Severe polyneuropathy in a patient with Churg-Strauss syndrome

Paola Bazzi, Lucia Tancredi, Elio Scarpini, Stefano Messina, Monica Sciacco, Simona Livraghi, Massimo Vanoli, Alessandro Prelle, Guglielmo Scarlato, Maurizio Moggio

Research output: Contribution to journalArticle

Abstract

We describe the clinicopathologic features of a 56-year-old woman affected with Churg-Strauss syndrome with major peripheral nerve involvement. The patient presented with a 1-month history of mainly distal upper-limb symmetrical paresthesias and hypostenia (bilateral 'wrist drop'), palpable purpura and eosinophilia. Multiple pulmonary infiltrates and asthma had been present since the age of 52. Skin biopsy demonstrated an eosinophilic necrotizing vasculitis. During the hospitalization she was submitted to cardiac, bronchopulmonary, renal, and gastrointestinal evaluation and EMG. Peripheral nerve and skeletal muscle biopsies were performed. Sural nerve biopsy showed a marked degree of demyelination. A perivascular cellular infiltrate within the epineurium was immunoreactive for T lymphocytes and macrophages. Strong HLA-DR immunostaining was present in the endoneurium. IgM, IgE and fibrinogen deposition was found in some epi- and endoneurial vessels. Muscle biopsy showed neurogenic changes and 1 thrombosed vessel surrounded by mononuclear cells. Membrane attack complex (MAC) deposition was present in a few capillaries and major histocompatibility complex products I (MHCP I) was expressed at the subsarcolemmal level in a few isolated perivascular muscle fibers. After immunosuppressive therapy, the patient showed progressive improvement of both clinical symptoms and neurophysiological parameters.

Original languageEnglish
Pages (from-to)106-110
Number of pages5
JournalJournal of the Peripheral Nervous System
Volume5
Issue number2
DOIs
Publication statusPublished - Jun 2000

Keywords

  • Churg-Strauss syndrome
  • Myositis
  • Peripheral neuropathy
  • Vasculitis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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    Bazzi, P., Tancredi, L., Scarpini, E., Messina, S., Sciacco, M., Livraghi, S., Vanoli, M., Prelle, A., Scarlato, G., & Moggio, M. (2000). Severe polyneuropathy in a patient with Churg-Strauss syndrome. Journal of the Peripheral Nervous System, 5(2), 106-110. https://doi.org/10.1046/j.1529-8027.2000.00010.x