[Anti-GD1b IgG positive case of overlapping Ficher's and Guillain-Barré syndromes]. / Une forme frontière de syndrome de Guillain-Barré sensitif proche du syndrome de Miller Fisher avec IgG anti-GD1b.

We describe a patient who developed overlapping sensory ataxic form of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) following Campylobacter jejuni infection. Two cerebrospinal fluid examinations shown albuminocytologic dissociation associated with Campylobacter jejuni infection after tongue pierced. He had high titers of monospecific anti-GD1b IgG antibody. Because of the rarety of this disorder the diagnostic was difficult. There is a close association of IgG anti-ganglioside GD1b antibodies in sensory ataxic GBS. The findings of the present study show that antibody to GD1b ganglioside is one of the immunological factors in the pathogenesis of sensory ataxic form of GBS, a rare specific immuno-clinical variant form of GBS with prominent sensory ataxia.

Long-term follow-up in patients treated with methotrexate, vinblastine, doxorubicin, and cisplatin (M-VAC) for transitional cell carcinoma of urinary bladder: cause for concern.

Twenty-one patients with high-stage transitional cell carcinoma (TCC) of the bladder were treated with a combination of methotrexate, vinblastine, doxorubicin (Adriamycin), and cisplatin (M-VAC). There was a minimum follow-up of thirty-six months (range 36-45) in all patients. Patients were divided into three groups: 10 patients with recurrent TCC post radical cystectomy, 6 patients staged as T3/T4 or N1 treated in a neo-adjuvant setting, and 5 patients who received adjuvant M-VAC four weeks post radical cystectomy. The overall initial response rate in patients with measurable disease was 68 percent (11 of 14), complete response rate 31 percent, and partial response rate 37 percent. There was no response in 31 percent. The durability of response in this series was very disappointing with all complete responders having recurrence of disease, with duration of responses ranging from eight to twenty-one months. Of the 5 patients who received M-VAC as adjunctive therapy, only 1 remains disease-free; the other 4 patients experienced disease progression four to seventeen months postoperative, and all have died. There was one drug-associated death due to nadir sepsis, and myelosuppression occurred in 12 patients. While M-VAC seems to be the best protocol currently available, the real durability of response is most disappointing.

Management of an enlarging aortic aneurysm in the presence of radiation induced retroperitoneal fibrosis.

Despite a thoracoabdominal retroperitoneal approach to an enlarging symptomatic infrarenal aortic aneurysm, proximal aortic dissection was hazardous due to radiation induced retroperitoneal fibrosis. Iliac artery ligation and thoracic aorta to iliac artery bypass has resulted in successful management during 14 months of follow-up.

Management of plasmacytomas of the spine.

Epidural plasmacytoma often reflects systemic myelomatous involvement and usually has a poor prognosis. The isolated spinal plasmacytoma, however, is a lesion with the potential for long term remission or even cure in some cases. We report six patients with isolated plasmacytoma of the low thoracic or lumbar region, all of whom presented with pain and minimal neurological deficits. Our approach to such cases included a complete medical work-up and radiographic definition of the lesion with a bone scan, a skeletal survey, myelography, computed tomography, and, in some instances, spinal angiography. All of these patients underwent laminectomy and spinal fusion with autologous bone and Harrington rods. There was no morbidity associated with the procedure, which was designed to prevent possible collapse during subsequent radiotherapy or chemotherapy, with its attendant potential for neurological catastrophe. Several patients have developed systemic involvement necessitating chemotherapy, but in follow-up extending now to 4 years all patients remain ambulatory and pain-free. We are encouraged by the potential for symptomatic relief and neurological improvement of patients with isolated spinal plasmacytomas who are treated with an aggressive medical and surgical approach.