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Show ORIGINAL CONTRIBUTION Reversible Tonic Pupils Caroline A. A. Hulsman, MD, PhD and Christine T. Langerhorst, MD, PhD Abstract: A 36- year- old man with a remote history of Hodgkin lymphoma and a recent history of non- Hodgkin lymphoma ( NHL) developed tonic pupils and absent deep tendon reflexes in the lower extremities. One year later, pupils were normal except for slight iris segmental contraction to light. Over the next 2 years, the patient remained asymptomatic, and pupils remained unchanged. The NHL went into remission, and no other neurologic manifestations appeared. This is the first report of reversible tonic pupils. They may have a pathogenesis different from that typically seen in irreversible tonic pupils. (/ Neuro- Ophthalmol 2007; 27: 308- 309) A tonic pupil is characterized by a poor to absent constriction to light but a normal to strong constriction to a near stimulus ( 1). A tonic pupil dilates slowly and is supersensitive to topically applied cholinergic agents. An accommodation paresis is often present. When hyporeflexia or areflexia of the lower limbs is also found, it is called Adie syndrome, which is reported to be a chronic phenomenon. To our knowledge, Adie syndrome has not been previously documented as reversible. We report a single case of transient bilateral tonic pupils with areflexia of the lower limbs in a patient with lymphoma. CASE REPORT A 36- year- old white man was referred to our neuro-ophthalmology clinic by the departments of neurology and internal medicine for pupillary abnormalities. The patient had had Hodgkin disease at age 16 with complete remission after treatment with three cycles of mechlorethamine, vincristine, procarbazine, and prednisone, together with total lymph node irradiation, which led to hypothyroidism. At age 33, he was found to have a large Department of Ophthalmology, Academic Medical Center, Amsterdam, The Netherlands. Address correspondence to Caroline A. A. Hulsman, MD, PhD, Department of Ophthalmology, Academic Medical Center, Postbus 22660, 1100 DD Amsterdam, The Netherlands; E- mail: c. a. hulsman@ amc. uva. nl B- cell non- Hodgkin lymphoma ( NHL) of the spine, considered secondary to the treatment of Hodgkin disease. For the NHL, he was treated with cyclophosphamide, doxorubicin, vincristine, and prednisone. Several months later, a suspected NHL in the lung was treated with focal irradiation. At the time of our examination 1 year later, medication was limited to 0.1 mg/ day levothyroxine. He complained of headache, blurred vision in the right eye, and a large pupil in the right eye for 1 week. Uncorrected visual acuity was 20/ 20 in both eyes. In a normally illuminated room, the right pupil measured 7 mm, and the left measured 4 mm. In response to direct light, the right pupil constricted to 5 mm and the left pupil to 2.5 mm. The right pupil constricted further to a near stimulus. The pupil redilated slowly, whereas the left pupil redilated briskly. At slit lamp examination bunching of the pupillary border was observed over 3 clock hours in the inferior part of the iris of the right eye. No structural abnormalities of the iris were present. Otherwise results of the slit lamp and ophthalmoscopic examinations were normal. Visual fields, tested by confrontation, were normal, and there were no abnormalities of eyelids, eye movements, or ocular alignment. After application of 0.1% pilocarpine to both eyes, the right pupil constricted to the same level as the left pupil ( 4 mm), which showed no constrictive effect. Five weeks later, the patient returned with the same complaints about the left eye. The right eye showed the same signs as during the first visit. The left pupil now did not constrict to light but did have a normal near response and showed segmental constriction at the slit lamp. Deep tendon reflexes of the lower limbs were absent, but there were no other neurologic abnormalities. We prescribed reading glasses and topical 0.1% pilocarpine. On a follow- up visit 1 year later, the patient was asymptomatic. He did not need his reading glasses anymore, his uncorrected visual acuity was 20/ 20 in both eyes, and both pupils were of equal and normal size. They constricted normally to light and a near stimulus, although slit lamp examination still showed slight segmental contraction of the iris to light in both eyes. At that time, a recurrence of NHL in the duodenum was treated with chlorambucil and prednisone. Two years later, the ophthalmic examination remained unchanged. Biopsies of the duodenum showed no evidence of lymphoma. 308 J Neuro- Ophthalmol, Vol. 27, No. 4, 2007 Reversible Tonic Pupils J Neuro- Ophthalmol, Vol. 27, No. 4, 2007 DISCUSSION Tonic pupil is a benign disorder, occurring mostly as an isolated manifestation and rarely in orbital trauma, retinal photocoagulation, inflammation, infection, infiltrative processes, ischemia, or dysautonomia associated with malignant disease and amyloidosis ( 1). It is caused by damage to the postganglionic parasympathetic pupillomotor fibers. The incidence is 4.7 cases per 100,000 persons per year ( 1). Tonic pupil is unilateral in 80% of cases. When it is bilateral, each eye is usually affected in separate episodes, with the second eye becoming affected with a frequency of 4% per year ( 2). Within months to years of onset, the affected pupil becomes smaller, continuing to fail to constrict to light but constricting in response to a near stimulus. The accommodation paresis usually recovers. The typical near- light dissociation is explained by the hypothesis that dysfunction or loss of a certain number of ciliary ganglion fibers has a smaller effect on accommodation than it has on light reaction because the number of fibers that regulate accommodation is much larger than the number regulating the pupil constriction to a light stimulus. Reinnervation of the iris sphincter by surviving axons of the accommodative ciliary nerve fibers may play a role in allowing pupillary constriction to a near stimulus ( 1). The recovery of the pupillary light reaction in our patient is very unusual. Perhaps the pathogenesis is different from that in the typical tonic pupil ( 2- 4). In our case, the pupillary light response might have recovered because fibers were not destroyed but merely rendered dysfunctional. The fact that the pupils of our patient returned to normal size, rather than becoming smaller in time as occurs in most cases of tonic pupil, suggests that pupilloconstrictor fibers may have regenerated. In our patient, no signs of lymphoma were observed in the iris or in any other part of the eye, and the malignancy was in remission at the time of occurrence of the tonic pupils. The tonic pupils may have been unrelated to the lymphoma. However, neuropathic tonic pupils have been described as part of a generalized autonomic neuropathy, which can occur as a paraneoplastic manifestation. Tonic pupils have been described in a patient with carcinomatous polyneuromyopathy secondary to metastases of an unknown tumor ( 3) and in patients with the paraneoplastic polyneuropathy associated with type 1 anti- neural nuclear autoantibodies ( ANNA- 1, anti- Hu) secondary to small cell lung cancer ( 5). In our patient, no polyneuropathy was present. However, the tonic pupils may have been a very local manifestation of a paraneoplastic syndrome. REFERENCES 1. Miller NR, Newman NJ. Walsh and Hoyt's Clinical Neuro- Ophthalmology. 5th ed. Baltimore: Williams & Wilkins; 1998. 2. Thompson HS. Adie's syndrome: some new observations. Trans Am Opthalmol Soc 1977; 75: 587- 626. 3. Bell TA. Adie's tonic pupil in a patient with carcinomatous neuromyopathy. Arch Ophthalmol 1986; 104: 331- 2. 4. Haider S. Tonic pupil in lymphomatoid granulomatosis. J Clin Neuroophthalmol 1993; 13: 38- 9. 5. Campellone j y Hageboutros A. Adie's pupils in paraneoplastic ganglionopathy with ANNA- 1. Clin NeurolNeurosurg 2006; 108: 712^. 309 |
