Brain, Vol. 124, No. 10, 1968-1977,
October 2001
© 2001 Oxford University Press
The clinical and laboratory features of chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies
1 Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, Glasgow, 2 Division of Clinical Neurology, University Hospital, Queen's Medical Centre, Nottingham, 3 Department of Clinical Neurology, Radcliffe Infirmary, Oxford, 4 Department of Neurology, St James's Hospital, Leeds, 5 Wessex Neurological Centre, Southampton General Hospital, Southampton, 6 Department of Neurology, Queen Elizabeth II Hospital, Birmingham, 7 Manchester Royal Infirmary, Manchester, 8 Department of Clinical Neurophysiology, St Bartholemew's Hospital and 9 University Department of Clinical Neurology, Institute of Neurology, University College, London, UK, 10 Neurologische Klinik, Universitätskliniken, Basel, Switzerland, 11 Department of Clinical Neurological Sciences, University of Western Ontario, London, Ontario, Canada and 12 Service de Neurologie, CHU Dupuytren, Limoges, France
Correspondence to:
Dr H. J. Willison, University Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, Glasgow G51 4TF, UK E-mail: h.j.willison{at}udcf.gla.ac.uk
The clinical and laboratory phenotype of a paraproteinaemic neuropathy syndrome termed chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies is described in a series of 18 cases. Previous single case reports have outlined some features of this syndrome. All 18 cases were defined by the presence of serum IgM antibodies which react principally with NeuAc (
2-8)NeuAc(2-3)Gal-configured disialosyl epitopes common to many gangliosides including GDlb, GD3, GTlb and GQlb. In 17 out of 18 cases, the serum contained benign IgM paraproteins, and in four of these cases at least two IgM paraproteins were present. The IgM antibodies were also cold agglutinins in 50% of cases. The clinical picture comprised a chronic neuropathy with marked sensory ataxia and areflexia, and with relatively preserved motor function in the limbs. In addition, 16 out of 18 cases had motor weakness affecting oculomotor and bulbar muscles as fixed or as relapsing-remitting features. When present in their entirety, these clinical features have been described previously under the acronym CANOMAD: chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies. This distribution of clinical features is reminiscent of Miller Fisher syndrome, in which acute-phase anti-disialylated ganglioside IgG antibodies are found. Clinical electrophysiology and nerve biopsy show both demyelinating and axonal features. A partial response to intravenous immunoglobulin and other treatments is reported in some cases.
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