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Show Journal of Neuro- Ophthalmology 20( 1): 1- 2, 2000. © 2000 Lippincott Williams & Wilkins, Inc., Philadelphia Horner Syndrome With Equal- sized Pupils in a Case With Underlying Physiologic Anisocoria Michael L. Slavin, MD A case is presented in which Horner syndrome occurred on the side of the larger pupil in underlying physiologic anisocoria. The unusual coincidence resulted in ipsilateral ptosis, but with clinically equal- sized and normally reactive pupils. Key Words: Anisocoria- Cluster headache- Horner syndrome. The hallmark signs of oculosympathoparesis ( Horner syndrome) are unilateral blepharoptosis and pupillary miosis. I recently examined a patient with isolated unilateral blepharoptosis, but with equal- sized and normal pupils because of Horner syndrome, who was later shown to have underlying physiologic anisocoria. CASE REPORT A 47- year- old man described recent headaches, which were part of long- standing periodic headaches, usually lasting for several weeks each year. The headaches were localized to the left temporal region and radiated to the left eye. They were described as being very severe and would last up to 1 Vi hours. Recently, a left blepharoptosis was noted. Neuro- ophthalmologic examination was normal, except for the finding of 2 mm of left upper eyelid blepharoptosis with mild " reverse" blepharoptosis. Pupils were round, with a diameter of 3.5 mm in each eye in bright illumination and 4.5 mm in each eye in the dark. No apparent dilation lag of the left pupil in the dark was noted. Pupillary light reflexes were normal, without evidence of segmental palsy. Hydroxyamphetamine 1% eyedrops were instilled and after 40 minutes, the right pupil dilated to 7 mm, while the left pupil did not dilate ( Fig. 1). A diagnosis of Horner syndrome involving the third order neuron was made. The combination of head- Manuscript received June 14, 1999; accepted October 7, 1999. From the Division of Neuro- Ophthalmology, Department of Ophthalmology, Long Island Jewish Medical Center, The Long Island Campus for the Albert Einstein School of Medicine, Great Neck, New York. Address correspondence and reprint requests to Dr. Slavin, Long Island Jewish Medical Center, Department of Ophthalmology, 600 Northern Blvd., Great Neck, NY 11021. aches and Horner syndrome suggested cluster headache syndrome. Cyproheptadine was prescribed for the headaches. On follow- up 2 weeks later, the patient noted that the headaches and the blepharoptosis had improved. On examination, there was 1 mm of left blepharoptosis. Pupils were 3.5 mm on the right and 4 mm on the left in the light and 4 mm on the right and 4.5 mm on the left in the dark ( Fig. 2A). Pupillary light reflexes were normal. Hydroxyamphetamine 1% drops were instilled and showed minimal dilation of the left pupil and marked dilation of the right pupil ( Fig. 2B). DISCUSSION The clinical diagnosis of isolated Horner syndrome requires the presence of both unilateral blepharoptosis and pupil miosis, notwithstanding the fact that the anisocoria or blepharoptosis may be subtle ( 1,2). The above combination of signs is so well ingrained in physicians that cases with isolated unilateral blepharoptosis alone are usually not considered for pharmacologic testing with either cocaine or hydroxyamphetamine drops. The finding of reverse ptosis, when present, is a highly spe- F1G. 1. Photograph after the instillation of hydroxyamphetamine 1% eyedrops. Note the left blepharoptosis and relative dilation of the right pupil, consistent with a left third order ( postganglionic) neuron Horner syndrome. / 2 M. L. SLAV IN *•*. _^ FIG. 2. Photograph showing left blepharoptosis and relative dilation of the left pupil ( A). Photograph after the instillation of hydroxyamphetamine 1% eyedrops. Note marked dilation of the right pupil with minimal dilation on the left ( B). cific sign. Cluster headache may be associated with Horner syndrome in 5% to 22% of cases ( 2). Patients with cluster headache may develop transient oculosympatho-paresis, after which the blepharoptosis and miosis often become permanent. In my patient, the improvement in the oculosympathoparesis ( as witnessed by decreasing left ptosis and a mild response to hydroxyamphetamine of the left pupil) resulted in unmasking of a physiologic ( simple) anisocoria ( 3). The unusual coincidence of oculosympathoparesis affecting the larger pupil in physiologic anisocoria resulted in pupils which in every respect initially appeared normal. Dilation lag of the pupil in dark illumination, if it had been present, would have suggested sympathoparesis of the pupil. REFERENCES 1. Giles CL, Henderson JW. Horner's syndrome. An analysis of 216 cases. Am J Ophthalmol 1958; 46: 289- 96. 2. Grimson BS, Thompson HS. Raeder's syndrome. A clinical review. Surv ophthalmol 1980; 24: 199- 210. 3. Loewenfeld IE. " Simple, central" anisocoria: A common condition, seldom recognized. Trans Am Acad Ophthalmol 1977; 83: 832- 9. ' J Neuro- Opluhalmol. Vol. 20, No. 1. 2000 |
