Neurologic Manifestations of Churg-Strauss Syndrome

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Objective

To determine the frequency and the types of neurologic involvement in a series of patients with Churg-Strauss syndrome (CSS).

Design

We reviewed the medical records of 47 consecutive patients with CSS who were examined at the Mayo Clinic between January 1974 and June 1992.

Material and Methods

The study patients were classified into two groups: (1) those with a histopathologically confirmed diagnosis of CSS who had evidence of either vasculitis or Churg-Strauss granuloma, the presence of asthma, and peripheral eosinophilia (more than 10% eosinophils) on at least one differential leukocyte count (N = 33) and (2) those with a clinical diagnosis of CSS who had evidence of vasculitis based on either multiple mononeuropathy or necrotizing cutaneous lesions, the presence of asthma, and peripheral eosinophilia (more than 10% eosinophils) on at least one differential leukocyte count (N = 14).

Results

Of the 47 patients, 29 (62%) had neurologic involvement. Peripheral neuropathy was detected in 25 patients: 17 had multiple mononeuropathy, 7 had distal symmetric polyneuropathy, and 1 had an asymmetric polyneuropathy. Three patients had cerebral infarctions. Less commonly identified problems included radiculopathies, ischemic optic neuropathy, and bilateral trigeminal neuropathy. Asthma preceded the onset of neurologic involvement in all cases (mean duration, 6.7 years). Follow-up data, when available, showed that corticosteroid therapy usually yielded improvement or stabilization.

Conclusion

Neurologic involvement is common in CSS, usually manifesting as peripheral neuropathy. In this series of patients, asthma preceded the neurologic manifestations.

Section snippets

MATERIAL AND METHODS

The criteria for patients with histopathologic confirmation of CSS consisted of asthma, peripheral eosinophilia, and a histopathologic finding of vasculitis (either fibrinoid necrosis of vessel walls or a perivascular inflammatory infiltrate), tissue eosinophil infiltration, or extravascular granulomas. Peripheral eosinophilia was defined as more than 10% eosinophils on at least one differential leukocyte count. Four patients had a history of eosinophilia before their visits to our institution,

RESULTS

From January 1974 through June 1992, 47 Mayo patients fulfilled the criteria for the diagnosis of CSS. A histopathologic diagnosis was made in 33 of the 47 cases, and a clinical diagnosis of CSS was made in 14 patients. The clinical information for these patients is summarized in Table 1.

DISCUSSION

Neurologic involvement affected 29 (62%) of our 47 patients with CSS, similar to the results in the studies by Chumbley and colleagues3 (63%) and Lanham and associates4 (75%). The most common manifestation in our series was peripheral neuropathy, which occurred in 25 of 29 patients with neurologic findings. The pattern of involvement and the temporal profiles of neurologic involvement in the two subgroups in our study—histopathologically confirmed cases of CSS and patients with a clinical

CONCLUSION

Our current study reveals the frequent occurrence of neurologic findings in patients with CSS. Further research on the prognosis and quality of life in patients with CSS and neurologic involvement is important for determining the benefits of early, aggressive corticosteroid therapy in these patients. The use of corticosteroid therapy early after the onset of asthma, but before development of neurologic manifestations, may decrease the frequency and severity of neurologic diseases.

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