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 language | English |
|---|---|
| Pages (from-to) | 106-110 |
| Number of pages | 5 |
| Journal | Journal of the Peripheral Nervous System |
| Volume | 5 |
| Issue number | 2 |
| DOIs | |
| Publication status | Published - Jun 2000 |
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Keywords
- Churg-Strauss syndrome
- Myositis
- Peripheral neuropathy
- Vasculitis
ASJC Scopus subject areas
- Clinical Neurology
- Neuroscience(all)
Cite this
Severe polyneuropathy in a patient with Churg-Strauss syndrome. / Bazzi, Paola; Tancredi, Lucia; Scarpini, Elio; Messina, Stefano; Sciacco, Monica; Livraghi, Simona; Vanoli, Massimo; Prelle, Alessandro; Scarlato, Guglielmo; Moggio, Maurizio.
In: Journal of the Peripheral Nervous System, Vol. 5, No. 2, 06.2000, p. 106-110.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Severe polyneuropathy in a patient with Churg-Strauss syndrome
AU - Bazzi, Paola
AU - Tancredi, Lucia
AU - Scarpini, Elio
AU - Messina, Stefano
AU - Sciacco, Monica
AU - Livraghi, Simona
AU - Vanoli, Massimo
AU - Prelle, Alessandro
AU - Scarlato, Guglielmo
AU - Moggio, Maurizio
PY - 2000/6
Y1 - 2000/6
N2 - 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.
AB - 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.
KW - Churg-Strauss syndrome
KW - Myositis
KW - Peripheral neuropathy
KW - Vasculitis
UR - http://www.scopus.com/inward/record.url?scp=0033920626&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0033920626&partnerID=8YFLogxK
U2 - 10.1046/j.1529-8027.2000.00010.x
DO - 10.1046/j.1529-8027.2000.00010.x
M3 - Article
VL - 5
SP - 106
EP - 110
JO - Journal of the Peripheral Nervous System
JF - Journal of the Peripheral Nervous System
SN - 1085-9489
IS - 2
ER -