihalj M, Dolić K, Kolić K, Ledenko V. CSF tap test - obsolete or appropriate test for predicting shunt responsiveness? A systemic review. J Neurol Sci 2016; 362: 78-84 https://doi.org/10.1016/j.jns.2016.01.028
46 Kahlon B, Sundbärg G, Rehncrona S. Comparison between the lumbar infusion and CSF tap tests to predict outcome after shunt surgery in suspected normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry 2002; 73 (06) 721-726 https://doi.org/10.1136/jnnp.73.6.721
47 Katzman R, Hussey F. A simple constant-infusion manometric test for measurement of CSF absorption. I. Rationale and method. Neurology 1970; 20 (06) 534-544 https://doi.org/10.1212/wnl.20.6.534
48 Krauss JK, Droste DW, Bohus M, Regel JP, Scheremet R, Riemann D. et al. The relation of intracranial pressure B-waves to different sleep stages in patients with suspected normal pressure hydrocephalus. Acta Neurochir (Wien) 1995; 136 3-4 195-203 https://doi.org/10.1007/BF01410626
49 Raftopoulos C, Chaskis C, Delecluse F, Cantraine F, Bidaut L, Brotchi J. Morphological quantitative analysis of intracranial pressure waves in normal pressure hydrocephalus. Neurol Res 1992; 14 (05) 389-396 https://doi.org/10.1080/01616412.1992.11740091
50 Woodworth GF, McGirt MJ, Williams MA, Rigamonti D. Cerebrospinal fluid drainage and dynamics in the diagnosis of normal pressure hydrocephalus. Neurosurgery 2009; 64 (05) 919-925 https://doi.org/10.1227/01.NEU.0000341902.44760.10
51 Panagiotopoulos V, Konstantinou D, Kalogeropoulos A, Maraziotis T. The predictive value of external continuous lumbar drainage, with cerebrospinal fluid outflow controlled by medium pressure valve, in normal pressure hydrocephalus. Acta Neurochir (Wien) 2005; 147 (09) 953-958 https://doi.org/10.1007/s00701-005-0580-9
52 Walchenbach R, Geiger E, Thomeer RTWM, Vanneste JAL. The value of temporary external lumbar CSF drainage in predicting the outcome of shunting on normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry 2002; 72 (04) 503-506 https://doi.org/10.1136/jnnp.72.4.503
53 Kahlon B, Sjunnesson J, Rehncrona S. Long-term outcome in patients with suspected normal pressure hydrocephalus. Neurosurgery 2007; 60 (02) 327-332 https://doi.org/10.1227/01.NEU.0000249273.41569.6E
54 Bergsneider M, Black PM, Klinge P, Marmarou A, Relkin N. Surgical management of idiopathic normal-pressure hydrocephalus. Neurosurgery 2005; 57 Sep;57(3 Suppl 3) S29-S39 https://doi.org/10.1227/01.neu.0000168186.45363.4d
55 Farahmand D, Hilmarsson H, Högfeldt M, Tisell M. Perioperative risk factors for short term shunt revisions in adult hydrocephalus patients. J Neurol Neurosurg Psychiatry 2009; 80 (11) 1248-1253 https://doi.org/10.1136/jnnp.2007.141416
56 Klinge P, Hellström P, Tans J, Wikkelsø C. European iNPH Multicentre Study Group. One-year outcome in the European multicentre study on iNPH. Acta Neurol Scand 2012; 126 (03) 145-153 https://doi.org/10.1111/j.1600-0404.2012.01676.x
57 Kazui H, Miyajima M, Mori E, Ishikawa M. SINPHONI-2 Investigators. Lumboperitoneal shunt surgery for idiopathic normal pressure hydrocephalus (SINPHONI-2): an open-label randomised trial. Lancet Neurol 2015; 14 (06) P585-P594 https://doi.org/10.1016/S1474-4422(15)00046-0
58 Giordan E, Palandri G, Lanzino G, Murad MH, Elder BD. Outcomes and complications of different surgical treatments for idiopathic normal pressure hydrocephalus: a systematic review and meta-analysis. J Neurosurg 2018; 131 (04) 1024-1036 https://doi.org/10.3171/2018.5.JNS1875
59 Sundström N, Lagebrant M, Eklund A, Koskinen L-OD, Malm J. Subdural hematomas in 1846 patients with shunted idiopathic normal pressure hydrocephalus: treatment and long-term survival. J Neurosurg 2018; 129 (03) 797-804 https://doi.org/10.3171/2017.5.JNS17481
60 Rinaldo L, Bhargav AG, Nesvick CL, Lanzino G, Elder BD. Effect of fixed-setting versus programmable valve on incidence of shunt revision after ventricular shunting for idiopathic normal pressure hydrocephalus. J Neurosurg 2019; 133 (02) 564-572 https://doi.org/10.3171/2019.3.JNS183077