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Show Journal of Clinical Neuro-ophthalmo[ogy 8(4): 249-251, 1988. Melkersson's Syndrome Associated with Syphilis James R. Keane, M.D. © 1988 Raven Press, Ltd., New York The onset of the Melkersson-Rosenthal syndrome, manifested by bifacial paralysis and severe edema of the upper lip in a 25-year-old man, was followed shortly by signs of secondary syphilis. Key Words: Melkersson-Rosenthal syndromeSyphilis. From the Department of Neurology, LAc/USC Medical Center, Los Angeles, California. Address correspondence and reprint requests to Dr. J. R. Keane at 1200 North State Street, Los Angeles, CA 90033, U.S.A. 249 In 1928, Ernst Melkersson described the combination of recurrent facial palsy with facial angioneurotic edema. Rosenthal, in 1931, completed the "classic triad" of the Melkersson-Rosenthal syndrome by demonstrating that plicated tongue is a common accompaniment. Oligosymptomatic variants are readily accepted and many additional features, such as facial pain and paresthesias, tongue atrophy, proptosis, papilledema, and meningitis, occur occasionally (1-3). By 1967, more than 400 cases of the Melkersson syndrome had been described (1). However, the condition remains rare, as evidenced by the inclusion of only 1 patient in a study of 140 patients with recurrent facial palsy (4). In the following patient, secondary syphilis was the probable cause of the Melkersson syndrome. CASE HISTORY A 25-year-old man, with a past history of a traumatic right corneal scar, developed right facial weakness, followed in several days by weakness on the left side of his face. On admission to the Los Angeles County/ University of Southern California Medical Center 1 week after the onset of symptoms, he showed complete bifacial paralysis and severe symmetric swelling of his upper lip. The remainder of his medical and neurological examinations were normal. His tongue was unremarkable. He had no history of allergies or serious medical illnesses. Computed tomography (CT) scan and brainstem auditory evoked potential tests were normal, and his cerebrospinal fluid showed four mononuclear cells, a protein value of 117 mg/dl, a glucose value of 63 mg/dl, and a negative VDRL. Serum VORL and human immunodeficiency virus (HIV) titers were normal. A biopsy of his upper lip was interpreted as showing chronic inflammation "consistent with Melkersson-Rosenthal syndrome"; stains for tuberculosis were normal. 250 f. R. KEANE Several weeks after discharge, he developed a generalized erythematous skin rash, most prominent on the palms of his hands. A repeat VORL and an MHA-TP serum test for syphilis were positive, and he was treated with penicillin. Within a month, his face began to recover strength. Three months after onset, he showed moderate symmetric bifacial weakness with mild synkineses and a history of tearing of the left eye when eating. One year after this exam, he still showed mild symmetric facial weakness with severe facial synkineses: smiling would narrow both palpebral fissures, causing his eyes nearly to close (Fig. 1). His upper lip was mildly swollen and he said that, periodically, the swelling would become severe for several days at a time, everting the lip and interfering with his speech. DISCUSSION This patient's facial paralyses and severe recurrent lip edema are sufficient for a diagnosis of Melkersson's syndrome (1), despite the absence of a furrowed tongue or recurrence of facial weakness. The abnormal gustolachrymal reflex ("croc- FI~. 1. Patient following recovery showing residual mild upper lip swelling and involuntary eye closure dunng attempts to smile. . '.'/ '''''/ ",./ 8. No.4, 1988 odile tears") that developed during recovery is a well-known autonomic sign of aberrant facial nerve regeneration, usually associated, as in our patient, with severe motor synkineses (5,6). Concurrent eruption of the skin rash of secondary syphilis strongly suggests a causal role for syphilis in this case, Merritt et al. (7) found the seventh and eighth nerves to be the most commonly affected cranial nerves in syphilitic meningitis. They described 2 cases of bifacial palsy and 16 with unilateral involvement in their own series of 76 patients, and found 62 further cases of syphilitic facial paresis in the literature to 1946. Syphilis is well documented in the older literature as a cause of isolated facial (8) and bifacial (9) palsy, and secondary syphilis as an unusual cause of "Bell's palsy" (10) has recently been reconfirmed (11). Simultaneous, isolated bifacial palsy is a rare event with a host of causes. While Lyme disease has received considerable recent publicity, and sarcoidosis has always been high in the differential diagnosis of bifacial weakness, trauma and the sequelae of viral illness are probably the most frequent causes (12,13). Meningeal tumor (especially lymphoma) is the most ominous of relatively common causes. The occasional case of postinfectious bifacial weakness with areflexia (14) suggests that there is no sharp separation of postinfectious isolated facial weakness and the cranial nerve varieties of the Guillain-Barre syndrome. Whether syphilis in the present case is considered to be a rare cause of the Melkersson syndrome, or to have triggered its onset, or to have mimicked it, is probably a semantic quibble, considering the vague boundaries of the Melkersson syndrome. Of importance is the reminder to consider syphilis in cases of the Melkersson syndrome, and, reasonably, in most patients with Bell's palsy. REFERENCES 1. Hornstein OP. Melkersson-Rosenthal syndrome. In: Vmken PJ. Bruyn GW, eds. Handbook of clinical neurolozy, Vol. 8, Part 2. New York: Elsevier, 1970:205-40. 2. Llaus SN,. Brunsting LA. Melkersson's syndrome (persistent swelhng of the face, recurrent facial paralysis, and lingua phcata): report of case. Mayo C/ill Proc 1959;34:365-70. 3. Stevens H. Melkersson's syndrome. Neurolooy 1965'15: 263--6. " ' 4. Pitts SB, Adour KK, Hilsinger RL. Recurrent Bell's palsy: analysis of 140 patients. Laryn8oscope 1988;98:535-40. 5. Axelsson A, Laage-Hellman J. The gusto-lachrymal reflex. Acta Otolaryngol 1962;54:239-54. 6. Chorobski J. The syndrome of crocodile tears. Arch Neurol PSl/chiatrv 1951;65:299-318. 7. Merritt HH, Adams RD, Solomon, He. Neurosyphilis. New York: Oxford Umverslty Press, 1946:39~0. MELKERSSON'S SYNDROME AND SYPHILIS 251 8. Rickles GA. Syphilitic peripheral seventh nerve paralysis, discussion of a case. JNero Ment Dis 1945;102:376-8. 9. Strauss MJ. Diplegia facialis in early syphilis. Arch Dermatol Syph 1929;20:306-14. 10. Hutchinson J. Deafness and facial paralysis in the secondary stage of syphilis. Arch Surg (Lond) 1896;7:169-70. 11. Davis LE, Sperry S. Bell's palsy and secondary syphilis: CSF spirochetes detected by immunofluorescence. Ann Neurol 1978;4:378-80. 12. Rontal E, Sigel ME. Bilateral facial paralysis. Laryngoscope 1972;82:607-16. 13. Guard 0, Dumas R, Soichot P, Ballivet J. Diplegies faciales peripheriques isolees, a propos de 3 recentes observations. Lyon Medical 1977;237:469-72. 14. Shuaib A, Becker WJ. Facial diplegia with areflexia and ataxia: another Guillain-Barre syndrome variant? Neurology 1987;37(suppl 1):375 (Abstract). lOin Neuro-ophthalmol, Vol. 8, No.4, 1988 |
