1/24. Severe polyneuropathy in a patient with churg-strauss syndrome.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.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/24. Allergic granulomatous angiitis (churg-strauss syndrome) associated with allergic bronchopulmonary candidiasis.We describe a case of churg-strauss syndrome (CSS) associated with allergic bronchopulmonary candidiasis (ABPC). A 61-year-old man who had been given a diagnosis of ABPC based on serologic and radiographic findings experienced pain and purpuric rash on the left leg accompanied with motor weakness. The diagnosis of CSS was made from skin, nerve and muscle biopsies. Although immunosuppressant and prednisolone were administered and resulted in transient improvement, candidal pneumonia was suspected to have developed 60 days after the administration and the patient finally died of respiratory failure. To our knowledge, this is the first case of CSS associated with ABPC.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
3/24. Childhood churg-strauss syndrome: report of a case.churg-strauss syndrome (CSS) (allergic granulomatosis and angitis) is an uncommon form of systemic vasculitis, which is rare in children. It is characterized by peripheral blood hypereosinophilia, systemic necrotizing vasculitis, and a preceding history of bronchial asthma. We described a boy with initial presentation of poorly controlled bronchial asthma, allergic rhinitis, recurrent sinusitis and several episodes of hemoptysis since the age of 9. He then developed purpuric skin lesions, generalized soreness, and symptoms of mononeuritis multiplex at age 11. On admission to our hospital at the age of 12, he developed marked pericardial effusion. After a series of studies including chest computed tomography (CT), skin biopsy, nerve conduction study, and serological tests for autoantibodies, CSS was diagnosed. Thereafter, he received regular corticosteroid therapy, and his symptoms were generally well-controlled with occasional acute exacerbation. The clinical characteristics, diagnosis and management of CSS in children are also reviewed.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
4/24. "Pseudo-conduction block" in a patient with vasculitic neuropathy.A 63-year-old man presented with progressive asymmetric weakness and numbness in his hands of 2 weeks duration. Nerve conduction studies showed low amplitude motor evoked potentials of both median nerves. The right ulnar, left tibial and peroneal nerves had normal potentials on distal stimulation with markedly decreased amplitudes proximally, suggestive of "conduction block". Three weeks later, amplitudes were decreased throughout. The patient was diagnosed with vasculitis. The acute ischemic injury presumably resulted in axonal damage between the distal and proximal stimulation sites, with subsequent wallerian degeneration.- - - - - - - - - - ranking = 0.66666666666667keywords = nerve (Clic here for more details about this article) |
5/24. Clinicopathological studies of peripheral neuropathy in churg-strauss syndrome.Clinicopathological studies were performed on the visceral organs and the sural nerve of a male patient with churg-strauss syndrome (CSS) in order to understand the mechanisms of peripheral nervous system damage. A 67-year-old man, with a 2-year history of bronchial asthma, developed acutely painful paraplegia and dyspnea. Laboratory data showed a leukocytosis, an elevated serum creatinine kinase (CK) and marked eosionophilia. autoantibodies including p- and c-ANCA were negative. Electrophysiological studies revealed a severe sensory-motor neuropathy of multiple mononeuritis type. Steroid pulse therapy performed a day after biopsy of skin, muscle and sural nerve was effective in resolving his respiratory and neurological dysfunction but a perforation of an intestinal ulcer occurred which required surgical intervention. In the biopsied sural nerve and the surgically resected intestine and mesentery there was vasculitis with fibrinoid necrosis accompanied by numerous eosinophils and macrophages containing eosinophil cationic protein (ECP). These findings suggest that in addition to ischemic changes due to vasculitis some neurotoxic substances generated by the eosinophils may be involved in the development of neuropathy in CSS.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
6/24. churg-strauss syndrome: diagnostic difficulties and pathogenesis.A 52-year-old woman with a history of chronic obstructive pulmonary disease presented with symmetrical polyarthritis involving her metacarpophalangeal and proximal interphalangeal joints, knees, ankles, and hips and with a purpuric rash involving her lower extremities. She had a history of recurrent episodes of purulent otitis often associated with myalgias and arthralgias. Laboratory studies at presentation included leukocytosis with 16% eosinophils, an elevated rheumatoid factor titer, and an elevated antineutrophil cytoplasmic antibody titer. Cultures from the right ear canal grew. skin biopsy revealed leukocytoclastic vasculitis with pericapillary eosinophils. The patient was treated with prednisone and then with azathioprine after the rash relapsed during the tapering of prednisone. Four months after her initial presentation, she developed bilateral foot drop. A sural nerve biopsy revealed vasa nervosum vasculitis. The diagnosis of churg-strauss syndrome was established, and she was treated with an increased dose of azathioprine and a slowly tapering prednisone regimen. This case report suggests that patients with churg-strauss syndrome can present with a syndrome suggesting rheumatoid arthritis. In this particular patient, recurrent staphylococcal infections may have triggered the vasculitic process.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
7/24. A case of cutaneous sensory neuropathy associated with churg-strauss syndrome.Cutaneous sensory neuropathy manifests as multiple, sharply demarcated areas of hypoesthesia with a variable degree of pain. This rare neuropathy is caused by a multifocal infection or inflammation of the small sensory nerves of the skin. We report a case in a patient with febrile arthritis and eosinophilia. Her chronic cough and the presence of extravascular infiltrates of eosinophils in a neuromuscular biopsy specimen suggested churg-strauss syndrome. The course was favorable under corticosteroid therapy.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
8/24. Asymptomatic optic neuropathy associated with churg-strauss syndrome.OBJECTIVE: To report asymptomatic optic disc edema in a patient with churg-strauss syndrome. DESIGN: Retrospective, observational case report. methods: Neuro-ophthalmic examination, sural nerve biopsy, and laboratory evaluation. MAIN OUTCOME MEASURES: visual acuity, funduscopy, automated perimetry, and histopathology of sural nerve biopsy. CONCLUSIONS: churg-strauss syndrome may be associated with optic neuropathy. Careful ophthalmic evaluation may reveal signs of optic neuropathy before the development of symptomatic vision loss.- - - - - - - - - - ranking = 0.66666666666667keywords = nerve (Clic here for more details about this article) |
9/24. asthma associated with worsening leg ulcer: a case of vasculitis in primary care.A 71-year-old black woman was admitted to the hospital with a 2-month history of a nonhealing leg ulcer. Her medical history included diabetes mellitus type 2, congestive heart failure, allergic rhinitis, and asthma. The patient's asthma was labile and steroid-dependent until 2 years before admission, at which time zafirlukast therapy was started. On further questioning, the patient revealed a 6-month history of malaise and a 40-lb weight loss. A physical examination showed a 2-cm Stage 3 ulcer on the medial aspect of the right ankle with diminished sensation in both feet and left footdrop. electromyography revealed mononeuritis multiplex. The patient's white blood cell count was 11,100/mm3 with 60% eosinophils. A sural nerve biopsy showed vasculitis consistent with churg-strauss syndrome. One week after prednisone therapy was initiated, the patient's foot strength was nearly normal and her eosinophilia had resolved. Although churg-strauss syndrome is a rare disorder, in the setting of asthma and multiple disparate signs and symptoms, the broad diagnostic category of serious vasculitic illness should be considered.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
10/24. Peripheral nerve involvement in churg-strauss syndrome.Peripheral neuropathy associated with bronchial asthma, multisystem organ dysfunction and idiopathic hypereosinophilia may be found in churg-strauss syndrome, hypereosinophilic syndrome and polyarteritis nodosa. Some authors have diagnosed their patients according to the presence in tissue biopsies of the three histological criteria of Churg and Strauss (necrotizing vasculitis, tissue eosinophilic infiltration, extravascular granulomas). We have observed three patients with a common history of a prodromal phase of allergic diseases (bronchial asthma and rhinitis) followed by a vasculitic phase with mononeuritis multiplex, purpura and arthritis, associated with hypereosinophilia of more than 1500 cells/mm3. All responded well to steroid treatment. sural nerve biopsy revealed true vasculitis in two of these cases and a mild perivascular inflammatory infiltration in the other. On the basis of their characteristic clinical pattern, we think that our cases best fit the diagnosis of churg-strauss syndrome even though the typical histological features were not found in the sural nerves examined.- - - - - - - - - - ranking = 2keywords = nerve (Clic here for more details about this article) |
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