INTRODUCTION Granulomatous inflammation is a distinctive ...

symmetrical polyneuropathy was seen in some patients, but peripheral neuropathy most often
manifests as acute mononeuritis multiplex. Cranial nerves II, VI, and VII are affected most
commonly (Nishino et al., 1993).
Clinical picture
The diagnosis of WG is suspected when patients present with constitutional symptoms such as
fever, weight loss, and anorexia with symptoms of upper respiratory tract affection chronic
sinusitis, nasal ulceration, other upper respiratory tract symptoms, or lower respiratory
symptoms of hemoptysis, dyspnea, or cough (Hoffman et al., 1992).
It is much less common for the patient to present with symptoms of renal involvement, although
renal involvement is often clinically evident at presentation. Manifestations include proteinuria,
hematuria, and renal insufficiency. Ocular disease is commonly manifested by conjunctivitis,
uveitis, dacrocystitis, retinitis, and/or proptosis due to orbital pseudotumor, which may cause loss
of vision in approximately 50% of patients with this complication (Paul, 2005).
Neurologic involvement manifestations are extremely varied, the most common neurological
presentation was peripheral neuropathy with mononeuritis multiplex or cranial neuropathy
involving the second, sixth, and seventh cranial nerves. Some patients has manifestations in the
CNS such as seizures, altered cognition (cerebritis), focal motor and sensory complaints, stroke
syndromes, or diabetes insipidus due to pituitary involvement. Presentations may involve chronic,
acute, or stepwise deterioration referable to parenchymal or meningeal inflammation and
scarring. A history of headaches and other symptoms related to inflammation of meningeal or
parenchymal structures should be sought initially and on follow up visits (Metwally, 2006-6).
Investigation
Abnormal findings on routine laboratory tests are nonspecific in WG. Abnormalities include
leukocytosis, thrombocytosis (>400,000/mm3), marked elevation of erythrocyte sedimentation rate
(ESR) and C-reactive protein (CRP), and normocytic and normochromic anemia.
Laboratory diagnosis of WG has been greatly aided by emergence of testing for c-antineutrophil
cytoplasmic antibodies (c-ANCA), which if present, is 97% specific for WG. Testing for c-ANCA
is 90% sensitive for the diagnosis when the presentation is classic involving both upper and lower
respiratory system and kidneys; sensitivity drops to 40% in limited WG (limited to only kidneys
or respiratory system) (Hagen et al., 1998).
Neuroimaging
In Wegener granulomatosis, MRI is superior to CT, and it shows variable findings. The meninges
are considered to be abnormal (involved by the disease) if they showed either diffuse or focal
thickening on contrast-enhanced MR images. Involvement of both the tentorium cerebelli and the
dura overlying the convexity of the cerebrum might occur in combination; however the tentorium
cerebelli might be the sole site of involvement (Figure 22, 23).
Figure 22. Wegener. Granulomatosis. Transverse T1-