Diagnostic and Therapeutic Approach to Autoimmune Neurologic Disorders

A. Sebastian López-Chiriboga, MD; Eoin P. Flanagan, MD


Semin Neurol. 2018;38(3):392-402. 

In This Article

Oncologic Evaluation

Neurological autoimmunity can be triggered by an underlying malignancy, and often such patients develop the paraneoplastic neurologic syndrome prior to detection of the cancer. In others, the paraneoplastic syndrome develops after the cancer has been treated, and these patients should be screened for cancer recurrence. In patients who develop the paraneoplastic syndrome prior to detection of the cancer, the autoantibodies can help guide the targeted oncologic search (e.g., pelvic ultrasound [US] to assess for ovarian teratoma in anti-NMDA-R encephalitis). This is particularly important in patients with antibodies targeting intracellular antigens "onconeuronal antibodies." The investigations to screen for cancer may be guided by the neural autoantibody found. However, in situations in which the type of cancer is not predicted by the neural antibody, a general approach of CT of the chest, abdomen, and pelvis may be considered, although FDG PET-CT may be considered in addition or as first line, as it increases the sensitivity for the detection of cancers in patients with paraneoplastic neurologic disorders.[52] Sex-specific tests such as pelvic US, mammogram, and testicular ultrasound should be considered if the initial screen is unrevealing. If the initial evaluation is negative and the neural antibody is strongly predictive of cancer (>80%; e.g., Purkinje cell cytoplasmic autoantibody type 1 [anti-Yo]), surveillance imaging and close neurologic evaluation for at least 3 to 5 years should be considered.[53] Human leukocyte antigen (HLA) analysis may help stratify the oncologic screening in some patients.[54]

Autoantibodies and their commonly associated neoplasms are summarized in the Table 2.