Abstract
A 76 year old lady presents with a 20 year history of a slowly progressive tremor-predominant, sensory ataxic neuropathy. Neurophysiology reveals slowed nerve conduction velocities with prolonged distal motor latencies. There is a positive IgM κ paraprotein with anti-MAG antibodies. A nerve biopsy demonstrates demyelinating features with widely-spaced myelin. The unsteadiness and imbalance this disease causes is its most disabling feature. Response to treatment can be highly variable. Currently, the best known treatment is rituximab, although there is conflicting evidence for its efficacy.
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Nobile-Orazio E, Meucci N, Baldini L, Di Troia A, Scarlato G. Long-term prognosis of neuropathy associated with anti-MAG IgM M-proteins and its relationship to immune therapies. Brain. 2000;123:710–7.
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Bhattacharjee, A., Phadke, R. (2017). A Lady with Tremor Not Due to Parkinson’s Disease. In: Manji, H., Turner, C., Evans, M. (eds) Neuromuscular Disease . Springer, London. https://doi.org/10.1007/978-1-4471-2389-7_4
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DOI: https://doi.org/10.1007/978-1-4471-2389-7_4
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