Management of idiopathic normal-pressure hydrocephalus: a multi-institutional study conducted in Japan

Koreaki Mori M.D., Ph.D. 1
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  • 1 Department of Neurosurgery, Kochi Medical School, Kochi, Japan
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Object. A cooperative study was undertaken to identify factors that could be used to predict a favorable outcome after extracranial cerebrospinal fluid (CSF) diversion (shunting) in patients with suspected idiopathic normal-pressure hydrocephalus (NPH).

Methods. Questionnaires concerning patients with suspected idiopathic NPH were sent to 14 members of the Committee for Scientific Research on Intractable Hydrocephalus, sponsored by the Ministry of Health and Welfare of Japan. After the questionnaires were returned, a retrospective analysis of the responses was undertaken. To be included in the study, patients had to be 65 years of age or older and had to have undergone surgery between October 1995 and October 1998. Clinical measures included degrees of gait disturbance, dementia, and urinary incontinence as evaluated before, 3 months after, and 3 years after shunt placement. Diagnostic tests in various combinations included lumbar puncture in which CSF was withdrawn; intracranial pressure monitoring; measurements of CSF outflow resistance, level of serum α-1-antichymotrypsin, cerebral arteriovenous differences of oxygen content, and cerebral blood flow; and computerized tomography cisternography.

In this study, 120 patients were identified as having idiopathic NPH and these patients underwent placement of shunts. A ventriculoperitoneal shunt with a programmable valve was used in two thirds of the patients. At the end of 3 months (early assessment), there was an 80% overall rate of clinical improvement, which dropped to 73.3% of the 105 patients who could be evaluated at the end of the 3-year study. Of the three variables, gait disturbance was most improved, both at early and late testing periods. Shunt complications occurred in 22 (18.3%) of the patients.

Conclusions. Patients suspected of having idiopathic NPH did not form a homogeneous group, making it difficult to select those who would most likely respond to CSF diversion. Of the diagnostic studies, the most reliable result was improvement in clinical symptoms following a lumbar puncture in which CSF was withdrawn. The use of a programmable valve is recommended because it offers advantages in preventing problems of over- and underdrainage.

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Contributor Notes

Address reprint requests to: Koreaki Mori, M.D., Ph.D., 2787–2, Ikku, Kochi City, 781–8130, Japan. email: morik@i-kochi.or.jp.
  • 1.

    Adams RD, , Fisher CM, & Hakim S, et al: Symptomatic occult hydrocephalus with “normal” cerebrospinal-fluid pressure. A treatable syndrome. N Engl J Med 273:117126, 1965 Adams RD, Fisher CM, Hakim S, et al: Symptomatic occult hydrocephalus with “normal” cerebrospinal-fluid pressure. A treatable syndrome. N Engl J Med 273:117–126, 1965

    • Search Google Scholar
    • Export Citation
  • 2.

    Bech RA, , Juhler M, & Waldemar G, et al: Frontal brain and leptomeningeal biopsy specimens correlated with cerebrospinal fluid outflow resistance and B-wave activity in patients suspected of normal-pressure hydrocephalus. Neurosurgery 40:497502, 1997 Bech RA, Juhler M, Waldemar G, et al: Frontal brain and leptomeningeal biopsy specimens correlated with cerebrospinal fluid outflow resistance and B-wave activity in patients suspected of normal-pressure hydrocephalus. Neurosurgery 40:497–502, 1997

    • Search Google Scholar
    • Export Citation
  • 3.

    Bech RA, , Waldemar G, & Gjerris F, et al: Shunting effects in patients with idiopathic normal pressure hydrocephalus; correlation with cerebral and leptomeningeal biopsy findings. Acta Neurochir 141:633639, 1999 Bech RA, Waldemar G, Gjerris F, et al: Shunting effects in patients with idiopathic normal pressure hydrocephalus; correlation with cerebral and leptomeningeal biopsy findings. Acta Neurochir 141:633–639, 1999

    • Search Google Scholar
    • Export Citation
  • 4.

    Boon AJW, , Tans JT, & Delwel EJ, et al: Dutch normal-pressure hydrocephalus study: prediction of outcome after shunting by resistance to outflow of cerebrospinal fluid. J Neurosurg 87:687693, 1997 Boon AJW, Tans JT, Delwel EJ, et al: Dutch normal-pressure hydrocephalus study: prediction of outcome after shunting by resistance to outflow of cerebrospinal fluid. J Neurosurg 87:687–693, 1997

    • Search Google Scholar
    • Export Citation
  • 5.

    Boon AJW, , Tans JT, & Delwel EJ, et al: Dutch normal-pressure hydrocephalus study: randomized comparison of low- and medium-pressure shunts. J Neurosurg 88:490495, 1998 Boon AJW, Tans JT, Delwel EJ, et al: Dutch normal-pressure hydrocephalus study: randomized comparison of low- and medium-pressure shunts. J Neurosurg 88:490–495, 1998

    • Search Google Scholar
    • Export Citation
  • 6.

    Boon AJW, , Tans JT, & Delwel EJ, et al: Dutch normal-pressure hydrocephalus study: the role of cerebrovascular disease. J Neurosurg 90:221226, 1999 Boon AJW, Tans JT, Delwel EJ, et al: Dutch normal-pressure hydrocephalus study: the role of cerebrovascular disease. J Neurosurg 90:221–226, 1999

    • Search Google Scholar
    • Export Citation
  • 7.

    Caruso R, , Cervoni L, & Vitale AM, et al: Idiopathic normal-pressure hydrocephalus in adults: result of shunting correlated with clinical findings in 18 patients and review of the literature. Neurosurg Rev 20:104107, 1997 Caruso R, Cervoni L, Vitale AM, et al: Idiopathic normal-pressure hydrocephalus in adults: result of shunting correlated with clinical findings in 18 patients and review of the literature. Neurosurg Rev 20:104–107, 1997

    • Search Google Scholar
    • Export Citation
  • 8.

    Chang CC, , Kuwana N, & Ito S, et al: Prediction of effectiveness of shunting in patients with normal pressure hydrocephalus by cerebral blood flow measurement and computed tomography cisternography. Neurol Med Chir 39:841846, 1999 Chang CC, Kuwana N, Ito S, et al: Prediction of effectiveness of shunting in patients with normal pressure hydrocephalus by cerebral blood flow measurement and computed tomography cisternography. Neurol Med Chir 39:841–846, 1999

    • Search Google Scholar
    • Export Citation
  • 9.

    Chen IH, , Huang CI, & Liu HC, et al: Effectiveness of shunting in patients with normal pressure hydrocephalus predicted by temporary, controlled-resistance, continuous lumbar drainage: a pilot study. J Neurol Neurosurg Psychiatry 57:14301432, 1994 Chen IH, Huang CI, Liu HC, et al: Effectiveness of shunting in patients with normal pressure hydrocephalus predicted by temporary, controlled-resistance, continuous lumbar drainage: a pilot study. J Neurol Neurosurg Psychiatry 57:1430–1432, 1994

    • Search Google Scholar
    • Export Citation
  • 10.

    de Jong DA, , Delwel EJ, & Avezaat CJ: Hydrostatic and hydrodynamic considerations in shunted normal pressure hydrocephalus. Acta Neurochir 142:241247, 2000 de Jong DA, Delwel EJ, Avezaat CJ: Hydrostatic and hydrodynamic considerations in shunted normal pressure hydrocephalus. Acta Neurochir 142:241–247, 2000

    • Search Google Scholar
    • Export Citation
  • 11.

    Del Bigio MR, , Cardoso ER, & Halliday WC: Neuropathological changes in chronic adult hydrocephalus: cortical biopsies and autopsy findings. Can J Neurol Sci 24:121126, 1997 Del Bigio MR, Cardoso ER, Halliday WC: Neuropathological changes in chronic adult hydrocephalus: cortical biopsies and autopsy findings. Can J Neurol Sci 24:121–126, 1997

    • Search Google Scholar
    • Export Citation
  • 12.

    Hakim S, & Adams RD: The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics. J Neurol Sci 2:307327, 1965 Hakim S, Adams RD: The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics. J Neurol Sci 2:307–327, 1965

    • Search Google Scholar
    • Export Citation
  • 13.

    Ishikawa M, , Mori K, & Mima T: [Cooperative study on early and late effects of CSF shunt in idiopathic normal pressure hydrocephalus in aged people.] Jpn J Neurosurg 8:771775, 1999 (Jpn) Ishikawa M, Mori K, Mima T: [Cooperative study on early and late effects of CSF shunt in idiopathic normal pressure hydrocephalus in aged people.] Jpn J Neurosurg 8:771–775, 1999 (Jpn)

    • Search Google Scholar
    • Export Citation
  • 14.

    Krauss JK, , Droste DW, & Bohus M, et al: The relation of intracranial pressure B-waves to different sleep stages in patients with suspected normal pressure hydrocephalus. Acta Neurochir 136:195203, 1995 Krauss JK, Droste DW, Bohus M, et al: The relation of intracranial pressure B-waves to different sleep stages in patients with suspected normal pressure hydrocephalus. Acta Neurochir 136:195–203, 1995

    • Search Google Scholar
    • Export Citation
  • 15.

    Krauss JK, , Droste DW, & Vach W, et al: Cerebrospinal fluid shunting in idiopathic normal-pressure hydrocephalus of the elderly: effect of periventricular and deep white matter lesions. Neurosurgery 39:292300, 1996 Krauss JK, Droste DW, Vach W, et al: Cerebrospinal fluid shunting in idiopathic normal-pressure hydrocephalus of the elderly: effect of periventricular and deep white matter lesions. Neurosurgery 39:292–300, 1996

    • Search Google Scholar
    • Export Citation
  • 16.

    Krauss JK, & Regel JP: The predictive value of ventricular CSF removal in normal pressure hydrocephalus. Neurol Res 19:357360, 1997 Krauss JK, Regel JP: The predictive value of ventricular CSF removal in normal pressure hydrocephalus. Neurol Res 19:357–360, 1997

    • Search Google Scholar
    • Export Citation
  • 17.

    Krauss JK, , Regel JP, & Vach W, et al: Vascular risk factors and arteriosclerotic disease in idiopathic normal-pressure hydrocephalus of the elderly. Stroke 27:2429, 1996 Krauss JK, Regel JP, Vach W, et al: Vascular risk factors and arteriosclerotic disease in idiopathic normal-pressure hydrocephalus of the elderly. Stroke 27:24–29, 1996

    • Search Google Scholar
    • Export Citation
  • 18.

    Krauss JK, , Regel JP, & Vach W, et al: White matter lesions in patients with idiopathic normal pressure hydrocephalus and in an age-matched control group: a comparative study. Neurosurgery 40:491496, 1997 Krauss JK, Regel JP, Vach W, et al: White matter lesions in patients with idiopathic normal pressure hydrocephalus and in an age-matched control group: a comparative study. Neurosurgery 40:491–496, 1997

    • Search Google Scholar
    • Export Citation
  • 19.

    Kristensen B, , Malm J, & Fagerland M, et al: Regional cerebral blood flow, white matter abnormalities, and cerebrospinal fluid hydrodynamics in patients with idiopathic adult hydrocephalus syndrome. J Neurol Neurosurg Psychiatry 60:282288, 1996 Kristensen B, Malm J, Fagerland M, et al: Regional cerebral blood flow, white matter abnormalities, and cerebrospinal fluid hydrodynamics in patients with idiopathic adult hydrocephalus syndrome. J Neurol Neurosurg Psychiatry 60:282–288, 1996

    • Search Google Scholar
    • Export Citation
  • 20.

    Meier U, , Zeilinger FS, & Kintzel D: Signs, symptoms and course of normal pressure hydrocephalus in comparison with cerebral atrophy. Acta Neurochir 141:10391048, 1999 Meier U, Zeilinger FS, Kintzel D: Signs, symptoms and course of normal pressure hydrocephalus in comparison with cerebral atrophy. Acta Neurochir 141:1039–1048, 1999

    • Search Google Scholar
    • Export Citation
  • 21.

    Miyake H, , Ohta T, & Kajimoto Y, et al: Diamox® challenge test to decide indications for cerebrospinal fluid shunting in normal pressure hydrocephalus. Acta Neurochir 141:11871193, 1999 Miyake H, Ohta T, Kajimoto Y, et al: Diamox® challenge test to decide indications for cerebrospinal fluid shunting in normal pressure hydrocephalus. Acta Neurochir 141:1187–1193, 1999

    • Search Google Scholar
    • Export Citation
  • 22.

    Mori K (ed): [Annual Report of the Research Committee of “intractable hydrocephalus.”] Tokyo: Ministry of Health and Welfare of Japan, 1997 (Jpn) Mori K (ed): [Annual Report of the Research Committee of “intractable hydrocephalus.”] Tokyo: Ministry of Health and Welfare of Japan, 1997 (Jpn)

    • Search Google Scholar
    • Export Citation
  • 23.

    Mori K (ed): [Annual Report of the Research Committee of “intractable hydrocephalus.”] Tokyo: Ministry of Health and Welfare of Japan, 1998 (Jpn) Mori K (ed): [Annual Report of the Research Committee of “intractable hydrocephalus.”] Tokyo: Ministry of Health and Welfare of Japan, 1998 (Jpn)

    • Search Google Scholar
    • Export Citation
  • 24.

    Mori K (ed): [Annual Report of the Research Committee of “intractable hydrocephalus.”] Tokyo: Ministry of Health and Welfare of Japan, 1999 (Jpn) Mori K (ed): [Annual Report of the Research Committee of “intractable hydrocephalus.”] Tokyo: Ministry of Health and Welfare of Japan, 1999 (Jpn)

    • Search Google Scholar
    • Export Citation
  • 25.

    Mori K, & Mima T: Can we predict the benefit of a shunting operation for suspected normal pressure hydrocephalus? Crit Rev Neurosurg 7:263275, 1997 Mori K, Mima T: Can we predict the benefit of a shunting operation for suspected normal pressure hydrocephalus? Crit Rev Neurosurg 7:263–275, 1997

    • Search Google Scholar
    • Export Citation
  • 26.

    Mori K, & Mima T: To what extent has the pathophysiology of normal-pressure hydrocephalus been clarified? Crit Rev Neurosurg 8:232243, 1998 Mori K, Mima T: To what extent has the pathophysiology of normal-pressure hydrocephalus been clarified? Crit Rev Neurosurg 8:232–243, 1998

    • Search Google Scholar
    • Export Citation
  • 27.

    Nacmias B, , Tedde A, & Guarnieri BM, et al: Analysis of apolipoprotein E, α 1-antichymotrypsin and presenilin-1 genes polymorphisms in dementia caused by normal pressure hydrocephalus in man. Neurosci Lett 229:177180, 1997 Nacmias B, Tedde A, Guarnieri BM, et al: Analysis of apolipoprotein E, α 1-antichymotrypsin and presenilin-1 genes polymorphisms in dementia caused by normal pressure hydrocephalus in man. Neurosci Lett 229:177–180, 1997

    • Search Google Scholar
    • Export Citation
  • 28.

    Sand T, , Bovim G, & Grimse R, et al: Idiopathic normal pressure hydrocephalus: the CSF tap-test may predict the clinical response to shunting. Acta Neurol Scand 89:311316, 1994 Sand T, Bovim G, Grimse R, et al: Idiopathic normal pressure hydrocephalus: the CSF tap-test may predict the clinical response to shunting. Acta Neurol Scand 89:311–316, 1994

    • Search Google Scholar
    • Export Citation
  • 29.

    Schwarzschild M, , Rordorf G, & Bekken K, et al: Normal-pressure hydrocephalus with misleading features of irreversible dementias: a case report. J Geriatr Psychiatry Neurol 10:5154, 1997 Schwarzschild M, Rordorf G, Bekken K, et al: Normal-pressure hydrocephalus with misleading features of irreversible dementias: a case report. J Geriatr Psychiatry Neurol 10:51–54, 1997

    • Search Google Scholar
    • Export Citation
  • 30.

    Takeuchi T, , Kasahara E, & Iwasaki M, et al: Indications for shunting in patients with idiopathic normal pressure hydrocephalus presenting dementia and brain atrophy (atypical idiopathic normal pressure hydrocephalus). Neurol Med Chir 40:3847, 2000 Takeuchi T, Kasahara E, Iwasaki M, et al: Indications for shunting in patients with idiopathic normal pressure hydrocephalus presenting dementia and brain atrophy (atypical idiopathic normal pressure hydrocephalus). Neurol Med Chir 40:38–47, 2000

    • Search Google Scholar
    • Export Citation
  • 31.

    Tanaka A, , Kimura M, & Nakayama Y, et al: Cerebral blood flow and autoregulation in normal pressure hydrocephalus. Neurosurgery 40:11611167, 1997 Tanaka A, Kimura M, Nakayama Y, et al: Cerebral blood flow and autoregulation in normal pressure hydrocephalus. Neurosurgery 40:1161–1167, 1997

    • Search Google Scholar
    • Export Citation
  • 32.

    Tokoro K, & Chiba Y: Optimum position for an anti-siphon device in a cerebrospinal fluid shunt system. Neurosurgery 29:519525, 1991 Tokoro K, Chiba Y: Optimum position for an anti-siphon device in a cerebrospinal fluid shunt system. Neurosurgery 29:519–525, 1991

    • Search Google Scholar
    • Export Citation
  • 33.

    Vanneste JA: Three decades of normal pressure hydrocephalus: are we wiser now? J Neurol Neurosurg Psychiatry 57:10211025, 1994 Vanneste JA: Three decades of normal pressure hydrocephalus: are we wiser now? J Neurol Neurosurg Psychiatry 57:1021–1025, 1994

    • Search Google Scholar
    • Export Citation

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