Abstract
Autoimmune diseases affecting the nervous systems are a common cause of admission
to the intensive care unit (ICU). Although there exist several well-described clinical
syndromes, patients more commonly present with progressive neurologic dysfunction
and laboratory and radiographic evidence of central nervous system (CNS) inflammation.
In the critical care setting, the urgency to intervene to prevent permanent damage
to the nervous system and secondary injury from the systemic manifestations of these
syndromes often conflicts with diagnostic uncertainty. Furthermore, treatment is limited
by current therapeutic agents that remain non-specific for individual diseases, especially
for those whose pathophysiology remains unclear. Primary autoimmune, paraneoplastic,
parainfectious, and iatrogenic neurologic disorders all share the common underlying
pathophysiology of an adaptive immune response directed against an antigen within
the nervous system. Several different mechanisms of immune dysfunction are responsible
for pathogenesis within each of these categories of disease, and it is at this level
of pathophysiology that the most effective and appropriate therapeutic decisions are
made. In this review, we outline the basic diagnostic and therapeutic principles in
the management of autoimmune diseases of the nervous system in the ICU. We approach
these disorders not as lists of distinct clinical syndromes or molecular targets of
autoimmunity but rather as clusters of syndromes based on these common underlying
mechanisms of immune dysfunction. This approach emphasizes early intervention over
precise diagnosis. As our understanding of the immune system continues to grow, this
framework will allow for a more sophisticated approach to the management of patients
with these complex, often devastating but frequently reversible, neurologic illnesses.
Keywords
autoimmune neurology - paraneoplastic disorder - limbic encephalitis - neurologic
disorder
