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Neurological pictures
Idiopathic hypertrophic cranial pachymeningitis: a rare but treatable cause of headache and facial pain
  1. Michael Khalil1,
  2. Franz Ebner2,
  3. Franz Fazekas1,
  4. Christian Enzinger1,2
  1. 1Department of Neurology, Medical University of Graz, Graz, Austria
  2. 2Division of Neuroradiology, Department of Radiology, Medical University of Graz, Graz, Austria
  1. Correspondence to Dr Michael Khalil, Department of Neurology, Medical University of Graz, Auenbruggerplatz 22, A-8036 Graz, Austria; michael.khalil{at}medunigraz.at

https://doi.org/10.1136/jnnp-2012-303295

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    Introduction

    Idiopathic Hypertrophic Cranial Pachymeningitis (IHCP) is a rare disease with pain and compression related cranial nerve dysfunction as main clinical features. The leading diagnostic finding of IHCP consists of diffuse or localised thickening of the dura, which demands appropriate imaging and image interpretation. This case description aims at increasing the awareness for the clinical symptoms and imaging findings of this rare disease to allow prompt diagnosis and treatment initiation.

    Case description

    An 82-year-old man presented with recurrent left sided headache and worsening facial pain, which had begun more than 1 year ago. Neurological examination at presentation revealed ptosis of the left eye and gaze-induced nystagmus when looking to the left; visual function was intact and no other neurological signs or symptoms were noted. Pre-existing medical conditions included atrial arrhythmia requiring treatment with oral anticoagulants and arterial hypertension. External brain MRI at …

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    Footnotes

    • Disclosure MK has received research support from the Austrian Science Fund (FWF) (J2992-B09). FF serves on the scientific advisory boards for Bayer Schering, Biogen Idec, Merck Serono, Novartis and Teva Pharmaceutical Industries/Sanofi-Aventis; serves on the editorial boards of Cerebrovascular Diseases, Multiple Sclerosis, the Polish Journal of Neurology and Neurosurgery, Stroke, and the Swiss Archives of Neurology and Psychiatry; and has received speaker honoraria from Biogen Idec, Bayer Schering, Merck Serono, Novartis and Sanofi-Aventis. CE has received speaker honoraria from Biogen Idec, Bayer Schering, Merck Serono and Sanofi-Aventis and has obtained unrestricted research grants from Merck Serono and Biogen-Idec.

    • Competing interests None.

    • Ethics approval All data for this report were obtained during routine diagnostic procedures. The MRI images presented are anonymised, and this report does not contain any information that may identify the patient.

    • Provenance and peer review Not commissioned; externally peer reviewed.