Unilateral Adie Pupil as Sole Ophthalmic Sign of Anti-Hu Paraneoplastic Syndrome

Introduction: Ophthalmological manifestations of systemic malignancies can be either direct, metastatic or paraneoplastic. The latter are remote effects of carcinoma, often caused by autoantibodies. Ophthalmological manifestations include cancer-associated retinopathy, melanoma-associated retinopathy, opsoclonus-myoclonus syndrome or motility disorders due to effects on the neurological system. A unilateral tonic pupil is usually a benign finding but has also been described in the context of paraneoplastic syndromes, in some cases associated with anti-Hu antibodies. Case Reports: The authors describe 2 patients with bilateral symptomatic tonic pupils due to a paraneoplastic syndrome. Both patients had been treated for small cell lung cancer and had evidence of anti-Hu antibodies (autoantibodies against nuclei of neural cells) in serum and cerebrospinal fluid. Both had typical pupillary findings and hypersensitivity to diluted pilocarpine. The first patient also had sensory neuronopathy, the second affection of several cranial nerves. Discussion: To the best of our knowledge, to date no case of bilateral tonic pupils has been published due to a paraneoplastic disorder with evidence of autoantibodies. This is surprising, as it is probable that autoantibodies in paraneoplastic disorders affect both ciliary ganglions in a similar way.

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Bilateral Tonic Pupils with Evidence of Anti-Hu Antibodies as a Paraneoplastic Manifestation of Small Cell Lung Cancer

Wabbels

Elflein

Lorenz

et al. 2004

Ophthalmologica

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Anti-Hu antibodies, sensory neuropathy, and Holmes–Adie syndrome in a patient with seminoma

Müller

Prass²,

Zschenderlein³

2005

Neurology

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Anti-Hu Antibody-Associated Adie's Pupil and Paraneoplastic Sensorimotor Polyneuropathy Caused by Primary Mediastinal Small Cell Carcinoma

et al. 2019

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We report a woman with unilateral Adie's pupil associated with peripheral sensorimotor polyneuropathy, elevated anti-Hu antibody, and primary mediastinal small cell carcinoma (SCC). To our knowledge, this is the first report of Adie's pupil in a patient with mediastinal SCC. Although rare, Adie's pupil and sensorimotor polyneuropathy may be the first manifestation of cancer. Its rapid recognition facilitates an early diagnosis and treatment.

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Unilateral Adie Pupil as Sole Ophthalmic Sign of Anti-Hu Paraneoplastic Syndrome

Cited by 4 publications

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Bilateral Tonic Pupils with Evidence of Anti-Hu Antibodies as a Paraneoplastic Manifestation of Small Cell Lung Cancer

Bilateral Tonic Pupils with Evidence of Anti-Hu Antibodies as a Paraneoplastic Manifestation of Small Cell Lung Cancer

Anti-Hu antibodies, sensory neuropathy, and Holmes–Adie syndrome in a patient with seminoma

Anti-Hu Antibody-Associated Adie's Pupil and Paraneoplastic Sensorimotor Polyneuropathy Caused by Primary Mediastinal Small Cell Carcinoma

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