Krenn Martin, Keir Geoff, Wieshmann Udo Carl
Medical University of Vienna, Vienna, Austria.
BMJ Case Rep. 2014 Apr 10;2014:bcr2013202545. doi: 10.1136/bcr-2013-202545.
A 48-year-old man presented with numbness in fingers and diplopia 1 week after a flu-like illness. He made a full recovery but 8 years later developed progressive and disabling sensory ataxia. He had superimposed acute flare-ups with numbness, double vision and ptosis, all following infections. A blood test showed antidisialosyl antibodies including GD1b, GD3, GT1b and GQ1b in keeping with the diagnosis of chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins and antidisialosyl antibodies (CANOMAD). Initial treatment with monthly courses of intravenous immunoglobulin (IVIg) 0.4 g/kg/day for 5 days every 4 weeks helped temporarily but there were marked disabling fluctuations of symptoms. With IVIg 0.6 g/kg/day weekly his symptoms are stable. He remains mobile and has no eye symptoms without need for any other medication. This case demonstrates that weekly IVIg infusions instead of one 5-day course monthly may be able to avoid fluctuations of symptoms in CANOMAD.
一名48岁男性在患流感样疾病1周后出现手指麻木和复视。他完全康复,但8年后出现进行性且致残的感觉性共济失调。他在每次感染后都会叠加急性发作,伴有麻木、复视和上睑下垂。血液检查显示存在抗唾液酸抗体,包括GD1b、GD3、GT1b和GQ1b,符合慢性共济失调性神经病、眼肌麻痹、IgM副蛋白、冷凝集素和抗唾液酸抗体(CANOMAD)的诊断。最初每4周进行一次为期5天、每天0.4 g/kg的静脉注射免疫球蛋白(IVIg)治疗,虽有暂时帮助,但症状有明显的致残性波动。采用每周一次、每天0.6 g/kg的IVIg治疗后,他的症状稳定。他仍可活动,且无眼部症状,无需任何其他药物治疗。该病例表明,对于CANOMAD,每周进行IVIg输注而非每月一次为期5天的疗程,或许能够避免症状波动。