The autonomic nervous system maintains homeostasis through the regulation of multiple involuntary body functions [1]. Thus, its dysfunction may manifest with a wide constellation of signs and symptoms ranging from vasomotor abnormalities and sweating alterations to gastrointestinal dysmotility and lose of bladder control (Table 1). Although the autonomic nervous system may be the sole target in a disease (e.g. pure autonomic failure or autoimmune autonomic ganglionopathy), dysautonomia is more frequently part of disorders with a broader involvement of the nervous system (e.g. multiple system atrophy) [2]. The causes damaging the autonomic nervous system include metabolic (e.g. diabetes), toxic (e.g. alcohol intake), genetic (e.g. hereditary sensory and autonomic neuropathy), traumatic (e.g. upper spinal cord lesions), infectious (e.g. Chagas disease), neurodegenerative (e.g. pure autonomic failure, Lewy-body disease) or autoimmune/inflammatory (e.g. Sjögren disease) etiologies [2], [3]. For the latter, the description and characterization of the nicotinic ganglionic acetylcholine receptor (α3-AchR) antibody [4], [5], [6], [7] was key to provide a model of antibody-mediated disease primarily involving the autonomic nervous system. However, in the majority of neuroinflammatory disorders, the autonomic nervous system will not be the primary target, and other neurological involvement (e.g. neuropathy) can mask the recognition of autonomic signs and symptoms [8]. In recent times, a growing number of studies have taken an interest in filling that gap by evaluating the type of dysautonomia present in different neuroinflammatory conditions, as well as its associated outcome and influence in patients’ quality of life [9], [10], [11], [12].

In this narrative review, we will revise the autonomic dysfunction that can be found in patients with paraneoplastic neurological syndromes (PNS), autoimmune encephalitides (AE) and autoimmune conditions primarily targeting the autonomic nervous system (i.e. autoimmune autonomic ganglionopathy), using for that purpose an antibody-based approach. Other autoimmune disorders that might present with autonomic features such as systemic autoinflammatory diseases (e.g. Sjögren, systemic lupus erythematosus), inflammatory neuropathies (e.g. Guillain-Barré syndrome) or demyelinating conditions (e.g. neuromyelitis optica) are beyond the scope of this review.