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Show JountiJl of Clinical Neuro- ophtluJlmology 10( 2): 121- 123, 1990. Lid Lag and the Guillain- Barre Syndrome Ersin Tan, M. D., Tillay Kansu, M. D., Pinar Kirkali, M. D., and Turgut Zileli, M. D. © 1990 Raven Press, Ltd., New York Lid lag in Guillain- Barre syndrome is not a well- known feature of the disease, Here we present two cases of Guillain- Barre syndrome with lid lag, In its formation, supranuclear levator dysfunction was the possible cause. Key Words: Guillain- Barre syndrome- Lid lag. From the Hacettepe University Hospitals, Department of Neurology, Neuro- ophthalmology Unit, Ankara, Turkey. Address correspondence and reprint requests to Dr. Ersln Tan at Department of Neurology, Neuro- ophtha! mo! ogy Unit, Hacetltepe, University Hospitals, Ankara, Turkey. 121 Guillain- Barre syndrome may present a variety of clinical complexes, some with unusual features ( 1). Although several weU- known eye movement abnormalities can be seen in the course of the Guillain- Barre syndrome and Fisher's variant, lid lag had been previously observed in only five patients in the literature ( 2- 3). In this article we present two patients with this rare sign, which developed in the acute phase of the Guillain- Barre syndrome, CASE 1 A 28- year- old woman was admitted with a history of weakness of the lower extremities after an upper respiratory tract infection. On the 2nd hospital day, the disease had progressed to the point where the patient had areflexic quadriparesis. Lid lag was observed in both eyes on downgaze, more prominently on the right ( Fig. 1), without associated frontalis contractions. Eyelid positions seemed in normal positions on other gazes, Ocular movements were normal with no facial weakness, There was marked diminution in perception of joint position, vibration, pain, and temperature in a stocking and glove distribution. Routine blood, urinalysis, thyroid function tests, and urine porphyrin levels were within normal limits. In cerebrospinal fluid ( CSF) examination there was an albumino- cytological dissociation ( CSF protein 620 mg/ 100 ml without any cells). Marked slowing of the motor and sensory conduction velocities was noted in all extremities, predominantly at distal sites. Twenty days later, she began to recover and the lid lag disappeared gradually without any treatment. CASE 2 A 55- year- old man was admitted with a 3- day history of weakness in his lower extremities. There 122 FIG. 1. Marked limitation of upper lid descent on downgaze bilaterally prominent on the right ( case 1). E. TAN ET AL. was no history of recent viral infection. On neurological examination, mild paraparesis with diminished deep tendon reflexes was noted. He had also a slight decrease in pin sensation in a stocking distribution. One day later he rapidly developed areflexic quadriparesis with facial diplegia. Extraocular movements were normal. Tracheostomy was performed because of respiratory insufficiency. On the 4th hospital day, bilateral lid lag was observed on downgaze ( Fig. 2). Eyelid positions were normal on other gazes. His routine blood, urinalysis, and thyroid function tests, and urine porphyrin levels were normal. The CSF examination showed no cells and a protein value of 320 mg/ lOO ml. Two weeks after admission he began a rapid recovery and lid lag disappeared without any treatment. DISCUSSION Lid lag is a limitation of upper lid descent on downgaze, often indicative of endocrine disease. It must be differentiated from eyelid retraction, which is the retraction of the lid from its normal position in relation to the limbus in a static state, and may be seen in any gaze position, though rarely on downward gaze. Posterior commissure lesions may cause bilateral lid retraction ( Collier's sign) when looking straight ahead or slightly upward, though lid retraction disappears on downgaze. However, lid lag is usually seen on attempted downward gaze. In our patients, lid lag was only observed on downward gaze. Lid lag has been observed in other conditions such as extrapyramidal syndromes ( postencephalitic parkinsonism, progressive supranuclear palsy, etc.), congenital disorders ( congenital myotonia), hyperkalemic or hypokalemic familial periodic paralysis, and myotonia dystrophica, but the pathogenesis is not fully understood ( 4). In Guillain- Barre syndrome, unusual ocular abnormalities such as weakness in eyelid closure with or without facial weakness could be seen and it is suggested that they were limited forms ( 1). In 1975, Keane described two cases of GuillainBarre syndrome with lid lag of possible supranuclear origin ( 2). In 1988, Neetens and Smet observed that bilaterality suggests central origin, as the levator palpebra receives stimuli from both crossed and uncrossed fibers ( 3). Their case 3 had basically one lid with lag, and the other two cases, though bilateral, seemed to have a direct association with facial weakness. According to Neetens and Smet, lid lag results from unopposed contraction of the levator with weakness of orbicularis muscle. In Keane's article he speculated that lid lag in Guillain- Barre syndrome is possibly due to supranuclear dysfunction, as complete facial weakness has a minimal or no effect on lid position during downgaze. We agree with Keane's hypothesis that lid lag in Guillain- Barre syndrome was probably due to supranuclear dysfunction, because: ( a) there was bilaterality of the lid lag; ( b) in our first case, lid lag was not associated with facial weakness; and ( c) in FIG. 2. Demonstrating bilateral lid lag on downgaze during the acute phase of Guillain- Barre syndrome ( case 2). J Om Neuro- uphthal: '/, Vul 1/), No 2, J9~ 1I LID LAG IN GUILLAIN- BARRE SYNDROME 123 case 2, lid lag disappeared after the resolution of facial weakness. The lid lag in our two patients indicates that this finding is one to look for in patients with GuillainBarre syndrome, and should be included among the ocular signs of Guillain- Barre syndrome. The underlying mechanisms may need further explanation. REFERENCES 1. Ropper AH. Unusual clinical variants and signs in GuillainBarre syndrome. Arch NeuroI1986; 43: 1l50-- 2. 2. Keane JR. Lid- lag in the Guillain- Barre- Strohl syndrome. Arch NeuroI1975; 32: 478- 9. 3. Neetens A, Smet H. Lid Lag in Guillain- Barre syndrome. Arch NeuroI1988; 45: 104fr7. 4. Miller NR. Walsh and Hoyt's clinical neuro- ophthalmology. Vol. 2. 4th ed. Baltimore: Williams & Wilkins, 1985: 94S- 57. JClin Neuro- ophthalmol, Vol. 10, No. 2, 1990 |
