Painful ophthalmoplegia in a patient with a history of marginal zone lymphoma

C Van Bogaert,A Wolfromm,N Trotta,S Goldman,L Rocq,V De Wilde,I Vierasu,C Mathey

doi:10.1186/s41824-021-00113-2

Abstract

A 73-year-old man with a history of marginal zone lymphoma was admitted to the emergency room for diplopia and ipsilateral headache. The Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) demonstrated intense and symmetrical hypermetabolism of the cavernous sinuses, and hypermetabolic lesions diffusely in the lymph nodes and bones. The diagnosis of high-grade relapse of lymphomatous disease was made. In this context, the homogenous and symmetric lesion of the cavernous sinuses, without any other encephalic or meningeal lesions, raised the hypothesis of a paraneoplastic origin. A plausible paraneoplastic link between the neuro-ophthalmological lesion and the malignant disorder is IgG4-related disease, a condition that may be associated with lymphoma. As in our case, this diagnosis is often presumptive because histopathological confirmation is difficult to obtain.

Highlights

Painful ophthalmoplegia refers to periorbital or hemicranial pain associated with ipsilateral oculomotor nerve palsy
The case here reported illustrates the value of cerebral 18F-FDG positron emission tomography (PET)/computed tomography (CT) images since this examination provided a clear demonstration of a homogenous and symmetric involvement of the cavernous sinuses that had not been seen on the CT and magnetic resonance imaging (MRI)
Since paraneoplastic syndromes are generally related to autoimmune processes, we considered the hypothesis that Tolosa Hunt syndrome (THS) in this case was a manifestation of a lymphoma-induced IgG4-related disease (IgG4-RD)

Summary

Background

Painful ophthalmoplegia refers to periorbital or hemicranial pain associated with ipsilateral oculomotor nerve palsy It may be caused by any process exerting a mass effect simultaneously on the internal carotid artery and the cavernous sinus and/or the superior orbital fissure. In the presence of painful ophthalmoplegia, the Tolosa Hunt syndrome (THS), firstly described in 1954, is a diagnosis of exclusion It is characterized by a unilateral headache associated with a paralysis of the ipsilateral third, fourth, and/or sixth cranial nerves caused by nonspecific granulomatous inflammation of the cavernous sinus, the superior orbital fissure, or the orbit (generally termed orbital pseudotumor). Outcome and follow‐up Six months later, the patient presented a DLBCL recurrence in the supra- and sub-diaphragmatic lymph nodes with no neurological symptom He was treated by Rituximabwith Gemcitabine/-, Dexamethasone, and Cisplatine (R-GDP), shifted to Rituximab with Gemcitabine, Dexamethasone, and Carboplatin (R-GDCarbo) for nephrotoxicity, with complete response after 3 cycles. Before the end of this therapy, the patient died from a septic shock accompanying a SARS-CoV-2 infection (COVID-19)

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Conclusions