11/33. sural nerve lesions: a report of 20 cases. Given the rarity of sural nerve mononeuropathy, this retrospective study reports 20 cases observed during the last 12 yr, with some cases having especially unusual mechanisms of the nerve lesion. Five of the 20 cases were considered S1 root disease until electrodiagnostic findings. In the 14 other cases, the nerve lesion was suspected because of concomitant trauma. This series is the largest sample of sural nerve lesions so far reported, and it underlines the importance of iatrogenic lesions, which account for 12 of the 20 cases. Some lesions are due to unusual conditions, such as small saphenous vein stripping and compression by the deep fascia. This series also demonstrates that even when lower limb trauma exists, some cases can be considered S1 root disease and that when the area of impaired sensation corresponds exactly to the cutaneous territory of a nerve trunk, one should first consider a truncular origin of the nerve lesion. ( info) |
| After 1 week of flu-like illness, a 64-year-old man developed rapidly progressive mononeuritis multiplex involving the right arm and both legs. Serologic studies identified coxiella burnetii as the cause of the febrile disease (q fever). Fourteen days doxycycline treatment (200 mg daily) induced rapid and complete recovery. After 6 months, flu-like symptoms, weakness and hypalgesia of the right leg reappeared. Antibody titers again identified q fever. doxycycline was re-established and induced prompt recovery. q fever has been associated with various neurologic complications such as meningoencephalitis, cerebellitis, optic neuritis or polyneuroradiculitis. This is the first report on q fever related mononeuritis multiplex. Prolonged antibiotic treatment may be required to prevent relapsing infection from the resistant bacterium. ( info) |
13/33. 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. ( info) |
14/33. Mononeuritis multiplex in diabetes mellitus: evidence for underlying immune pathogenesis. Four patients with type 2 diabetes mellitus developed mononeuritis multiplex subacutely. sural nerve biopsies showed multifocal axonal loss in all patients, with epineurial perivascular inflammation affecting small calibre vessels in three. Three patients improved with immunotherapy. These observations suggest that mononeuritis multiplex in diabetes may be caused by an immune mediated vasculopathy and that it is pathogenetically akin to the more common and better recognised diabetic amyotrophy. ( info) |
15/33. 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. ( info) |
| 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. ( info) |
17/33. Hereditary neuropathy with liability to pressure palsies in infancy. Hereditary neuropathy with liability to pressure palsies is an autosomal-dominant disorder, classically characterized by recurrent mononeuropathies, associated with a deletion at 17p11.2, encompassing the peripheral myelin protein 22 gene. The typical clinical episodes of pressure palsy are usually noted for the first time during the 2nd or 3rd decade of life. We found only few reports in prepubertal children. We report a case of a 7.5-year-old child with muscle weakness and severe hypotonia associated with developmental gross motor delay. We suspect that bilateral peroneal nerve palsies after birth were the first episode of pressure palsy. Nerve conduction studies demonstrated slightly prolonged distal latencies with normal conduction velocity. Typical features of hereditary neuropathy with liability to pressure palsies with recurrent mononeuropathies were found in the father. dna analysis revealed 1.5-Mb deletion at 17p11.2 in both father and child. To the best of our knowledge, this patient is one of the youngest ever found with this disease. ( info) |
18/33. Idiopathic thrombocytopenic purpura and mononeuropathy multiplex. Peripheral neuropathy is an uncommon complication of idiopathic thrombocytopenic purpura (ITP). We report a 61-year-old man with ITP who developed acute-onset mononeuropathy multiplex. An electrophysiologic study revealed active axonal degenerative alteration, and a sural nerve biopsy showed axonal degeneration. Intraneural hemorrhage was suggested to be the most likely cause. ( info) |
19/33. 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. ( info) |
20/33. 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. ( info) |