Neurochirurgie Scan 2014; 02(02): 121-134
DOI: 10.1055/s-0034-1365557
Fortbildung
Pädiatrische Neurochirurgie und Hydrozephalus
© Georg Thieme Verlag KG Stuttgart · New York

Der idiopathische Normaldruckhydrozephalus

Martin Reiss-Zimmermann
,
Matthias Preuß
Further Information

Publication History

Publication Date:
15 May 2014 (online)

Zusammenfassung

Der idiopathische Normaldruckhydrozephalus ist charakterisiert durch eine Symptomentrias aus Gangstörung, demenzieller Entwicklung und Inkontinenz sowie einer pathologischen Erweiterung des Ventrikelsystems. Die Standardtherapie besteht in der Implantation eines ventrikuloperitonealen Shunts. Trotz moderner Untersuchungsmethoden bleibt die Diagnosestellung schwierig und die zugrundeliegende Pathophysiologie in elementaren Anteilen unverstanden.

Kernaussagen

Die Symptomentrias ist nur in ca. der Hälfte der Patienten vorhanden. Als essenzieller Bestandteil für die Diagnosestellung gilt jedoch die Gangstörung.

In einer Vielzahl der Patienten liegt eine Komorbidität mit anderen Erkrankungen vor, wodurch die Diagnosestellung erschwert und/oder verzögert wird.

Die CT-Diagnostik ist für die Diagnosestellung ausreichend. Die MRT-Untersuchung bietet Vorteile für die Differenzialdiagnostik.

Die zugrundeliegende Pathophysiologie bleibt trotz unterschiedlicher Theorien bisher in wesentlichen Teilen unverstanden. Eine bessere Kenntnis dieser Vorgänge könnte zu einer Modifizierung der Therapie führen.

 
  • Literatur

  • 1 Wallenstein MB, McKhann 2nd GM. Salomon Hakim and the discovery of normal-pressure hydrocephalus. Neurosurgery 2010; 67: 155-159 ; discussion 159
  • 2 Adams RD, Fisher CM, Hakim S et al. Symptomatic occult hydrocephalus with "normal" cerebrospinal-fluid pressure. A treatable syndrome. N Engl J Med 1965; 273: 117-126
  • 3 Hakim CA, Hakim R, Hakim S. Normal-pressure hydrocephalus. Neurosurg Clin N Am 2001; 12: 761-773, ix
  • 4 Meier U, Zeilinger FS, Kintzel D. Signs, symptoms and course of normal pressure hydrocephalus in comparison with cerebral atrophy. Acta Neurochir (Wien) 1999; 141: 1039-1048
  • 5 Gallia GL, Rigamonti D, Williams MA. The diagnosis and treatment of idiopathic normal pressure hydrocephalus. Nat Clin Pract Neurol 2006; 2: 375-381
  • 6 Relkin N, Marmarou A, Klinge P et al. Diagnosing idiopathic normal-pressure hydrocephalus. Neurosurgery 2005; 57: 4-16 ; discussion ii–v
  • 7 Brean A, Fredo HL, Sollid S et al. Five-year incidence of surgery for idiopathic normal pressure hydrocephalus in Norway. Acta Neurol Scand 2009; 120: 314-316
  • 8 Tanaka N, Yamaguchi S, Ishikawa H et al. Prevalence of possible idiopathic normal-pressure hydrocephalus in Japan: the Osaki-Tajiri project. Neuroepidemiology 2009; 32: 171-175
  • 9 Krauss JK, Halve B. Normal pressure hydrocephalus: survey on contemporary diagnostic algorithms and therapeutic decision-making in clinical practice. Acta Neurochir (Wien) 2004; 146: 379-388 ; discussion 388
  • 10 Bech-Azeddine R, Hogh P, Juhler M et al. Idiopathic normal-pressure hydrocephalus: clinical comorbidity correlated with cerebral biopsy findings and outcome of cerebrospinal fluid shunting. J Neurol Neurosurg Psychiat 2007; 78: 157-161
  • 11 Silverberg GD, Mayo M, Saul T et al. Continuous CSF drainage in AD: results of a double-blind, randomized, placebo-controlled study. Neurology 2008; 71: 202-209
  • 12 Freimann F, Streitberger K-J, Klatt D et al. Alteration of brain viscoelasticity after shunt treatment in normal pressure hydrocephalus. Neuroradiology 2011; 1-8 , DOI: DOI: 10.1007/s00234-011-0871-1.
  • 13 Streitberger KJ, Wiener E, Hoffmann J et al. In vivo viscoelastic properties of the brain in normal pressure hydrocephalus. NMR Biomed 2010; , DOI: DOI: 10.1002/nbm.1602.
  • 14 Momjian S, Owler BK, Czosnyka Z et al. Pattern of white matter regional cerebral blood flow and autoregulation in normal pressure hydrocephalus. Brain 2004; 127: 965-972
  • 15 Bradley Jr WG, Bahl G, Alksne JF. Idiopathic normal pressure hydrocephalus may be a “two hit” disease: benign external hydrocephalus in infancy followed by deep white matter ischemia in late adulthood. J Magn Reson Imaging 2006; 24: 747-755
  • 16 Lenfeldt N, Larsson A, Nyberg L et al. Idiopathic normal pressure hydrocephalus: increased supplementary motor activity accounts for improvement after CSF drainage. Brain 2008; 131: 2904-2912
  • 17 Preuss M, Hoffmann KT, Reiss-Zimmermann M et al. Updated physiology and pathophysiology of CSF circulation-the pulsatile vector theory. Childs Nerv Syst 2013; 29: 1811-1825 DOI: 10.1007/s00381-013-2219-0.
  • 18 Andren K, Wikkelso C, Tisell M et al. Natural course of idiopathic normal pressure hydrocephalus. J Neurol Neurosurg Psychiat 2013; , DOI: DOI: 10.1136/jnnp-2013–306117.
  • 19 Tsakanikas D, Relkin N. Normal pressure hydrocephalus. Semin Neurol 2007; 27: 58-65
  • 20 Yakovlev P. Paraplegias of hydrocephalus (a clinical note and interpretation). Am J Mental Deficiency 1947; 51: 56-76
  • 21 Scheel M, Diekhoff T, Sprung C et al. Diffusion tensor imaging in hydrocephalus-findings before and after shunt surgery. Acta Neurochir (Wien) 2012; 154: 1699-1706 DOI: 10.1007/s00701–012–1377–2.
  • 22 Williams MA, Relkin NR. Diagnosis and management of idiopathic normal-pressure hydrocephalus. Neurol Clin Pract 2013; 3: 375-385
  • 23 Ambarki K, Israelsson H, Wahlin A et al. Brain ventricular size in healthy elderly: comparison between Evans index and volume measurement. Neurosurgery 2010; 67: 94-99 ; discussion 99
  • 24 Ishii K, Kanda T, Harada A et al. Clinical impact of the callosal angle in the diagnosis of idiopathic normal pressure hydrocephalus. Eur Radiol 2008; 18: 2678-2683
  • 25 Virhammar J, Laurell K, Cesarini KG et al. The callosal angle measured on MRI as a predictor of outcome in idiopathic normal-pressure hydrocephalus. J Neurosurg 2014; 120: 178-184
  • 26 Ivkovic M, Liu B, Ahmed F et al. Differential diagnosis of normal pressure hydrocephalus by MRI mean diffusivity histogram analysis. AJNR Am J Neuroradiol 2013; 34: 1168-1174
  • 27 Raftopoulos C, Chaskis C, Delecluse F et al. Morphological quantitative analysis of intracranial pressure waves in normal pressure hydrocephalus. Neurol Res 1992; 14: 389-396
  • 28 Droste DW, Krauss JK. Oscillations of cerebrospinal fluid pressure in nonhydrocephalic persons. Neurol Res 1997; 19: 135-138
  • 29 Aschoff A, Kremer P, Hashemi B et al. The scientific history of hydrocephalus and its treatment. Neurosurg Rev 1999; 22: 67-93 ; discussion
  • 30 Oikonomou J, Aschoff A, Hashemi B et al. New valves--new dangers? 22 valves (38 probes) designed in the “nineties in ultralong-term tests (365 days)”. Eur J Pediatr Surg 1999; 9 (Suppl. 01) 23-26
  • 31 Lemcke J, Meier U, Muller C et al. Safety and efficacy of gravitational shunt valves in patients with idiopathic normal pressure hydrocephalus: a pragmatic, randomised, open label, multicentre trial (SVASONA). J Neurol Neurosurg Psychiat 2013; 84: 850-857
  • 32 Meier U, Stengel D, Muller C et al. Predictors of subsequent overdrainage and clinical outcomes after ventriculoperitoneal shunting for idiopathic normal pressure hydrocephalus. Neurosurgery 2013; 73: 1054-1060
  • 33 Golz L, Lemcke J, Meier U. Indications for valve-pressure adjustments of gravitational assisted valves in patients with idiopathic normal pressure hydrocephalus. Surg Neurol Int 2013; 4: 140
  • 34 Bouras T, Sgouros S. Complications of endoscopic third ventriculostomy: a systematic review. Acta Neurochir Suppl 2012; 113: 149-153
  • 35 Wikkelso C, Hellstrom P, Klinge PM et al. 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 Psychiat 2013; 84: 562-568
  • 36 Speil A, Sosa JC, Will BE et al. Lack of correlation of overnight monitoring data and lumbar infusion data in iNPH patients. Acta Neurochir Suppl 2012; 114: 213-216
  • 37 Toma AK, Papadopoulos MC, Stapleton S et al. Systematic review of the outcome of shunt surgery in idiopathic normal-pressure hydrocephalus. Acta Neurochir (Wien) 2013; 155: 1977-1980
  • 38 Cage TA, Auguste KI, Wrensch M et al. Self-reported functional outcome after surgical intervention in patients with idiopathic normal pressure hydrocephalus. J Clin Neurosci 2011; 18: 649-654
  • 39 Bech-Azeddine R, Waldemar G, Knudsen GM et al. Idiopathic normal-pressure hydrocephalus: evaluation and findings in a multidisciplinary memory clinic. Eur J Neurol 2001; 8: 601-611
  • 40 Stolze H, Kuhtz-Buschbeck JP, Drucke H et al. Comparative analysis of the gait disorder of normal pressure hydrocephalus and Parkinson's disease. J Neurol Neurosurg Psychiat 2001; 70: 289-297
  • 41 Bouras T, Sgouros S. Complications of endoscopic third ventriculostomy. World Neurosurg 2013; 79: S22 9–12
  • 42 Chan AK, McGovern RA, Zacharia BE et al. Inferior short-term safety profile of endoscopic third ventriculostomy compared with ventriculoperitoneal shunt placement for idiopathic normal-pressure hydrocephalus: a population-based study. Neurosurgery 2013; 73: 951-960 ; discussion
  • 43 Pinto FC, Saad F, Oliveira MF et al. Role of endoscopic third ventriculostomy and ventriculoperitoneal shunt in idiopathic normal pressure hydrocephalus: preliminary results of a randomized clinical trial. Neurosurgery 2013; 72: 845-853 ; discussion