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Hypertrophe kraniale Pachymeningitis als seltene Ursache für Kopfschmerzen

Hypertrophic cranial pachymeningitis as a rare cause of headache

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Zusammenfassung

Die hypertrophe kraniale Pachymeningitis (HP) ist selten und wurde erst durch die magnetresonanztomographische Diagnostik besser bekannt. Das Krankheitsbild ist gekennzeichnet durch eine fibröse und entzündliche Verdickung der Dura mater, die zu Kompressionen nervaler Strukturen führt. Das Kardinalsymptom ist der Kopfschmerz. Die weitere Symptomatik besteht vorwiegend in Hirnnervenläsionen und zerebellärer Ataxie. Am häufigsten tritt die Erkrankung idiopathisch auf, in zweiter Linie liegen ihr autoimmunologische Prozesse und erregerbedingte Erkrankungen zugrunde. Die Diagnose stützt sich auf die Magnetresonanztomographie, ist aber definitiv nur bioptisch beweisbar. Die idiopathische Form und auch die sekundären Manifestationen sprechen sehr gut auf hochdosierte Kortikosteroide an, des Weiteren auf Immunsuppressiva. Die erregerbedingte sekundäre HP wird antibiotisch behandelt. Die Übersicht handelt den aktuellen Wissensstand der HP in Hinsicht auf Ätiologie, klinische Symptomatik, Diagnostik und Therapie ab.

Summary

Hypertrophic cranial pachymeningitis is an uncommon, fibrosing, inflammatory process that involves the dura mater. The condition is being reported more frequently owing to the use of cranial MRI. The main clinical feature is headache, whereas cranial nerve lesions, cerebellar symptoms, and epileptic seizures occur more rarely. A variety of autoimmune and infectious diseases can result in this condition, which is labeled as idiopathic in the absence of any definite inciting factor. The diagnosis of hypertrophic cranial pachymeningitis is based on neuroimaging of thickened and enhancing dura mater. It can be defined pathologically on biopsy. A specific treatment is indicated in some cases of secondary hypertrophic cranial pachymeningitis. Mostly, treatment relies on corticosteroids and immunosuppressive agents. This review summarizes the current knowledge on causes, clinical presentation, diagnosis, and treatment of this disorder.

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Authors and Affiliations

  1. Neurologische Klinik, Krankenhaus Merheim, Kliniken der Stadt Köln gGmbH

    J. Kuhn, W. Müller & H. Bewermeyer

  2. Radiologische Klinik, Krankenhaus Merheim, Kliniken der Stadt Köln gGmbH

    A. Harzheim

  3. Department of Neurology, Social Insurance Chukyo Hospital, Minami, Nagoya, Japan

    S. Riku

  4. Neurologische Klinik, Krankenhaus Merheim, Kliniken der Stadt Köln gGmbH, Ostmerheimer Straße 200, 51109, Köln, Deutschland

    J. Kuhn

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Kuhn, J., Harzheim, A., Riku, S. et al. Hypertrophe kraniale Pachymeningitis als seltene Ursache für Kopfschmerzen. Nervenarzt 77, 423–429 (2006). https://doi.org/10.1007/s00115-005-2012-8

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