Neurologic Manifestations of Churg-Strauss Syndrome

Sehgal, Manu; Swanson, Jerry W.; DeRemee, Richard A.; Colby, Thomas V.

doi:10.4065/70.4.337

Cited by 197 publications

(117 citation statements)

References 15 publications

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“…According to our study and previous reports [4,6,9], many CSS patients develop neuropathy at higher middle age, we also observed no gender bias in our patients. The initial symptom and expanding pattern was consistent with previous reports [9].…”

Section: Discussionsupporting

confidence: 87%

“…There are not many articles describing CSS-associated neuropathy. These reports describe CSS-associated neuropathy in the relation to systemic small-vessel vasculitis, Wegener's granulomatosis and microscopic polyangiitis [4,5], or with central nervous system involvement [6], or present a single case [7,8];…”

Section: Introductionmentioning

confidence: 99%

“…The mean onset age of neuropathy was about 50 years old, but the individual age of onset was wide-ranged, 15-78 years old [4,6,9]. There is controversy over the presence of a gender bias as the female ratio of CSS-associated neuropathy patients was reported to be 26.7 % [4], 55.3 % [6], and 78.6 % [9].…”

Section: Introductionmentioning

confidence: 99%

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Clinical characterization and successful treatment of 6 patients with Churg–Strauss syndrome-associated neuropathy

Nakamura

Yabe

Yaguchi

et al. 2009

Clinical Neurology and Neurosurgery

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Objective: To confirm the reported findings and clarify unknown clinical features of Churg-Strauss syndrome (CSS) -associated neuropathy and design appropriate treatment.Patients and Methods: We assessed the clinical features of 6 patients with CSS-associated neuropathy.Results: Mononeuritis multiplex was present in 4 cases and polyneuropathy in the remaining cases. Both groups progressed to sensori-motor polyneuropathy in an acute or subacute course. All cases showed bronchial asthma and eosinophilia. Two cases with serum antineutrophil cytoplasmic antibodies to myeloperoxidase (MPO-ANCA) had an acute clinical course and severe symptoms. Nerve conduction studies (NCS) of these 2 cases revealed conduction blocks at the initial stage, although NCS finally indicated sensori-motor axonopathy at the involved extremities. For treatment, high-dose corticosteroid therapy for 4 cases, and cyclophosphamide combined with corticosteroids for one case, were effective. For the remaining case, intravenous immunoglobulin (IVIg) at the chronic phase resulted in a slow improvement of neuropathy in the symptomatic aspect. There was no relapse of neuropathy with low dose corticosteroid treatment for 14-24 months after the initial treatment, except one case. There was also no relapse in the other case that was treated with moderate-dose steroids.Conclusion: Our study showed that CSS-associated neuropathy is a treatable disorder and that the first choice therapy is high-dose corticosteroid. In cases where corticosteroids are ineffective or for severe cases, immunosuppressive therapy (cyclophosphamide) with steroids should be considered, and IVIg might be a treatment option.M. Nakamura et al 3

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Section: Discussionsupporting

confidence: 87%

Section: Introductionmentioning

confidence: 99%

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Clinical characterization and successful treatment of 6 patients with Churg–Strauss syndrome-associated neuropathy

Nakamura

Yabe

Yaguchi

et al. 2009

Clinical Neurology and Neurosurgery

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“…CNS involvement is seen in approximately 6 to 8% of patients with CSS and cerebral infarction, as well as intracerebral and subarachnoid hemorrhage being reported [160][161][162]. Patients with active disease, especially with neurological involvement, should undergo induction therapy with corticosteroids and cyclophosphamide [146].…”

Section: Cssmentioning

confidence: 99%

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Geldern¹,

McPharlin

Becker

2012

Neurotherapeutics

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This chapter will review the spectrum of immunemediated diseases that affect the nervous system and may result in an admission to the neurological intensive care unit. Immunomodulatory strategies to treat acute exacerbations of neurological diseases caused by aberrant immune responses are discussed, but strategies for long-term immunosuppression are not presented. The recommendations for therapeutic intervention are based on a synthesis of the literature, and include recommendations by the Cochrane Collaborative, the American Academy of Neurology, and other key organizations. References from recent publications are provided for the disorders and therapies in which randomized clinical trials and large evidenced-based reviews do not exist. The chapter concludes with a brief review of the mechanisms of action, dosing, and side effects of commonly used immunosuppressive strategies in the neurocritical care unit.

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“…Cerebral infarction is an uncommon manifestation of CSS, occurring in less than 10 % of patients in one series [25], and is thought to result from vasculitis. When vasculitis presents as infarcts, MRI usually shows multiple, bilateral lesions involving both gray and white matter [8], as seen in the present case.…”

Section: Bone Marrow Biopsymentioning

confidence: 99%