1/13. "Pseudo-conduction block" in vasculitic neuropathy.The predominant electrophysiologic feature of vasculitic mononeuropathy multiplex is axonal loss. Electrophysiologic findings interpreted as conduction block have, however, also been reported to occur in neuropathy secondary to necrotizing vasculitis. We report 3 patients with mononeuropathy multiplex and biopsy proven vasculitis in whom eight nerves met criteria for conduction block. In each circumstance, serial study demonstrated conversion of the electrophysiologic findings to those most consistent with severe axonal loss. "Conduction block" in vasculitic mononeuropathy multiplex is secondary to focal axonal conduction failure presumably related to infarctive axonal injury. The term conduction block should be used with caution in this disorder and only if serial studies demonstrate findings consistent with this electrophysiologic diagnosis.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
2/13. Localized neurological necrotizing vasculitides. Three cases with isolated mononeuritis multiplex.Localized vasculitic neuropathies are increasingly reported. We describe 3 cases of peripheral neuropathy with necrotizing vasculitis confined to nerves and muscles without systemic involvement. These neuropathies were severe and relapsing, in contrast to a usually benign prognosis. Our cases appear to be isolated vasculitic neuropathies, with vasculitis strictly limited to the peripheral neuromuscular system without nonspecific clinical and/or biological systemic involvement.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
3/13. Sensory perineuritis presented as a mononeuritis multiplex associated with livedo vasculitis.We report a 52-year-old patient affected by a mononeuritis multiplex and livedo vasculitis. sural nerve biopsy showed mild thickening of the perineurium, vascular alterations with inflammatory cell infiltration in the perineurium, and remarkable loss of large and small myelinated fibers. A therapeutic trial of acetylsalicylic acid, danazol, and dipyridamole controlled the skin lesion without improvement of nerve conduction studies.- - - - - - - - - - ranking = 2.5keywords = vasculitis (Clic here for more details about this article) |
4/13. Confluent peripheral multiple mononeuropathy associated to acute hepatitis B: a case report.A thirty three year-old, male patient was admitted at the Hospital of the Sao Paulo University School of medicine, at the city of Sao Paulo, brazil, with complaint of pains, tingling and decreased sensibility in the right hand for the last four months. This had progressed to the left hand, left foot and right foot, in addition to a difficulty of flexing and stretching in the left foot. Tests were positive for HBeAg, IgM anti-HBc and HBsAg, thus characterizing the condition of acute hepatitis B. The ALT serum level was 15 times above the upper normal limit. blood glucose, cerebral spinal fluid, antinuclear antibodies (ANA) and anti-hiv and anti-HCV serum tests were either normal or negative. Electroneuromyography disclosed severe peripheral neuropathy with an axon prevalence and signs of denervation; nerve biopsy disclosed intense vasculitis. The diagnosis of multiple confluent mononeuropathy associated to acute hepatitis B was done. This association is not often reported in international literature and its probable cause is the direct action of the hepatitis b virus on the nerves or a vasculitis of the vasa nervorum brought about by deposits of immune complexes.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
5/13. Orbital inflammatory pseudotumor due to hypersensitivity vasculitis and mononeuritis multiplex in a patient with atypical, cANCA-positive Wegener's granulomatosis.OBJECTIVE: We report on a 60-year-old woman with a retro-orbital pseudotumor and polyneuropathy. The retro-orbital inflammation was histologically diagnosed as hypersensitivity vasculitis (HV). As cytoplasmatic antineutrophilic cytoplasmatic antibody (cANCA) and anti-proteinase-3 antibody were detected, the differential diagnosis also included atypical Wegener's granulomatosis. hypersensitivity vasculitis is defined as small-vessel vasculitis mediated by the deposition of immune complexes (arthus reaction) after exposure to various agents such as drugs, toxins, and infections. Since an inflammatory retro-orbital pseudotumor due to HV has not previously been reported, the following case is presented. methods AND MAIN OUTCOME MEASURES: magnetic resonance imaging (MRI) revealed retro-orbital infiltrate without granuloma. histology from an orbital biopsy confirmed HV. electromyography was used for the diagnosis of polyneuropathy. serum investigation indicated erythrocyte sedimentation rate (ESR) >100 mm/h, c-reactive protein (CRP) 223 mg/l, antinuclear antibodies 1:80, and cANCA 100 U/ml. RESULTS: The bilateral orbital pseudotumor, polyneuropathy, and serum levels of inflammation reactants (ESR and CRP) improved from therapy with corticosteroids (1 g of methylprednisolone initially) and azathioprine (150 mg/day). CONCLUSIONS: Because of cANCA and anti-proteinase-3 antibody positivity, this case can be viewed more as an atypical Wegener's granulomatosis than a systemic HV. The causal variety of inflammatory orbital pseudotumor, including HV and different therapeutic consequences, requires histological differentiation from usual orbital pseudotumors.- - - - - - - - - - ranking = 3.5keywords = vasculitis (Clic here for more details about this article) |
6/13. Microvasculitic paraproteinaemic polyneuropathy and B-cell lymphoma.Microvasculitis may play a greater part in the pathogenesis of paraproteinaemic neuropathies than is generally recognised, producing tissue destruction by convergent immune and physical mechanisms. We present a patient with a clinical syndrome of mononeuritis multiplex and a circulating IgM lambda paraprotein, in whom bone marrow aspiration revealed a lymphoplasmacytoid lymphoma. Microvasculitic changes were present in the first nerve biopsy, and the second showed extensive destruction of neural architecture and deposition of IgM-related material. A 2-stage pathogenic cascade is postulated and explored with a review of the relevant literature.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
7/13. Acute cholecystitis and duodenitis associated with churg-strauss syndrome.We describe a patient with acute cholecystitis and duodenitis associated with churg-strauss syndrome. A 36-year-old male, who had been healthy, had abdominal pain following high fever. He had marked hypereosinophilia of 17,000/mm3. Radiographs of the chest disclosed a transient infiltrated lesion in the left lower lung. Ultrasonographic and gastroendoscopic examinations revealed acute cholecystitis and duodenitis, respectively. Endoscopic retrograde cholangiopancreatography demonstrated a filling defect suspecting aberrant ascariasis in the common bile duct. The patient suddenly developed distally dominant mononeuritis multiplex, especially in the upper limbs. Muscle biopsy revealed vasculitis of intramuscular arteries with infiltration of eosinophils. These findings fulfilled the diagnostic criteria of churg-strauss syndrome. Corticosteroid dramatically resolved the abdominal symptoms. cholecystectomy and removal of the foreign body were performed. Histological examinations revealed that necrosis of the gallbladder was caused by occlusion due to thrombosed arteries and that the foreign body in the common bile duct was an aggregate of necrotic epithelium of the bile duct wall surrounded by inflammatory cells. Although abdominal complaints rarely appeared as an initial symptom in the patients with churg-strauss syndrome, this syndrome should be taken into consideration for an accurate diagnosis when the patients with abdominal pain of unknown origin had eosinophilia, asthma, or allergic rhinitis.- - - - - - - - - - ranking = 0.5keywords = vasculitis (Clic here for more details about this article) |
8/13. Paediatric mononeuritis multiplex: a report of three cases and review of the literature.Mononeuritis multiplex results from disease processes causing multifocal injury to the peripheral nerves, with or without involvement of one or more cranial nerves. Most reported cases of paediatric mononeuritis multiplex have been related to autoimmune disorders. We present clinical and neurophysiologic findings in three cases of multiple mononeuropathy occurring in adolescence, with onset at ages 16, 17 and 13 years. Two of these cases were related to systemic vasculitis, one possibly representing a paediatric case of non-systemic vasculitis. sural nerve biopsy confirmed the diagnosis of vasculitis in one patient with systemic lupus erythematosus, but was non-diagnostic in another case. Mononeuritis multiplex is an extremely uncommon form of acquired peripheral neuropathy in childhood. We discuss the differential diagnosis and review previous descriptions of this rare condition.- - - - - - - - - - ranking = 1.5keywords = vasculitis (Clic here for more details about this article) |
9/13. Mononeuropathy multiplex in association with livedoid vasculitis.Livedoid vasculitis is a chronic dermatological disorder associated with petechiae and recurrent, unusually shaped ulcers that heal to form hyperpigmentated areas and atrophie blanche. This condition is more correctly termed a vasculopathy, rather than a vascultis, and is often associated with an underlying hypercoagulable disorder. We report a patient with livedoid vasculitis and mononeuropathy multiplex. We propose that peripheral nervous system involvement arises from multifocal areas of ischemia due to fibrin and thrombin deposition within both the wall and lumen of vasa nervorum.- - - - - - - - - - ranking = 3keywords = vasculitis (Clic here for more details about this article) |
10/13. Severe proximal myopathy and mononeuritis multiplex in rheumatoid arthritis: manifestations of rheumatoid vasculitis.Vascular injury is considered to be a key finding in the pathogenesis of rheumatoid arthritis (RA). Manifestations are varied depending on the vessel size and the organ system involved. Vasculitis leading to symptomatic inflammatory myositis is a rare complication of RA. We describe a 62-year-old man with seropositive erosive RA of 1-year duration, who presented with severe proximal weakness and mononeuritis multiplex. His joint disease was clinically mild at the time of presentation. creatine kinase was normal and the electromyogram did not suggest myopathy. However, muscle biopsy revealed extensive small vessel vasculitis and severe inflammatory myositis. This report emphasizes the importance of fully evaluating patients with RA who present with proximal myopathy. The myopathy in our patient was not related to active joint disease, disuse atrophy, or complication of therapy. rheumatoid vasculitis leading to myositis is a rare and not well-recognized complication of RA for which aggressive immunosuppressive therapy is warranted.- - - - - - - - - - ranking = 3keywords = vasculitis (Clic here for more details about this article) |
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