Paraproteinaemic neuropathies comprise a heterogeneous group of neuro-haematological conditions with some distinct neurological, haematological and systemic phenotypes. The spectrum of disease varies from mild to severe, indolent to rapidly progressive and from small fibre sensory involvement to dramatic sensorimotor deficits. The haematological association may be overlooked, resulting in delayed treatment, disability, impaired quality of life and increased mortality. However, the presence of an irrelevant benign paraprotein can sometimes lead to inappropriate treatment. In this review, we outline our practical approach to paraproteinaemic disorders, discuss the utility and limitations of diagnostic tests and the distinctive clinical phenotypes and touch on the complex multidisciplinary management approaches.
- clinical neurology
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Contributors ASC and MPTL contributed to the design, writing and review of the manuscript.
Funding MPTL is supported by the UCLH NHS Foundation Trust Biomedical Research Centre.
Competing interests ASC and MPTL received no funding or sponsorship for this commissioned paper. MPTL Consultancy: UCB Pharma, CSL Behring and Polyneuron. PI on trials with Polyneuron and UCB Pharma for which his institution receives investigator fees. DSMB: Octapharma, IoC trial, AstraZeneca Pharmaceuticals.
Provenance and peer review Provenance and peer review. Commissioned. Externally peer reviewed by Gareth Llewelyn, Cardiff, UK.