93. CANOMAD: Clinical and neurophysiological findings in two cases

Abstract

The acronym CANOMAD (Chronic Ataxic Neuropathy, Ophthalmoplegia, IgM paraproteinemia, cold Agglutinin and Disialosyl antibodies) refers a rare immune-mediated peripheral neuropathy, whose clinical spectrum has still to be fully delineated. Herein we report the clinical and neurophysiological features of two CANOMAD patients. A 52-year-old man suffering from distal sensory symptoms and ataxia had received a first diagnosis of chronic inflammatory prominent-sensory polyneuropathy 11 years ago. High-dose i.v. steroid treatment resulted in paradoxical severe worsening with ophthalmoplegia and ambulation loss. Based on immunological and clinical–neurophysiological revaluation a diagnosis of CANOMAD was made. Since then IVIg periodic infusions had allowed good clinical remission up to-day, although minor degree of motor impairment and ataxia relapsed during last years. This 62-year-old man had a 5-year history of mild lower limb ataxia. More recently, a fluctuating bilateral ptosis was seen. Serum was positive for anti-disialosyl IgM antibodies and cold agglutinins. NCS/EMG showed a mild chronic sensory-motor neuropathy. Cortical somatosensory evoked potentials by tibial nerve stimulation at the ankle were deranged, whereas responses at the popliteal fossa and sural NCS were largely preserved. This suggests a proximal alteration of peripheral sensory conduction. These data further confirm the heterogeneous presentation of CANOMAD.