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

Therapeutic Approach

At present, the treatment of antibody-associated autoimmune neurologic disorders is based on recommendations from expert opinion and information from available case reports and case series; several prospective randomized trials are currently evaluating the immunotherapy response for different agents in patients with NMOSD and autoimmune encephalitis/epilepsy. The goal of immunotherapy is to provide maximum response, reducing nervous system inflammation and decreasing symptoms, maintain improvements by preventing relapses, and minimize side effects.[55] Following antibody titers in the management of patients has several limitations and rarely helps guide therapy, which should be based on clinical findings as titers are not reliable markers of disease severity,[56] but they can sometimes predict relapses and support the use of prolonged immunotherapy for prevention.

Lack of response to immunotherapy should prompt evaluation of alternative etiologies (infections, metabolic, genetic, neurodegenerative disorders). Furthermore, response to corticosteroid treatment does not imply autoimmunity, and other disorders can also respond dramatically to steroids (e.g., lymphoma).

The modality of immunotherapy is based on the serological findings and the patient's clinical condition. Patients with onconeural antibodies can have limited response to immunotherapy but can stabilize with treatment of the underlying neoplasm, and immunotherapy can be tried with often limited success.

In addition to immunotherapy, other important aspects of the treatment include supportive care, particularly in the inpatient setting, symptomatic therapy (seizures,[57] spasms, neuropsychiatric symptoms)[58] and rehabilitation.