Autoantibody-mediated neurological diseases

In 1672 Thomas Willis first described a patient with fatiguable muscle weakness, which is likely to have been myasthenia gravis (MG), a prototypic antibody-mediated neuroimmunological condition. However, the linking between MG and anti-acetylcholine receptor antibody only began 3 centuries later in the 1970s, a few years after discovering the antibody structure.

Several clinical syndromes and autoantibodies of neuroimmunological disorders have been identified since, especially in the past two decades. The presentation is typically subacute and can span the whole nervous system and occasionally involve other systems. Some of these conditions tend to be associated with malignancy, demanding a thorough and persistent tumour hunt.

The incidence has increased with the ability to test for autoantibodies and the increasing availability of imaging and other supportive investigations. Early recognition and immunotherapy are crucial to better patient outcomes. More than ever, it is now essential for any practising clinician to become familiar with common and important neuroimmunological conditions.

Here we summarize central and peripheral neuroimmunological conditions with well-characterized antibodies in terms of ‘typical’ clinical presentations, investigations and standard management. These conditions include autoimmune encephalitis, paraneoplastic disorders, neuromyelitis optica, stiff person syndrome, paranodopathies and myasthenic syndromes. We also briefly describe novel immunotherapies, which are gathering increasing evidence in these conditions.