ABSTRACT
OBJECTIVE: To determine whether anti-Plexin D1 antibody (Plexin D1-immunoglobulin G [IgG]), which is associated with limb and trunk neuropathic pain (NP) and binds to pain-conducting small unmyelinated dorsal root ganglion (DRG) neurons, exists in patients with idiopathic painful trigeminal neuropathy (IPTN) and whether Plexin D1-IgG binds to trigeminal ganglion (TG) neurons. METHODS: We enrolled 21 consecutive patients with IPTN and 35 age- and sex-matched controls without NP (25 healthy persons and 10 with neurodegenerative diseases). We measured serum Plexin D1-IgG using a mouse DRG tissue-based indirect immunofluorescence assay (IFA) and by Western blotting (WB) using a recombinant human Plexin D1 (rhPlexin D1) accompanied by immunoadsorption tests with rhPlexin D1. The reactivity of Plexin D1-IgG toward mouse TG, brain, heart, and kidney was assessed by tissue-based IFAs. RESULTS: Serum Plexin D1-IgG was detected more frequently in IPTN than in controls by both IFA and WB (14.3% vs 0%, p = 0.048). Three Plexin D1-IgG-positive patients also had limb or trunk NP and commonly showed tongue pain. In tissue-based IFAs, IgG from 2 Plexin D1-IgG-positive patients immunostained small TG neurons, which was prevented by preincubation with rhPlexin D1. Moreover, Plexin D1-IgG immunostaining mostly colocalized with isolectin B4-positive pain-conducting unmyelinated TG neurons. IFAs of other tissues with the same IgG revealed weak immunoreactivity only in endothelial cells, which was prevented by preincubation with rhPlexin D1. CONCLUSIONS: Plexin D1-IgG, which binds to pain-conducting small TG neurons in addition to DRG neurons, can be present in IPTN as well as limb and trunk NP.
Subject(s)
Autoantibodies/blood , Intracellular Signaling Peptides and Proteins/immunology , Membrane Glycoproteins/immunology , Neuralgia/blood , Trigeminal Nerve Diseases/blood , Adult , Aged , Animals , Female , Ganglia, Spinal/metabolism , Humans , Immunoglobulin G/blood , Male , Mice , Middle Aged , Trigeminal Ganglion/metabolismSubject(s)
Autoantibodies/immunology , Autoimmune Diseases/immunology , Ku Autoantigen/immunology , Muscular Atrophy, Spinal/immunology , Myositis/immunology , Spinal Curvatures/immunology , Trigeminal Nerve Diseases/immunology , Aged , Autoantibodies/blood , Autoimmune Diseases/blood , Female , Humans , Ku Autoantigen/blood , Muscular Atrophy, Spinal/blood , Myositis/blood , Myositis/diagnostic imaging , Spinal Curvatures/blood , Trigeminal Nerve Diseases/bloodSubject(s)
Immunoglobulin G/blood , Orbital Diseases/blood , Trigeminal Nerve Diseases/blood , Trigeminal Nerve/pathology , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Orbital Diseases/complications , Orbital Diseases/drug therapy , Steroids/therapeutic use , Trigeminal Nerve Diseases/complications , Trigeminal Nerve Diseases/drug therapyABSTRACT
PURPOSE: The purpose of this study was to describe the clinical characteristics of a series of patients presenting with benign trigeminal sensory neuropathy. PATIENTS AND METHODS: We conducted a retrospective analysis of the clinical and pathologic characteristics of 23 patients presenting with facial numbness of unknown etiology. RESULTS: Patients presented with diverse medical histories but could be grouped into those with a connective tissue disorder, neurologic disease, psychologic problems, or a medical history of unknown significance. The age of the patient, the severity and distribution of the trigeminal neuropathy, and symptoms other than neuropathy closely reflected the patient's medical history. The majority of patients underwent magnetic resonance imaging, but the results did not facilitate the diagnosis of the condition or reflect the extent and severity of the symptoms. In 60% of patients, the symptoms remained unchanged during the course of the study and outcome was not influenced by medical treatment. CONCLUSIONS: The diagnosis and management of benign trigeminal sensory neuropathy remain a significant clinical challenge.
