Intermittent pupillary dilation in a young woman

SURVEY OF OPHTHALMOLOGY

VOLUME 31 - NUMBER 1 . JULY-AUGUST

1986

CLINICAL CHALLENGES RONALD M. BURDE AND PAUL HENKIND, EDITORS

Intermittent Pupillary Dilation in a Young Woman NEIL

R. MILLER,

M.D.

Comments by John L. Keltner, M.D., John W. Gittinger, Jr., M.D., and Ronald

The IVilmer

Ophthalmological

(In keep@ with the purpose the article.)

Institute,

Johns

Hopkins

Hospital,

Baltimore.

M. Burde, M.D.

Maryland

of a clinicalfiathological conference. the abstract and kg words appear at the end ?#

Case Report. A 28-year-old woman in otherwise good general health noted the onset of pounding, right-sided headaches. The headaches would occur about once every one to two months. There was no aura. The headaches would last from several hours to one or two days. They were not relieved by aspirin or Tylenol, but the patient found that ifshe went into a dark room and lay down, she would feel much better. The headaches were occasionally associated with nausea and photophobia but not with vomiting. During several headaches, the patient noted that her right pupil became dilated, although she was not aware of blurred vision in the right eye. The pupil would remain dilated for several minutes to several hours and then return to normal. When examined by an ophthalmologist, no specific abnormalities were found. What further history wouldyou obtain in this patient? What would_vou expect tojnd ifrou examined this patient while her right pupil was dilated? Do -YOU think that an?, of the pupillar?, jndings would change if they were unassociated with headache? What is the appropriate management of this patient? Is either a CT (MRI) or angiogram warranted - why or whv not?

Comments Comments by John L. Keltner, M.D., Departments of Ophthalmology, Neurology, and Neurosurgery. UnioersiQ of California at Davis, Davis, California Dr. Miller reports a 28-year-old woman who was in good health except for pounding, right-sided headaches occurring every one to two months. The headaches lasted hours to days, were relieved by rest in a dark room, and were associated with nausea and photophobia. It is of interest that during several headaches her right pupil became dilated, but there was no blurred vision. The dilation would last several hours and then become normal. Further history we might ask this patient is whether there is a family history of migraine, and whether she ever experiences diplopia or ptosis during any of the headache attacks. A variety of ocular, autonomic and other nervous system dysfunctions have been associated with migraines. These include retinal migraine, migraine, ophthalmoplegic Horner’s syndrome, episodic mydriasis, focal spasms of the iris dilator muscle. and a variety of visual phenomena. The greatest worry in any patient of this type is whether she could harbor more serious neurologic

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3 I (1) July-August

1986

disease, such as an aneurysm or a neoplastic compressive lesion. I believe we can emphatically state that any periodic episodic phenomena involving solely the pupil is the result of autonomic instability in either the sympathetic or parasympathetic nervous system and has not been associated with more serious neurologic disease. While certainly patients with classic migraine symptoms and other patients have had scintillating visual phenomena associated with z teriovenous malformations, there is not, to my knowledge, an association of intermittent pupillary dilation with a more serious neurologic syndrome.“J* The appropriate management for this particular patient would be to treat her for migraine headaches and forget about the pupillary phenomenon. I believe that no neuroradiologic intervention is necessary in this particular case. However, once any patient with migraine develops permanent neurologic sequellae, such as a third nerve paresis, visual field deficit, or other permanent neurologic phenomena, then I believe appropriate neuroradiologic studies should be undertaken.2~6~“*‘8~22 But with intermittent unilateral pupillary mydriasis, the patient should simply be followed and reassured that he or she has no more serious neurologic problems. Some patients have been reported with unilateral pupillary mydriasis without headache,6*8 and these patients have been found to be free of any neurologic or systemic symptomatology. These may well represent a variant of migraine or vascular autonomic instability, but it is difficult to prove without other associated features. Examining the patient during one of these attacks may show a pupil which is either unreactive or fully reactive, depending on the etiology of the mydriasis. Some authors believe that mydriasis during migraine attacks represents a variant ofophthalmoplegic migraine,6,2g while others believe it represents intermittent sympathetic hyperactivity.g,‘4 Tadpole-shaped pupils described by Thompson and associates2’ are felt to be a phenomenon of segmental spasm of the iris dilator muscle, which produces intermittent pupillary distortion of brief duration with a definite or probable relationship to the migraine syndrome. It is unclear in these patients with unilateral pupillary mydriasis whether all of them represent sympathetic hyperactivity or whether indeed some may have parasympathetic paralysis.*O Vijayan and Watson 24 discuss the pericarotid syndrome in six patients who demonstrated oculosympathetic paralysis, ipsilateral head pain, and anhidrosis over the forehead. This is a variant of cluster headache syndrome.‘6,24 Ophthalmoplegic migraine is most likely due to a delayed ischemic neuropathy of the third cranial

MILLER ET AL

nerve, possibly related to swelling of the walls of the carotid or basilar artery leading to the occlusion of the smaller vessels which supply the involved cranial nerves. The ophthalmoplegia suggests that ischemia leads to a border-zone infarction of the nerves similar to that seen in diabetic ischemic neuropathy. Migraine headaches are a primary disorder of the cerebral blood vessels with a variety of autonomic nerve system dysfunctions. In patients with classic a wave of diminished perfusion Bows migraine, cephalad across the territories of the major cerebral arteries, whereas patients with common migraine show no such changes. 5,7,12.3’ Since the brain metabolism has not been measured, it is not known whether a deficit of blood relative to tissue needs occurs (ischemia) or whether cerebral blood flow decreases in proportion to the diminished metabolism, suggesting a primary metabolic or neurogenic pathoresonance imaging Perhaps magnetic genesis. (MRI), with its potential for measuring blood flow and metabolism simultaneously, will establish whether headache, nausea and neurologic symptoms are various manifestations of one disorder or common symptoms of several entities.’ Comments by John W. Gittinger, Jr., M.D., Division of Ophthalmology, University of Massachusetts, Worcester, Massachusetts Headaches and pupillary dilation raise the specter of neurological catastrophe. The bad reputation of anisocoria, acquired in emergency rooms and intensive care units where patients with severe head trauma are likely to be encountered, is largely undeserved. Even persistent unilateral mydriasis in a conscious, alert patient has only once been reported as the sole manifestation of an internal carotid-posterior communicating artery aneurysm.6,‘5 The inveterate pupil-watcher, H. Stanley Thompson, states categorically that “any patient who walks into an ophthalmologist’s ofice with an isolated internal ophthalmoplegia has a peripheral problem (e.g. Adie’s, drops, glaucoma, injury) until proven otherwise.“lg The problem presented by Dr. Miller’s patient, however, is that of a history of transient pupillary dilation in the face of a currently normal examination. Episodic unilateral mydriasis has been reported with seizures, usually associated with conjugate deviation of the eyes. 3” Intermittent dilation of the pupil is seen in some patients with Horner’s syndrome,25 and intermittent Horner’s syndrome is a feature of cluster headaches. Pharmacological dilation is probably the most common cause of transient anisocoria.” None of these situations is present. What remains are cases where no cause is found.8

INTERMITTENT

PUPILLARY

DILATION

Variable anisocoria, often with alternation in the side of the enlarged pupil, is observed in normals, increasing in incidence with age.” Sometimes the pupillary border is irregular (“amoeboid,” “tadpole-shaped”), suggesting segmental spasm of the dilator pupillae. ?’ A recent series supports the proposition that episodic unilateral mydriasis is related to migraine.‘” perhaps representing a forme fruste of ophthalmoplegic migraine, although affecting different populations. In patients examined by Woods and colleagues during the acute attack,2q the pupils were unequal and the reaction to light of the larger pupil was decreased. There was no ptosis, and when accommodation was tested, it was equal bilaterally. These authors conclude, “Recurrent transient mydriasis without extraocular involvement has not, to our knowledge, been reported in any case of aneurysm, cerebral herniation, diabetes, or brain tumor.” I would reassure this patient, tell her that I would be available if her symptoms worsened or changed, and perform no further workup.

Comments b_yRonald M. Burde, M.D., Departments of Ophthalmology and Neurology and Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri This patient presents with a rather characteristic history of common migraine. The prodromal phase of common migraine may be so ill-defined that if it exists it may be ignored. It may precede the headache by hours and can include any of the following: depression. a feeling of ill-being, gastrointestinal distress, fatigue, and fluid retention. Thus, further confirmatory historical information should be aimed toward uncovering a prodromal phase, if it exists. The headache phase of common migraine is generally described as one in which the patient experiences a unilateral or generalized pulsating pain often accompanied by nausea or vomiting or both and a preference for dark, quiet places. A relative paraphrase of the history is given by Dr. Miller. It is estimated that 25-29% of women and 15-19% of‘ men suffer from migraine,‘R and 80% of these attacks would fall into the classification of “common migraine. ” Since the familial incidence of‘ migraine is reported to occur in 65-90% of patients queried.’ the presence of a family history of migraine, if elicited, woulcl be of more than passing interest. In addition, migraine is often associated with social factors such as the completion of a stressful period or task, trigger agents such as tyramineor phenylalanine-containing foods. red wines or a reduction in caffeine intake. Inclusion or exclusion

hi

TABIX Etiolo~v of E@odic

PARASYMPATHETIC

I Pu@la!,l

Dilation

DYSFUNCTION

Paresis

Uncal herniation Seizure disorders Migraine Hyperactivitv

Cyclic &lomotor Parasympathetic SYMPATHETIC Paresis (Horner’s)

paresis spasm DYSFUNCTION

Qmpathetic illternating

&&ractirlifv Anisocoria -

BENIGN

PUPILLARY

(Claude Bernard syndrome) secondary to cervical cord lesions DILATION

ofsuch associated factors, by history, would he most helpful. In a young woman it is important to delve into the patient’s menstrual history. Common migraine tends to be more intimately related to hormonal changes in women than is classic migraine (prodrome-aura-headache). Since migraine attacks have been induced de novo or increased in severity in women placed on oral contraceptives,” exclusion of oral contraceptive use may be lifesaving, as these women appear to be at increased risk for cerrbrovascular accidents. At this point there is little doubt that Dr. Miller’s patient is suffering from common migraine, superimposed upon which is the finding of episodic pupillary dilation. II is important, of course, to yuestion the patient about the simultaneous occurrence of ptosis and diplopia, but I would he surprised if a person who notes unilateral pupillary dilation would not also spontaneously report the presence of these findings. Purely for intellectual satisfaction, it would be interesting to know if the blurring ofvision was greater for near than for distance, suggesting involvement of the ciliary muscle as well as the sphincter muscle of the pupil. In either case, my course of action would not be altered. The problem of episodic pupillary dilation has been addressed recently by Burde, Savino, and Trobe.” Basically, there are three possible etiologies listed with their subsets in Table 1. This patient either has isolated parasympathetic paresis associated with migraine, in which case during the attack the patient will have a decreased light reaction (direct and consensual), decreased near response and decreased accommodation,“,” or this patient has benign pupillary dilation, in which case the patient will maintain brisk light and near papillary reflexes ‘t.lLx27 As has been

pointed

out. Walsh

and

Hoyt”’ and

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3 I( 1) July-August

1986

MILLER

Payne and Adamkiewicz I5 have each observed one case of internal ophthalmoplegia as the initial sign of posterior communicating artery aneurysm, but such cases are so rare that even on initial presentation in the setting of known migraine, no radiologic studies are indicated. In this case in which the patient has experienced repeated bouts of pupillary dilation without involvement of the extraocular muscles, this dictum is absolute.2g Unless these headaches are debilitating, I would not recommend the use of prophylactic medications. The clinical points of greatest importance are: (1) the diagnosis of headache syndromes is dependent upon the examiner’s aural acuity, i.e., from the history this patient has common migraine; and (2) all women who suffer from migraine and who use oral contraceptives should be advised to discontinue their use.

Il. 12.

13.

14. 15.

16. 17. 18. 19.

20. 21.

References

22.

1.Appenzeller 2. 3.

4. 5. 6. 7. 8. 9. 10.

0, Feldman RG, Friedman AP: Migraine, headache, and related conditions - Panel 7. Arch Nemo1 36.784-805, 1979 Bailey TD, O’Connor PS, Tredici TJ, Shacklett DE: Ophthalmoplegic migraine. J Clin Neuro-ophthalmol 4:225-228, 1984 Burde RM, Savino PJ, Trobe JD: Anisocoria and abnormal pupillary light reactions, in Burde RM, Savino PJ, Trobe JD: Clinical Decisions in Neuro-Ophthalmology. St Louis, CV Mosby, 1985, pp 22%233 Dalessio DJ (ed): Wolfs Headache and Other Head Pain. New York, Oxford University Press, 1980, ed 4, p 111 Dalessio DJ: Is there a difference between classic and common migraine? Arch Neural 42:275276, 1985 Edelson RN, Levy DE: Transient benign unilateral pupillary dilation in young adults. Arch New01 31:12-14, 1974 Hachinski V: Common and classic migraine. One or two entities? Arch Neural 42:277, 1985 Hallett M, Cogan DG: Episodic unilateral mydriasis in otherwise normal patients. Arch Ophthalmol 84: 130-136, 1970 Herman P: Severe headaches, large pupils, and mitral valve prolapse in young women. Neurology 33(Suppl2):144, 1983 Johnson SF, Moore RJ: Transderm pupil and confusion in a IO year old. Ann Neural 13r583, 1983

23. 24. 25.

26. 27. 28. 29. 30. 31.

Kattah JC, Luessenhop AJ: Resolution of classic migraine after removal of an occipital lobe AVM. Ann Neural 7:93, 1980 Lauritzen M, Olesen J: Regional cerebral blood flow during migraine attacks by xenon-133 inhalation and emission tomography. Brain 107:447-461, 1984 Loewenfeld IE: ‘Simple, central’ anisocoria: a common condition, seldom recognized. Trans Am Acad Ophthalmol OtolaTngol 833:OP832-839. 1977 Miller NR: Walsh and Hoyt’s Clinical Neuro-Ophthalmology, Baltimore, Williams & Wilkins, 1985, vol 2, ed 4, p 517 Payne J, Adamkiewicz J Jr: Unilateral internal ophthalmoplegia with intracranial aneurysm. Am J Ophthalmol 68:34%352, 1969 Riley FC, Moyer NJ: Oculosympathetic paresis associated with cluster headaches. Am J Ophthalmol 72:763-768, 1971 Salmon ML, Winkelman JZ, Gay AJ: Nemo-ophthalmic sequelae in users of oral contraceptives. JAMA 206:85-91, 1968 Spector RH: Migraine. Sum Ophthalmol29:193-207, 1984 Thompson HS: The pupil, in Lessell S, van Dalen JTW (eds): Neuro-Ophthalmolou 1984, Amsterdam, Elsevier, 1984, vol 3, p 280 Thompson HS, Corbett JJ: Spasms of the iris sphincter. Ann Neural 8:547-549, 1980 Thompson HS, Zackon DH, Czarnecki JSC: Tadpole-shaped pupils caused by segmental spasm of the iris dilator muscle. Am J Ophthalmol96:467-477, 1983 Troost BT, Mark LE, Maroon JC: Resolution of classic migraine after removal of an occipital lobe AVM. Ann Neuml 5:19%201, 1979 Vijayan N: Ophthalmoplegic migraine: Ischemic or compressive neuropathy? Headache 2&300-304, 1980 Vijayan N, Watson C: Pericarotid syndrome. Headache 18: 244-254, 1978 Voorhies RM, Fraser RA: Transient mydriasis: In Horner’s syndrome following surgery. Neuro-Ophthalmologv lt295296, 1981 Walsh FB, Hoyt WF: Clinical Neuro-Ophthalmolo~, Baltimore, Williams & Wilkins, 1969, vol I, p 252 Walsh FB, Hoyt WF: Clinical Neuro-Ophthalmology, Baltimore, Williams & Wilkins, 1969, vol 1, p 253 Waters WE: Prevalence of migraine. J Neural Neurosurg Psvchiat 38:613-616, 1975 Woods D, O’Connor PS, Fleming R: Episodic unilateral mydriasis and migraine. Am J Ophthalmol 98:229-234, 1984 Zee DS, Griffin J, Price DL: Unilateral pupillary dilatation during adversive seizures. Arch Neural 30: 4OS-405, 1974 Ziegler DK: The headache symptom. How many entities? Brch Neural 42:273-274, 1985

Reprints

Abstract.

are not available.

A 28-year-old woman presented with a history of unilateral throbbing headache associated intermittently with transient pupillary dilation. The differential diagnosis and suggested method of investigation are discussed. Therapeutic recommendations are mentioned for the treatment of common migraine. (Surv Opbthalmol 31:65-68, 1986)

Key words.

headache

l

migraine

l

ET AL

pupil dilation