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Unusual Response to Immunotherapy
Seventy per cent of patients respond to one or another of the three standard therapies, and probably 90% respond overall. A failure to respond to treatment, or rapid deterioration of symptoms despite an adequate trial of treatment with at least two treatment modalities, should prompt a re-evaluation of the diagnosis.
Motor-predominant forms of CIDP present with clinical motor involvement only, but sensory involvement on electrical studies. Motor CIDP often behaves more like multifocal motor neuropathy with conduction block; these cases may worsen with corticosteroid treatment, and more rarely plasma exchange, but usually respond to treatment with intravenous immunoglobulin. Other conditions, such as MADSAM (Lewis–Sumner syndrome), chronic inflammatory sensory polyneuropathy, DADS and relapsing sensory ataxic neuropathy, are all considered atypical variants of classical CIDP. Although the clinician may be fortunate in treating such patients effectively, atypical variants have a somewhat unpredictable and typically poorer treatment response compared to typical CIDP. A careful clinical history and examination and use of appropriately comprehensive electrical studies can differentiate these clinically important variants. The DADS acronym is confusingly also applied to anti-MAG neuropathies (see below), and this may be because some laboratory tests in current use are unable to detect all anti-MAG antibodies.
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