Die Diagnose und Behandlung des Normaldruckhydrozephalus

Zusammenfassung

In der Diagnostik des Normaldruckhydrozephalus als Ursache von demenzieller Entwicklung, Gangstörungen und Inkontinenz im Alter ist die frühzeitige Erkennung von besonderer Bedeutung, da die Shunttherapie die Symptomatik wesentlich bis vollständig beseitigen kann. Die Pathophysiologie ist bislang unverstanden. In der differenzialdiagnostischen Abgrenzung des idiopathischen Normaldruckhydrozephalus spielen vaskuläre Demenz im Rahmen einer subkortikalen arteriosklerotischen Enzephalopathie, der Morbus Alzheimer und die Parkinson-Erkrankung wesentliche Rollen, insbesondere beim Vorliegen einer Komorbidität. Selbst bei Vorliegen der typischen Symptomtrias und beim Nachweis einer eindeutigen Seitenventrikelerweiterung im CT oder MRT können im Einzelfall diagnostische Unsicherheiten bestehen, sodass akzessorische Testverfahren zur Sicherung der Diagnose und des Therapieerfolgs eingesetzt werden. Der einfachste Test, der Liquorablassversuch (spinaler „Tap-Test”) kann durch eine klinische Besserung den Erfolg einer Shuntimplantation präjudizieren. Der direkte Nachweis der bei diesem Krankheitsbild vorliegenden Hirndruckschwankungen wahrscheinlich im Rahmen einer gestörten Liquorresorption wird mit invasiven Langzeitdruckmessungen über mehrere Tage geführt. Mithilfe spinaler Infusionstests wird eine pathologische intrakranielle Druck-Volumen-Beziehung erfasst. Das heterogene Patientengut, nicht standardisierte Testverfahren, Mitbeteiligung zerebrovaskulärer Prozesse, verschiedene Shunttechniken und andere Faktoren haben dazu geführt, dass die Erfolge der Shunttherapie in einem Bereich von 30 - 90 % variieren. Ziel der nächsten Jahre muss es sein, durch kritische und frühzeitige Selektion der Patienten, welche von einer Shunttherapie profitieren, möglichst alle NPH-Patienten zu identifizieren, aber auch nur diejenigen Patienten invasiv zu therapieren, die hiervon bei vertretbarem Interventionsrisiko profitieren.

Abstract

Early diagnosis and treatment of normal pressure hydrocephalus, a syndrome of cognitive impairment, gait disturbances and incontinence in the elderly has become important as shunt treatment may bring about the complete alleviation of symptoms in these patients. Pathophysiology, however, still lacks a clear understanding. There is emphasize on the differential diagnosis of idiopathic NPH including vascular dementia due to cerebrovascular disease, Alzheimer's Disease and Parkinson's Disease among the most important, which could resemble or may even co-exist with iNPH. Even in the presence of the typical „clinical triad” and of an enlarged ventricular system as evidenced by CT and MRI, uncertainty remains with regard to establishing the diagnosis of NPH. Therefore, additional testing is required in order to support the diagnosis and to identify patients which might benefit from shunting. Most simply, cerebrospinal fluid (CSF-) withdrawal (spinal „tap-test”) has increased certainty about „shunt-responsive” NPH, moreover, the occurrence of intracranial pressure variations obtained by invasive long-term pressure measurement has indicated a disturbed CSF-absorption. Furthermore, the spinal infusion test has evidenced an impaired intracranial pressure-volume-relationship in these patients. The heterogeneity of the patient population, the lack of standardized tests procedures, co-morbid conditions such as cerebrovascular disease and different shunt techniques are but some of the most important factors, which have contributed to a highly variable response to shunt treatment ranging from 30 - 90 %. Future efforts should focus on a timely and critical identification and treatment of those NPH patients, which are most likely to improve by shunting, taking the individual benefits and risks of treatment into consideration.

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