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Show Journal of Clillical Neuro-ophlhalmology 8(2): 113-114. 1988. Crocodile Tears Following the Guillain-Barre Syndrome Steven M. Bloom, M.D., and Thomas R. Hedges III, M.D. ci) 1988 Raven Press. Ltd .• New York The syndrome of crocodile tears usually follows trauma to the facial nerve or a Bell's palsy and is thought to be caused by aberrant regeneration. We present a patient with crocodile tears following the Guillain - Barre syndrome. To the best of our knowledge, this is the first such reported case. Key Words: Crocodile tears-Aberrant regenerationGuillain - Barre syndrome. From the Departments of Ophthalmology and Neurology, Tufts-New England Medical Center, Boston, Massachusetts. Address correspondence to Dr. T. R. Hedges III at TuftsNew England Medical Center, Box 381,750 Washington Street, Boston, MA 02111, U.S.A. 113 The crocodile's salivary and lacrimal glands are located so closely together that when the crocodile eats, it "cries." For centuries, people have equated these "false tears" with hypocrisy. Bogorad (1) in 1928 was the first to ascribe the syndrome of "crocodile tears," or the gustolacrimal reflex with lacrimation during eating in humans. We have observed a man with gustatory lacrimation following the Guillain-Barre syndrome. This previously unreported finding may have pathophysiologic implications regarding aberrant regeneration and the Guillain-Barre syndrome. CASE REPORT A 30-year-old man was well until he developed an upper respiratory infection. Two weeks afterward, he experienced 2 days of progressive weakness in all of his extremities with difficulty walking and paresthesias of his face and tongue. On admission to the hospital, his neurologic examination showed absent deep tendon reflexes, a stocking-glove distribution of sensory loss to pinprick, and bilateral peripheral facial nerve paralysis. Cerebrospinal fluid contained 1 red blood cell and 0 white blood cells per mm3 with 61 mg protein per dl. Electromyogram and nerve conduction studies showed increased latencies of the ulnar, median, and posterior tibial nerves. A diagnosis of Guillain-Barre syndrome was made. The patient was treated with multiple cycles of plasma exchange, and his extremity weakness and facial paralysis improved over the next month. However, several weeks into his illness, he began to complain of bilateral epiphora whenever he ate. Visual acuities were 20/25 in each eye. There was minimal bilateral orbicularis weakness and the puncta well opposed the normal tear film. Instillation of 10% fluorescein in both eyes demonstrated patency of the lacrimal drainage systems 114 5. M. BLOOM AND T. R. HEDGES III bilaterally. Extraocular movements were full and the remainder of the ocular examination was normal. No epiphora was noted during the examination. However, after several minutes of eating an apple, tears began to roll down both cheeks (Fig. 1). In addition, when asked to gently close his eyes, there was coincident contraction of the upper left and lower right orbicularis muscles. These findings have persisted after 1 year of follow-up. COMMENT The major diagnostic criteria for the GuillainBarre syndrome are progressive symmetric motor weakness and areflexia. Additional findings include mild sensory symptoms or signs, autonomic dysfunction, cardiac arrhythmias, and vasomotor symptoms. Cranial nerve palsies occur in over 50% of patients and most often involve the facial nerve bilaterally. Rarely, papilledema (2) or lid-lag (3) may occur. An ophthalmic variant called the Miller Fisher syndrome consists of total external ophthalmoplegia, ataxia, and areflexia. The Guillain- Barre syndrome is thought to be the result of a selective attack by macrophages and lymphocytes on the myelin of peripheral nerves. Although not the primary target, axons can be destroyed in severe cases (4). The gustolacrimal reflex consists of lacrimation during eating and occurs weeks to months after a facial nerve palsy. Associated findings include ipsilateral decreased psychogenic tearing, synkinesis of associated facial muscles (also noted in our patient), and increased nasal secretions while eating. Unfortunately, this embarrassing phenomenon is usually permanent, although it has been reported to disappear spontaneously. Crocodile tears most commonly follow Bell's palsy or a traumatic facial nerve paralysis. Additional causes previously reported include Herpes zoster, cerebrovascular accidents, congenital anomalies of in- ,f8Cl while eating an nervation (usually associated with a sixth or se~enth nerve palsy and Duane's syndrom~), acoustic neuroma, following surgical resech~n of the greater superficial petrosal nerve for mtract~ble headaches, syphilitic involvement of the ~~mculate ganglion, vasomotor rhinitis, and speCIfIC ear operations (5). . The pathogenesis of crocodile .tea~,s and other syndromes of "aberrant regener~tlOn. such as hemifacial spasm, oculomotor synkmesIs, and gustatory sweating is poorly understood .(6). The most popular theory is aberrant regeneraho~,where regenerating secretory fibers for the sali~ary glands become miswired to innervate the lacnmal gland. A second theory is ephaptic transmission, or "cross-talk," where electrical information from one axon inappropriately excites a neighboring axon (7). The third theory, which is thought to be the primary cause in cases of congenital crocodile tears, invokes a central mechanism. It is thought that the changes in the cell body that occur with axonal injury, including collateral sprouting from nearby axons, microglial proliferation, and hyperexcitability of the chromatolytic neuron, result in central reorganization. The last theory is denervation supersensitivity of the affected muscle or gland. One would expect ephaptic transmission to be the most likely cause of aberrant regeneration in patients with the Guillain - Barre syndrome since it is primarily a demyelinating disease. Although persistent ephaptic transmission has been reported in humans (8), it is possible that perineural inflammation was severe enough in our patient to cause axonal destruction, thus allowing for any of the other proposed mechanisms of aberrant regeneration to occur. REFERENCES 1. Bogorad FA (introduction and translation by Seckersen A). The symptom of crocodile tears. JHistal Med Allied Sci 1979;34:74-9. 2. Farrell K, Hill A, Chuang S. Papilledema in Guillain-Barre syndrome-a case report. Arch Neural 1981;38:55-7. 3. Keane JR. Lid-lag in the Guillain-Barre syndrome. Arch NeuroI1975;32:478-9. 4. Haymaker W, Kernohan JW. The Landry-Guillain-Barre syndrome. A clinicopathologic report of fifty cases and a critique of the literature. Medicine 1949;28:59-141. 5. Regenbogen L, Stein R. Crocodile tears associated with homolateral Duane's syndrome. OphthalnlOlagica 1968;156:353-60. 6. Sibony PA, Lessell 5, Gittinger JW Jr. Acquired oculomotor synkinesis. Surv OphthalmaI1984;28:382-90. 7. Gardner WJ. Cross talk-the paradOXical transmission of a nerve impulse. Arch NeuroI1966;14:149-56. 8. Fullerton PM, Gilliatt RW. Axon reflexes in human motor nerve fibers. J Neural Neurosurg Psychiatr 1965;28:1-11. |
