A 23-year-old woman with near vision difficulty
Digital Journal of Ophthalmology 2007
Volume 13, Number 5
January 9, 2007
|A 23-year-old female university student was admitted to the Department of Neurology with near vision difficulty and fatigue for 2 months. She was on neither eye drops nor any systemic medication. Her medical history revealed that she had presented to her physician with left shoulder pain six months ago. A mediastinal mass was found and a thymectomy was planned. She had an erythrocyte sedimentation rate (ESR) of 70 mm/h, moderate leukopenia (2600/uL) and thrombocytopenia (118,000/uL) at that time. Hematological investigations including bone marrow aspiration and biopsy all appeared normal. A thymectomy was performed through left sided thoracotomy. The mass was a benign thymoma (type B1) with cystic components. A left sided Horner's Syndrome developed just after the surgery and resolved over time. She reported a six to eight kilogram weight loss in four months following the operation. Two months after the operation she noticed difficulty with near vision.|
|Her physical examination was normal. The neurological examination was normal with the exception of the eye findings. Ophthalmological examination revealed a slight ptosis of 1-2 mm on the left with normal levator muscle function. Her visual acuity was 20/30 OU (20/20 with +0.75 D correction) bilaterally. Retinoscopy with and without cycloplegia showed a refractive error of +0.75 D and dynamic retinoscopy revealed that she had no accommodation on either side. Slit lamp biomicroscopy, funduscopic and motility examinations were normal. Pupil diameters were six millimeters on both sides and pupillary reactions were sluggish for both light and near. After 1% pilocarpine eyedrop, both eyes constricted. Two days later, 0.1% pilocarpine eyedrops were applied. Her pupils became smaller and her reading improved.|
|The ESR was 49mm/h. Blood leukocyte and platelet counts were 2700/uL and 196,00/uL, respectively. The hemoglobin level was 10.7g/dL. Vitamin B12, folate, and thyroid function tests were normal. Routine electromyography (EMG), repetitive nerve stimulation test, single fiber EMG, sympathetic skin response study, cranial magnetic resonance investigation, thoracic computerized tomogram (CT), routine cerebrospinal fluid examination and abdominal ultrasonogram were all normal. Gastric emptying was remarkably delayed. |
Tests for brusellosis, HIV infection and syphilis were negative. Serum anti-HU, Anti-Ri, Anti-Yo and Collapsin response-mediator protein-5 (CRMP-5) IgGs by western blot were normal. Ach receptor (muscle) blocking antibody titer was 21% (normal:0-25%).
The following tests were abnormal: Ach receptor (muscle) binding antibody [2,44nmol/L (normal: IU 0.02 nmol/L)], striational antibody [1/122880 titer (normal: <1/60)], Ach receptor (muscle) modulating antibody [83% (normal: 0-20%)]. She was seropositive for ganglionic AChR antibody and GAD65 antibody.
|Intravenous Immunglobulin was administered at a dose of 0.4 g/kg a day for five days and repeated at a single dose once a month for six months. A pilocarpine eyedrop was offered so that she could read with one eye and use the other eye for distance vision. This attempt did not provide a functional improvement. Eyeglasses were prescribed then for distance and near. One year later, she still had blurred vision. Her blood leukocyte and platelet counts were 3200/uL and 151,000/uL, respectively. Her hemoglobin level was 12.2 g/dL. A CT scan of the chest was normal.|
|Diagnosis and Discussion|
|This young woman had bilateral Adie’s pupil and delayed gastric emptying as well as leukopenia and mild anemia. The mentioned smooth muscle related dysfunctions seem to be a consequence of autonomic involvement primarily affecting the pupillary parasympathetics. Unilateral Adie’s pupil is usually a benign condition (1). It can also be associated with neoplastic processes (2,3,4). Bilateral Adie’s pupil may be due to dysautonomia, polyneuropathy, Sjogren’s syndrome, or syphilis (5,6,7). Recently, two cases of small cell lung cancer have been reported with bilateral Adie’s pupil (4). Together with the anti-Hu positivity, the authors suggested that the ophthalmological abnormality was paraneoplastic.|
Thymoma associated neurological paraneoplastic syndromes include myasthenia gravis, autonomic neuropathy, myositis, encephalitis, limbic encephalitis, Isaac’s syndrome, and hearing loss (8). A unilateral Adie’s pupil associated solitary thymoma has been reported previously (9). It has been diagnosed with CT but not confirmed histologically.
To our knowledge, the patient reported here is the first reported bilateral Adie’s pupil associated with histologically proven thymoma. Her Ach receptor (muscle) binding antibody, striational antibody, Ach receptor (muscle) modulating antibody, ganglionic Ach receptor antibody, and GAD65 antibody levels were elevated. These antibodies are associated with thymic abnormalities (8). Vernino and Lennon reported thymoma associated autoantibodies and their clinical significance (8). A high titer of ganglionic Ach receptor antibody has been detected in the sera of three out of ten gastrointestinal dysautonomic patients with thymoma although they found no difference in seroprevalance of this antibody compared with patients without a neurological diagnosis.
Together with the detected thymoma and existing autoantibodies associated with thymic abnormalities, the autonomic involvement in this patient seems to be paraneoplastic.
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