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Normaldruckhydrozephalus

Normal pressure hydrocephalus

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Zusammenfassung

Klinisches Problem

Der Normaldruckhydrozephalus („normal pressure hydrocephalus“, NPH) ist eine Erkrankung des älteren Menschen (ab der 6. Dekade) mit steigender Prävalenz im Alter und gehört zu den wenigen behandelbaren Demenzursachen. Unbehandelt führt der NPH häufig zu schweren motorischen, psychomotorischen und irreversiblen kognitiven Defiziten. Die Pathogenese der Erkrankung ist bis heute nicht vollständig geklärt. Klinisch zeigt die Erkrankung einen langsamen Progress mit Gleichgewichts-/Gangstörungen als Hauptsymptom, später folgen Urininkontinenz und kognitive Einbußen. Differenzialdiagnostische Schwierigkeiten bereitet die Abgrenzung zur Parkinson-Krankheit (ähnliches Gangbild), Alzheimer- und vaskulären Demenz, nicht zuletzt wegen der häufigen Komorbidität.

Radiologische Standardverfahren

Radiologisches Standardverfahren zur NPH-Abklärung ist die konventionelle Schnittbildgebung (CT oder MRT), hier liegt definitionsgemäß eine Ventrikulomegalie vor (Cella-media-Index < 4, Evans-Index > 0,3), in klassischen Fällen ein Mismatch zwischen weiter sylvischer Fissur und engen Konvexitätszisternen („DESH-pattern“, DESH „disproportionately enlarged subarachnoid-space hydrocephalus“). Radiologisch wichtig ist die Abgrenzung zur Atrophie.

Methodische Innovationen

Spezielle MRT-Techniken lassen nähere Aussagen über den Liquorfluss zu, haben sich aber bislang nicht als Standard in den Leitlinien zur NPH-Diagnostik durchgesetzt.

Bewertung/Empfehlung

Neben der konventionellen Schnittbildgebung sind klinische Tests, z. B. Ganganalyse und neuropsychologische Untersuchung sowie invasiv diagnostische Tests wie Liquorablassversuche und Lumbaldrainagen etablierte Verfahren und geben präoperativ Aufschluss über den möglichen Erfolg einer Shuntoperation. Bei differenzierter Patientenselektion gilt die ventrikuloperitoneale Shuntoperation, auch dank Weiterentwicklungen der Shuntventiltechnik, als einzige bewährte NPH-Therapie mit hohen Erfolgsraten.

Abstract

Clinical issue

Normal pressure hydrocephalus (NPH) is a disorder found mainly in the elderly (> 60 years) with an increasing prevalence with age and is one of the few treatable causes of dementia. If untreated NPH often leads to severe motor, psychomotor and irreversible cognitive deficits. The pathogenesis is not yet fully understood. Clinical symptoms consist of the (not always complete) classical triad of equilibrium and gait disturbances followed later by incontinence and dementia. Symptoms often show a gradual progression to irreversibility in non-treated patients; therefore, early diagnosis and treatment are mandatory. Important differential diagnoses are Parkinson’s disease (similar gait), Alzheimer’s disease and vascular dementia, not least due to the high comorbidity of these conditions with NPH.

Standard radiological methods

The standard radiological method for evaluation of NPH is conventional cross-sectional imaging that typically shows ventriculomegaly (Evans’ index > 0.3 and cella media index < 4) often combined with the so-called disproportionately enlarged subarachnoid space hydrocephalus (DESH) pattern (tight convexity sulci and enlarged sylvian fissure). These findings should be differentiated from ventriculomegaly in atrophy combined with enlarged convexity sulci.

Methodical innovations

Special magnetic resonance imaging (MRI) techniques can be used to evaluate cerebrospinal fluid (CSF) flow but are not yet part of the diagnostic guidelines.

Achievements/practical recommendations

Combined with cross-sectional imaging, well-established clinical and invasive diagnostic tests, such as repeated spinal tap or lumbar drainage with re-evaluation of clinical symptoms lead to a diagnosis and help with preoperative patient selection for CSF diversion. Ventriculoperitoneal CSF shunting has proven to be safe and is the only known successful therapy for NPH.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. J.M. Lieb, C. Stippich und F.J. Ahlhelm geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Abteilung für diagnostische und interventionelle Neuroradiologie, Klinik für Radiologie und Nuklearmedizin, Universitätsspital Basel, Petersgraben 4, 4031, Basel, Schweiz

    J.M. Lieb & C. Stippich

  2. Abteilung für Neuroradiologie, Institut für Radiologie, Kantonspital Baden AG, Baden, Schweiz

    F.J. Ahlhelm

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  1. J.M. Lieb
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  2. C. Stippich
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  3. F.J. Ahlhelm
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Correspondence to J.M. Lieb.

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Lieb, J., Stippich, C. & Ahlhelm, F. Normaldruckhydrozephalus. Radiologe 55, 389–396 (2015). https://doi.org/10.1007/s00117-014-2797-1

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