93. CANOMAD: Clinical and neurophysiological findings in two cases. Issue 1 (January 2015)

• Record Type: Journal Article
• Authors: Del Sordo, E.; Casali, S.; Ginanneschi, F.; Insana, L.; Capoccitti, G.; Cardinali, C.; Battista, D.; Borgheresi, A.; Cincotta, M.; Borsini, W.; Giannini, F.
• Is Part Of: Clinical neurophysiology. Volume 126:Issue 1(2015:Jan.)
• Page Start: e21
• Page End: e22
• Publication Date: 2015-01
• Subjects: Neurophysiology -- Periodicals; Electroencephalography -- Periodicals; Electromyography -- Periodicals; Neurology -- Periodicals; 612.8
• DOI: 10.1016/j.clinph.2014.10.112
• Languages: English
• ISSNs: 1388-2457

Abstract

<abstract xml:lang="en" abstract-type="author" id="ab005"> <title> <x xml:space="preserve">Abstract</x> </title> <sec> <p id="sp005">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.</p> <p id="sp010">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.</p> <p id="sp015">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.</p> </sec> </abstract>