Table 1. Clinical features that raise suspicion for AGID.

Detection of neural autoantibody associated with dysmotility† Symptom characteristics that may warrant consideration of an immunotherapy trial: Extra intestinal neurologic (usually autonomic) symptoms Subacute onset Severe symptoms and failure of symptomatic medications (pro-motility agents)* Objective evidence of extraintestinal autonomic dysfunction Thermoregulatory sweat test (sudomotor function) Autonomic reflex screen (adrenergic and cardiovagal function) Personal or family history (1st degree relative) of autoimmunity or serological evidence of extra neurologic autoimmunity (e.g. anti-nuclear antibody) History of recent or past neoplasia or risk factors for cancer (e.g. long history of smoking)

Abbreviations: AGID, autoimmune gastrointestinal dysmotility.

^†type 1 anti-neuronal nuclear autoantibody (ANNA-1, also called “anti-Hu”)(3), ganglionic nicotinic acetylcholine receptors (AChR) containing α3 subunits(7-9), voltage-gated neuronal potassium channel-complex (VGKC);(1, 10) calcium-channel antibodies (N-type > P/Q-type), muscle AChR, striational, glutamic acid decarboxylase 65 (GAD65) and peripherin.(1, 11)

^*As immunotherapy is not without risk we generally reserve a trial for patients with severe symptoms refractory to pro-motility agents