Wegener’s Granulomatosis presenting with multiple cranial neuropathies.

Read printer friendly
 Subscribe in a reader

Share with others

 

Max R. Lowden MD
Department of Neurology, Penn State College of Medicine, Milton S. Hershey Medical Center, 30 Hope Drive, MC EC037, Hershey, Pennsylvania 17033

Address:

Aiesha Ahmed MD
Department of Neurology, Penn State College of Medicine, Milton S. Hershey Medical Center, 30 Hope Drive, MC EC037, Hershey, Pennsylvania 17033

Address:

Citation: M. Lowden & A. Ahmed : Wegener’s Granulomatosis presenting with multiple cranial neuropathies.. The Internet Journal of Neurology. 2009 Volume 11 Number 2

Keywords: Wegener’s Granulomatosis | intra cranial pachymeningitis | all neuro otology | all clinical neurology

A 41–year-old man had headaches, hearing loss, and left facial palsy for five months. Examination was remarkable for left seventh nerve palsy, left sided hearing loss and hemi tongue atrophy. MRI showed left cerebellopontine angle dural thickening.(Figure1and 2). Laboratory studies (CSF and blood) were unremarkable for an infectious, neoplastic and autoimmune etiology except for an elevated Anti-neutrophil cytoplasmic antibody titer. A dural biopsy showed chronic inflammation with granuloma formation. He was started on prednisone with plans to start immunosuppresants as outpatient.

Wegener’s Granulomatosis can present with multiple cranial neuropathies as initial clinical manifestation¹ due to focal meningeal involvement ².

Figure1. Coronal MRI T1–weighted image with gadolinium (A and B) showing dural thickening and enhancement along the left cerebellopontine angle and surrounding the left seventh and eighth nerves.

Figure 2. Axial MRI T1–weighted image with gadolinium (A and B) showing dural thickening and enhancement along the left cerebellopontine angle. There is also noted surrounding inflammation extending into the left external auditory canal.

Correspondence to

Max R. Lowden, MD Department of Neurology, The Pennsylvania State University Milton S. Hershey Medical Center 30 Hope Drive Hershey, PA 17033.Tel:(717) 531-0003 x283934; FAX (717)531-0384.
E mail: mlowden@hmc.psu.edu.

References

1. Nagashima T, Maguchi S, Terayama Y, et al. P-ANCA-positive Wegener's granulomatosis presenting with hypertrophic pachymeningitis and multiple cranial neuropathies: case report and review of literature.Neuropathology. March 2000; 20(1):23-30. (s)

2. Di Comite G, Bozzolo EP, Praderio L, Tresoldi M, Sabbadini MG. Meningeal involvement in Wegener's granulomatosis is associated with localized disease. Review Clinical & Experimental Rheumatology.2006; 24(2 Suppl 41):S60-4. (s)

This article was last modified on Thu, 22 Oct 09 19:39:09 -0500

This page was generated on Fri, 18 Dec 09 07:01:38 -0600, and may be cached.