title: Facial Weakness, Otalgia, and Hemifacial Spasm: A Novel Neurological Syndrome in a Case-Series of 3 Patients With Rheumatic Disease.
abstract: Bell palsy occurs in different rheumatic diseases, causes hemifacial weakness, and targets the motor branch of the 7th cranial nerve. Severe, persistent, and refractory otalgia having features of neuropathic pain (ie, burning and allodynic) does not characteristically occur with Bell palsy. Whereas aberrant regeneration of the 7th cranial nerve occurring after a Bell palsy may lead to a variety of clinical findings, hemifacial spasm only rarely occurs. We identified in 3 rheumatic disease patients (2 with Sjögren syndrome, 1 with rheumatoid arthritis) a previously unreported neurological syndrome of facial weakness, otalgia with neuropathic pain features, and hemifacial spasm. We characterized symptoms, examination findings, and response to therapy. All 3 patients experienced vertigo, as well as severe otalgia which persisted after mild facial weakness had completely resolved within 1 to 4 weeks. The allodynic nature of otalgia was striking. Two patients were rendered homebound, as even the barest graze of outdoor breezes caused intolerable ear pain. Patients developed hemifacial spasm either at the time of or within 3 months of facial weakness. Two patients had a polyphasic course, with recurrent episodes of facial weakness and increased otalgia. In all cases, otalgia and hemifacial spasm were unresponsive to neuropathic pain regimens, but responded in 1 case to intravenous immunoglobulin therapy. No patients had vesicles or varicella zoster virus in spinal-fluid studies. We have defined a novel neurological syndrome in 3 rheumatic disease patients, characterized by facial weakness, otalgia, and hemifacial spasm. As described in infectious disorders, the combination of otalgia, facial weakness, and 8th cranial nerve deficits suggests damage to the geniculate ganglia (ie, the sensory ganglia of the 7th cranial nerve), with contiguous involvement of other cranial nerves causing facial weakness and vertigo. However, the relapsing nature and association with hemifacial spasm constitute a unique part of this neurological syndrome.
authors.affiliation: From the Division of Rheumatology, Department of Neurology, The Johns Hopkins University, School of Medicine, Baltimore, Maryland, USA.
authors.forename: Julius
authors.initials: J
authors.lastname: Birnbaum
mesh_terms: D000328:Adult; D001172:Arthritis, Rheumatoid / Q000150:complications; D004433:Earache / Q000209:etiology*; D005152:Facial Muscles; D005260:Female; D019569:Hemifacial Spasm / Q000209:etiology* / Q000523:psychology / Q000628:therapy; D006801:Humans; D008875:Middle Aged; D018908:Muscle Weakness / Q000209:etiology* / Q000523:psychology / Q000628:therapy; D009437:Neuralgia / Q000209:etiology* / Q000523:psychology / Q000628:therapy; D012216:Rheumatic Diseases / Q000150:complications*; D012859:Sjogren's Syndrome / Q000150:complications / Q000523:psychology / Q000628:therapy; D013577:Syndrome; D016896:Treatment Outcome; D014717:Vertigo / Q000209:etiology