Imaging of Central Nervous System Autoimmune, Paraneoplastic, and Neuro-rheumatologic Disorders

Lama Abdel Wahed, MD; Tracey A. Cho, MD, FAAN Neuroimaging p. 255-291 February 2023, Vol.29, No.1 doi: 10.1212/CON.0000000000001244
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OBJECTIVE This article provides an overview of the imaging modalities used in the evaluation of central nervous system (CNS) autoimmune, paraneoplastic, and neuro-rheumatologic disorders. An approach is outlined for interpreting imaging findings in this context, synthesizing a differential diagnosis based on certain imaging patterns, and choosing further imaging for specific diseases.

LATEST DEVELOPMENTS The rapid discovery of new neuronal and glial autoantibodies has revolutionized the autoimmune neurology field and has elucidated imaging patterns characteristic of certain antibody-associated diseases. Many CNS inflammatory diseases, however, lack a definitive biomarker. Clinicians should recognize neuroimaging patterns suggestive of inflammatory disorders, as well as the limitations of imaging. CT, MRI, and positron emission tomography (PET) modalities all play a role in diagnosing autoimmune, paraneoplastic, and neuro-rheumatologic disorders. Additional imaging modalities such as conventional angiography and ultrasonography can be helpful for further evaluation in select situations.

ESSENTIAL POINTS Knowledge of imaging modalities, both structural and functional, is critical in identifying CNS inflammatory diseases quickly and can help avoid invasive testing such as brain biopsy in certain clinical scenarios. Recognizing imaging patterns suggestive of CNS inflammatory diseases can also facilitate the early initiation of appropriate treatments to diminish morbidity and future disability.

Address correspondence to Dr Tracey A. Cho, Department of Neurology, University of Iowa Hospitals and Clinics, 200 Hawkins Dr, Iowa City, IA 52242, [email protected].

RELATIONSHIP DISCLOSURE: Dr Abdel Wahed has received personal compensation in the range of $0 to $499 for serving as a resident honorary speaker with the Iowa Neurological Association and as an author for MedLink, LLC. Dr Cho has received personal compensation in the range of $500 to $4999 for serving as a consultant for Horizon Therapeutics plc and on a data safety monitoring board for the National Institutes of Health and has received publishing royalties from a publication relating to health care. The institution of an immediate family member of Dr Cho has received research support from the National Institutes of Health.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Drs Abdel Wahed and Cho discuss the unlabeled use of corticosteroids for the treatment of autoimmune neurologic diseases that have no US Food and Drug Administration–approved medications.

© 2023 American Academy of Neurology.