title: [Small fiber neuropathy in a patient with complete Heerfordt syndrome manifesting as refractory facial pain].
abstract: We report a case of complete Heerfordt syndrome accompanied by the involvement of small fiber neuropathy (SFN) manifesting as refracory facial pain. A 30-year-old man presented with pyrexia, a 2-week history of facial burning pain, and difficulty of mastication. After admission to our hospital, neurological examinations showed bilateral facial pain, trigeminal motor palsy, left facial nerve palsy, bilateral sensory neural deafness, uveitis and swelling of the parotid gland. Other examinations revealed bilateral hilar lymphadenopathy, high serum titer of angiotensin coenzyme, and no response in a tuberculin-tested, non-caseating epithelioid granuloma from lip biopsy, leading to the diagnosis of complete Heerfordt syndrome. Mandibular skin biopsy with immunostaining for PGP 9.5 showed SFN. High-dose corticosteroids proved somewhat effective against SFN as facial pain, but reducing the corticosteroid dose proved difficult, as symptoms were refractory to other immunosuppressants and pain-control drugs such as anti-epileptics and anti-depressants. The patient died of acute pancreatitis 3 years after disease onset. Autopsy showed no granuloma in hilar lymph node, trigeminal nerve, cranial base, nerve root, and muscle. SFN in this case probably represent a cause of refractory facial pain.
authors.affiliation: Department of Neurology, The Jikei University Kashiwa Hospital.
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Hirai
authors.forename: Shinji
authors.initials: S
authors.lastname: Miyagawa
authors.forename: Kazutaka
authors.initials: K
authors.lastname: Matsui
authors.forename: Akira
authors.initials: A
authors.lastname: Kurita
mesh_terms: D000208:Acute Disease; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000328:Adult; D015415:Biomarkers / Q000032:analysis; D004916:Erythromelalgia / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D005157:Facial Pain / Q000188:drug therapy / Q000209:etiology*; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008334:Mandible; D010195:Pancreatitis; D012867:Skin / Q000378:metabolism / Q000473:pathology; D043222:Ubiquitin Thiolesterase / Q000032:analysis; D014608:Uveoparotid Fever / Q000150:complications* / Q000175:diagnosis / Q000473:pathology
chemical_list: D000305:Adrenal Cortex Hormones; D015415:Biomarkers; C482860:UCHL1 protein, human; D043222:Ubiquitin Thiolesterase