Neurological involvement in Wegener's granulomatosis: An analysis of 324 consecutive patients at the Mayo Clinic

Abstract

Neurological involvement in Wegener's granulomatosis was studied by reviewing the charts of 324 consecutive patients in whom the diagnosis was made at the Mayo Clinic. One hundred nine patients (33.6%) had neurological involvement. Peripheral neuropathy occurred in 53; cranial neuropathy, in 21; external ophthalmoplegia, in 16; cerebrovascular events, in 13; seizures, in 10; cerebritis, in 5; and miscellaneous involvement, in 25. The mean age and sex ratio were similar in the patients with and those without neurological involvement. Among the patients with peripheral neuropathy, 42 had mononeuropathy multiplex; 6, distal symmetrical polyneuropathy; and 5, unclassified peripheral neuropathy. Multiple mononeuropathy was a major presenting symptom in 8 patients. A significantly higher percentage of patients with peripheral neuropathy, compared to those without peripheral neuropathy, had kidney involvement (p < 0.001). The second, sixth, and seventh cranial nerves were most frequently affected. Multiple cranial nerves were affected in 8 patients. Unusual neurological manifestations in the miscellaneous group were spastic paraparesis, temporal arteritis, Horner's syndrome, and papilledema.

Limited Wegener's granulomatosis with central nervous system involvement and fatal outcome

“…This may reveal the rapid course of the disease once there is a relapse, or demonstrate the limits of MRI in detecting microscopic lesions. In our patient, we could not confirm a close relationship between CNS vasculitis and haemorrhages at the autopsy microscopy, although intracerebral haemorrhages as a consequence of cerebral vasculitis have been described in WG [4,12,13]. However, previous vasculitic weakening of the vessel walls combined with the raised blood pressure during the grand mal seizures may well account for the multiple intracerebral bleedings.…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…The PNS is affected more commonly than the CNS, and usually manifests as polyneuropathy or mononeuritis multiplex due to inflammation of the vaso nervorum. CNS involvement generally takes the form of vasculitis and meningitis; it is rare and occurs in approximately 4% of patients [143]. Granulomatous invasion of the orbit with involvement of the cranial nerves, however, is much more common [144].…”

Horner's syndrome as manifestation of Wegener's granulomatosis

“…1,2 Reported neuro-ophthalmologic manifestations include palsies of the second, third, fourth, and sixth cranial nerves. 3 Horner's syndrome as manifestation of Wegener's granulomatosis is extremely rare: only five cases are reported. 3,4 Diseases such as Wegener's granulomatosis that have a broad range of clinical manifestations and no known aetiology may be difficult to diagnose.…”

“…3 Horner's syndrome as manifestation of Wegener's granulomatosis is extremely rare: only five cases are reported. 3,4 Diseases such as Wegener's granulomatosis that have a broad range of clinical manifestations and no known aetiology may be difficult to diagnose. According to the classification system of the American College of Rheumatology (1990, designed for categorization for clinical trials), a patient could be diagnosed with Wegener's granulomatosis if two or more of these criteria are present: (1) nasal or oral inflammation, (2) abnormal chest radiograph, (3) abnormal urine sediment, or (4) granulomatous inflammation on biopsy.…”