1/5. 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 = 1keywords = syndrome (Clic here for more details about this article) |
2/5. 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 = 8keywords = syndrome (Clic here for more details about this article) |
3/5. A limited form of churg-strauss syndrome presenting without asthma and eosinophilia.We report a young woman presenting with digital gangrene, paranasal sinusitis, mononeuritis multiplex, and rapidly progressive glomerulonephritis without asthma and eosinophilia - an extremely rare variant of this disease.- - - - - - - - - - ranking = 4keywords = syndrome (Clic here for more details about this article) |
4/5. Presence of perineuritis in a case of papular purpuric gloves and socks syndrome associated with mononeuritis multiplex attributable to B19 parvovirus.A previously healthy 16-year-old boy developed papular purpuric gloves and socks syndrome concomitant with mononeuritis multiplex attributable to acute infection with parvovirus B19. A cutaneous biopsy showed lymphocytic lichenoid vasculitis and perineuritis. We discuss the relevance of these pathologic findings, and we suggest a correlation with the patient's neurologic condition. This case illustrates the difficulties in differentiating dysesthesia accompanying cutaneous manifestations of exanthem from neurologic disease on the basis of the clinical appearance alone.- - - - - - - - - - ranking = 5keywords = syndrome (Clic here for more details about this article) |
5/5. Mononeuropathy multiplex (AAEE case report #11).Mononeuropathy multiplex is a syndrome of diverse causes, the most common of which is nerve ischemia due to microangiopathy associated with diabetes or the collagen-vascular diseases. The acquired inflammatory demyelinating neuropathies invariably are multifocal, although the clinical manifestations of these and other multifocal neuropathies may appear symmetrical. The identification of multifocal neuropathies is important because of the frequent therapeutic implications. A case of mononeuropathy multiplex associated with polyarteritis nodosa is described.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |