Indian Journal of Medical Specialities

CASE REPORT
Year
: 2022  |  Volume : 13  |  Issue : 4  |  Page : 251--253

A paraneoplastic optic neuropathy revealing a pulmonary epidermal carcinoma


Yassine Mouzari, Issam Fiqhi, Belfaiza Soukaina, Taoufiq Abdellaoui, Mohammed Allaoui, Karim Reda, Abdel Bar Oubaaz 
 Department of Ophthalmology, Military Hospital Mohammed V, Rabat, Morocco

Correspondence Address:
Prof. Yassine Mouzari
Military Hospital Mohammed V, Rabat
Morocco

Abstract

Paraneoplastic optic neuropathy is a relatively rare ocular manifestation. We report the case of a patient with bilateral papilledema revealing a pulmonary squamous cell carcinoma. Paraneoplastic neuropathy is linked to an immune reaction by the presence of autoantibodies against collapsin responsive mediator protein 5 . Diagnosis is based on the determination of these autoantibodies and the search for occult cancer by PET scan.Treatment is often based on corticosteroid therapy and sometimes immunosuppressive treatment is necessary in the case of corticosteroid unresponsiveness.



How to cite this article:
Mouzari Y, Fiqhi I, Soukaina B, Abdellaoui T, Allaoui M, Reda K, Oubaaz AB. A paraneoplastic optic neuropathy revealing a pulmonary epidermal carcinoma.Indian J Med Spec 2022;13:251-253


How to cite this URL:
Mouzari Y, Fiqhi I, Soukaina B, Abdellaoui T, Allaoui M, Reda K, Oubaaz AB. A paraneoplastic optic neuropathy revealing a pulmonary epidermal carcinoma. Indian J Med Spec [serial online] 2022 [cited 2023 Feb 1 ];13:251-253
Available from: http://www.ijms.in/text.asp?2022/13/4/251/358775


Full Text



 Introduction



Paraneoplastic optic neuropathy is a relatively rare ocular manifestation[1],[2] that often occurs at an advanced stage of lung or gynecological cancer. It is sometimes indicative of occult cancer, which can be diagnosed early and managed appropriately to improve the patient's prognosis.[2] We report the case of a patient with bilateral papilledema revealing a pulmonary squamous cell carcinoma.

 Clinical Case Report



We report the case of a 72-year-old man, who was a heavy smoker. He presented to the emergency service with a progressive decrease in bilateral visual acuity in the context of an alteration of general condition. On ophthalmological examination, visual acuity was limited 6/60 according to the Snellen scale on both eyes; slit-lamp examination showed a relative afferent pupillary defect and nuclear cataract on both eyes. Funduscopy revealed bilateral papillary edema Stage II [Figure 1]. Fluorescein angiography showed diffusion in the papilla confirming the diagnosis of papilledema on both eyes [Figure 2].{Figure 1}{Figure 2}

An exhaustive etiological workup was performed. Several tests came back normal, in particular the biological inflammatory test, the infectious tests (viral serologies and tuberculosis test), the cerebrospinal fluid analysis, and immunological tests. Similarly, the orbito-cerebral magnetic resonance angiography was without anomalies. However, a chest X-ray revealed an interstitial syndrome and was completed by a chest computed tomography scan which revealed a suspicious pulmonary nodule classified as T4N2M1a [Figure 3]. To explore the histological type of this nodule, an anatomical pathology examination of a transbronchial biopsy revealed a squamous cell carcinoma [Figure 4]. A positron emission tomography (PET) scan did not reveal any distant metastases. After the elimination of other etiologies, we retained the diagnosis of paraneoplastic optic neuropathy. The patient received three intravenous boluses of 1 g of corticosteroids with oral relay at a dose of 1 mg/kg. The evolution was favorable, and visual acuity improved to 54/60 on the right and 30/60 on the left according to the Snellen scale. The funduscopy on the 10th day of treatment showed a regression of papilledema [Figure 5]. To complete the treatment, the patient was transferred to the oncology department for chemotherapy.{Figure 3}{Figure 4}{Figure 5}

 Discussion



The most common ocular manifestations of paraneoplastic syndrome are choroidoretinal involvement, which includes cancer-associated retinopathy syndrome, melanoma-associated retinopathy syndrome, paraneoplastic vitelliform maculopathy, and bilateral diffuse uveal melanocytic proliferation,[3],[4] whereas optic neuropathy is rarely described in the literature.[4] It is clinically manifested by a profound progressive visual acuity decline and papilledema.[4] It is often associated with retinitis, hyalitis, or neurological signs such as Lambert–Eaton myasthenic syndrome, paraneoplastic cerebellar degeneration, opsoclonus-myoclonus, and rhombencephalitis.[4] According to the literature, lung cancer is the first cause of paraneoplastic syndromes[5] which often occur at an advanced stage rarely revealing the cancer as in our case.[5] Paraneoplastic neuropathy is linked to an immune reaction by the presence of autoantibodies against collapsin-responsive mediator protein-5 (also called anti-CV2).[6] This protein is present in photoreceptor cells, retinal ganglion cells, and nerve fibers. Diagnosis is based on the determination of these autoantibodies and the search for occult cancer by PET scan.[7] Treatment is often based on corticosteroid therapy and sometimes immunosuppressive treatment is necessary in the case of corticosteroid resistance.[8] Treatment of the underlying tumor is important, as previous studies have shown that regression of the tumor size leads to a decrease in autoantibody levels and consequently to a regression of the ocular involvement.[8] The visual prognosis is often good,[9] which is consistent with our case, whereas some other cases studied had an unfavorable evolution.[10] Finally, the vital prognosis depends on the stage and histological type of the underlying cancer.

 Conclusion



Paraneoplastic optic neuropathy is an exceptional ocular manifestation that should always be considered, as it may be indicative of an occult cancer. Early diagnosis not only improves the visual prognosis but also the vital prognosis and quality of life of the patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

None.

Conflicts of interest

There are no conflicts of interest.

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