Antibodies against disialosyl and terminal NeuNAc( α 2-3)Gal ganglioside epitopes in acute relapsing sensory ataxic neuropathy

JO N 2 80 3 Neurological examination revealed ophthalmoplegia and gait ataxia with a positive Romberg’s sign. Deep tendon reflexes were absent as was sensation to vibration up to the knees and elbows. Sensory loss mainly affecting propioceptive and kinesthetic sensations was predominant in lower limbs. Bulbar involvement was manifested by the presence of dysphagia, nasal voice and phonation difficulties. Muscle strength was normal. Electrophysiologic findings were consistent with a demyelinating sensorymotor polyneuropathy. CSF was acellular with elevated protein levels (1.200 g/L, normal < 0.450 g/ L). Serum immunofixation revealed an IgM-κ type M component. Cold agglutinins were present. Screening tests for malignancy, serum level of vitamin B12 and E, folate, VDRL and HIV, anti-Ro and anti-La antibodies, and anti-MAG antibodies were normal or negative. The patient improved after a course of intravenous immunoglobulins (0.4 g/kg body weight) and symptoms resolved completely. Nine years later he developed a similar episode, following another upper respiratory tract infection, and spontaneously recovered two weeks later. Serum samples were obtained during the acute phase of both episodes and six months later. ELISA showed high titres of serum antiGM3 (1:25500), GD1a (1:1100), Ricard Rojas-Garcia Eduard Gallardo Monica Povedano Noemi de Luna Jordi Bruna Candido Juarez Jordi Diaz-Manera Juan Antonio Martinez-Matos Isabel Illa