Intern Med. 2013;52(10):1121-4.

Tolosa-hunt syndrome associated with cytomegalovirus infection.

Okawa S, Sugawara M, Takahashi S, Otani T, Hashimoto M, Kusunoki S, Ohnishi H.

Department of Neurology, Akita University Graduate School of Medicine, Japan. sato@doc.med.akita-u.ac.jp

 

ABSTRACT:

We herein present the case of a 38-year-old woman with left-sided oculomotor paralysis with ocular pain that developed after a respiratory infection. Her serum was positive for IgM against GM2 and GalNAc-GD1a gangliosides and cytomegalovirus. Thin-slice magnetic resonance imaging revealed enhanced abnormal tissue located primarily in the superolateral part of the left-sided cavernous sinus, which corticosteroids subsequently obscured with immediate resolution of the patient’s ocular symptoms. These clinical features were consistent with those of Tolosa-Hunt syndrome (THS). Our findings in the present patient suggest that cytomegalovirus may provoke granuloma formation in the cavernous sinus, as reported in other various organs, thereby leading to the development of THS.

PMID: 23676602

SUPPLEMENT:

This case raises the possibility that a subset of granulomas in patients with THS may be formed in relation to cytomegalovirus (CMV) infection and GM2 gangliosides. Granulomatous inflammation can be provoked by various infectious agents [1]. To date, CMV has also been identified in granulomas of the liver, bone marrow, endometrium, stomach and gingiva. The cytopathological effects of CMV infection are not completely known. CMV can infect macrophages, inducing cellular nodule and multinucleated giant cell formation [2] Furthermore, CMV can also infect lymphocytes and fibroblasts [3, 4], and CMV-infected fibroblasts have been shown to express the GM2 epitope [4]. Macrophages can express recognition sites for GM2 gangliosides that are considered to be important for cell-cell interactions [5]. Granulomas are focal accumulations of modified macrophages that can be surrounded by lymphocytes and fibroblasts [1, 6], and some gangliosides, including GM2, are detected in granulomas during their growth [6]. In our patient, the presence of strongly positive anti-GM2 antibodies implied the existence of highly expressed GM2 epitopes. In the patient’s cavernous sinus, CMV-infected modified macrophages might have recognized GM2 epitopes, which might have been induced in fibroblasts and lymphocytes by CMV infection, thereby attracting the fibroblasts and lymphocytes and leading to the rapid formation of the granuloma. Further studies are needed to clarify whether GM2 gangliosides are involved in the granuloma formation related to CMV infection.

Our findings in the present case suggest that THS may be symptomatic of some CMV infections in immunocompetent patients, although most cases are considered to be subclinical. Examinations of infectious agents in THS patients are expected to reinforce the notion that CMV infection can also be involved in intracavernous granuloma formation.

Reference:

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