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Severe polyneuropathy in a patient with Churg‐Strauss syndrome
Author(s) -
Bazzi Paola,
Tancredi Lucia,
Scarpini Elio,
Messina Stefano,
Sciacco Monica,
Livraghi Simona,
Vanoli Massimo,
Prelle Alessandro,
Scarlato Guglielmo,
Moggio Maurizio
Publication year - 2000
Publication title -
journal of the peripheral nervous system
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1
H-Index - 67
eISSN - 1529-8027
pISSN - 1085-9489
DOI - 10.1046/j.1529-8027.2000.00010.x
Subject(s) - medicine , pathology , endoneurium , nerve biopsy , muscle biopsy , eosinophilia , epineurium , biopsy , polyneuropathy , peripheral neuropathy , anatomy , peripheral nerve , endocrinology , diabetes mellitus
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.