Idiopathische intrakranielle Hypertension (Pseudotumor cerebri)

Zusammenfassung

Anhaltende Kopfschmerzen sowie eine Sehstörung mit Visusverlust bei Papillenstauung und Gesichtsfeldeinschränkung sind die führenden klinischen Symptome der idiopathischen intrakraniellen Hypertension bzw. des Pseudotumor cerebri. Es handelt sich um eine Ausschlussdiagnose, bildgebende Untersuchungen und Liquor zeigen unauffällige Befunde, jedoch ist der Liquoreröffnungsdruck erhöht. Ein sorgfältiger Ausschluss einer sekundären intrakraniellen Hypertension durch Raumforderung, eine Thrombose der Hirnsinus, Liquorzirkulationsstörungen, chronisch infektiöse oder tumoröse meningeale Reizung oder medikamentös-toxische Auslöser muss immer erfolgen. Die Therapie der idiopathischen intrakraniellen Hypertension besteht aus einer konsequenten Gewichtsreduktion, der medikamentösen Therapie mit dem Carboanhydrasehemmer Azetazolamid oder des Diuretikums Furosemid. Auch Topiramat kann zum Einsatz kommen. Bei einem kleinen Teil der Patienten sind chirurgische Eingriffe (Fensterung der Optikusscheide, Shuntanlage, Adipositaschirurgie) notwendig, wenn es mit konservativer Behandlung nicht gelingt eine Verbesserung der Kopfschmerzen und der Sehstörung zu erreichen.

Abstract

Persistent hedache with impaired vision and loss of visual acuity due to papilloedema and visual field defect are the leading signs and symptoms of idiopathic intracranial hypertension (IIH). IIH is a diagnosis of exclusion. Neuroimaging and cerebrospinal fluid findings are unremarkable except for elevated CSF opening pressure at lumbar puncture. Secondary causes of IIH such as intracranial tumours, venous sinus thrombosis, chronic inflammatory, infectious meningeal irritation and carcinomatous meningitis or pharmacogenic toxic reaction should be ruled out carefully. Therapy for IIH consists of consequent weight reduction and medical treatment with the carbonic anhydrase inhibitor azetazolamide or diuretic therapy with furosemide. Topiramate may be effective as well. In a minority of the patients that are refractory to conservative medical treatment surgical therapy is recommended (optic nerve sheat fenestration, CSF diversion procedures, bariatric surgery).

Schlüsselwörter

idiopathisch intrakranielle Hypertension - Adipositas - Therapie - Diagnostik

Keywords

idiopathic intracranial hypertension - obesity - therapy - diagnostics

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