Autoimmune Neurology

Abstract

Autoimmune neurology is a rapidly developing specialty driven by an increasing recognition of autoimmunity as the cause for a broad set of neurologic disorders and ongoing discovery of new neural autoantibodies associated with recognizable clinical syndromes. The diversity of clinical presentations, unique pathophysiology, and the complexity of available treatments requires a dedicated multidisciplinary team to diagnose and manage patients. In this article, we focus on antibody-associated autoimmune encephalitis (AE) to illustrate broader themes applicable to the specialty. We discuss common diagnostic challenges including the utilization of clinical assessment tools along with the determination of the prognostic significance of certain autoantibodies, with a focus on implications for long-term management. A growing body of literature demonstrates the long-term cognitive, behavioral, and physical sequelae of AE. Dedicated resources are needed to effectively manage these patients. These resources may be best provided by experienced neurology clinics in partnership with other neurologic subspecialists, as well as psychiatrists, neuropsychologists, and physical medicine and rehabilitation providers.

Long-term outcomes and health-related quality of life in patients with autoimmune encephalitis: An observational study

“…[3,5,7,28,32] The significance of various resources such as experienced neurology clinics, psychiatrists, neuropsychologists, physical medicine, and rehabilitation in partnership with neurologic specialists was also advocated. [33] Considering the tight association between sequelae and social activity limitations, timely diagnosis and treatment in the acute phase and appropriate follow-up in the post-acute phase prevent sequelae and reduce difficulty in social activities, which potentially lead to better HRQOL of patients many years after AE onset. Therefore, the establishment of clinical guidelines for AE, which includes recommendations for the treatment and management in the acute phase with high evidence level, is urgently needed to improve the long-term HRQOL of patients.…”

What is autoimmune encephalitis‐associated epilepsy? Proposal of a practical definition

“…Further options of care for AEAE are social–medical counseling and neurocognitive training or ergotherapy, as appropriate. Psychological coping strategies for a chronic condition in everyday life are important and may be shared and trained by rehabilitation services 38,39 …”

Life after autoantibody-mediated encephalitis: optimizing follow-up and management in recovering patients

“…AME patients commonly require prolonged hospitalization, multidisciplinary care, and staged escalation of immunotherapies [4 ▪▪ ], with most surviving to discharge. Longer-term management is decidedly more variable.…”

“…Outside of the acute period, interventions are lacking to improve longterm outcomes. Targeted rehabilitative measures, incorporating goal-directed occupational therapy may be useful in selected patients [4 ▪▪ ]. Ideally, these would be delivered together with lifestyle interventions known to improve cognition (e.g., physical exercise) and other potentially modifiable ‘dementia’ risk factors [78].…”

“…Beyond cognitive deficits, anxiety, depression [57,79], and psychosis [6,80] are common contributors to functional impairment, compromising return to work or school and quality of life in recovering patients (and caregivers) [59,81]. Active screening for these AME sequelae is important, recognizing that many symptoms may improve with use of established pharmacotherapies (e.g., selective serotonin reuptake inhibitors for anxiety and depression [82], antipsychotics for persistent psychoses [83]) and nonpharmacological interventions (e.g., meditation, biofeedback [4 ▪▪ ], and counseling). A comprehensive assessment should also screen for medications (e.g., opiates, benzodiazepines, anticholinergics) or other substances (e.g., alcohol, marijuana use) that may directly or indirectly impair cognition.…”