Orbital apex syndrome associated with diffuse hypertrophic pachymeningitis in isolated neurosarcoidosis

A 43-year-old woman reported acute onset of right retro-orbital headache followed by ptosis, paralysis of extraocular muscles, and subsequently amaurosis, characterizing an orbital apex syndrome[1]. Brain MRI showed diffuse pachymeningitis with leptomeningeal and parenchymatous involvement ([Figure 1]). CSF analysis was unremarkable. ACE and IgG4 levels were normal. However, investigation was done under oral steroid use. Lymphoproliferative and exocrine gland disorders were excluded. Lung scintigraphy and CT were normal. Meningeal biopsy disclosed diffuse non-caseous granulomatosis. Intravenous steroid pulse improved ocular movements ([Figure 2]), but amaurosis persisted.

Figure 1 Brain MRI at disease onset (A); two months after steroid pulse therapy (B); and one year after treatment (C). At disease onset (A), there is a cortical and subcortical FLAIR hyperintensities (arrows in A.I) in the anterior right temporal pole and left temporal lobe. Those areas show T1 contrast enhancement (A.II) and highlight the pachymeningitis involving right temporal pole and parasellar region, including the orbital apex (A and B, arrow heads). Sagittal T1 with contrast (A.III and A.IV) show pachymeningitis in the left cerebellar tentorium (arrows), with subtle leptomeningeal involvement, characterized by sulci enhancement shown in the axial plane (arrows in A.II). Two months after pulse therapy (B) there is a reduction in the FLAIR hyperintensity and cortical/sulci enhancement in the left temporal lobe and a decrease of pachymeningeal thickening and enhancement. One year after pulse therapy (C) there is an almost complete resolution of meningeal abnormalities with a residual focal FLAIR hyperintensity in the right temporal pole, in the biopsy site.

Figure 2 Evaluation of ocular motricity of the right eye (RE): A. Around 3 weeks after pulse intravenous methylprednisolone therapy. B. Two months after pulse therapy, a mild improvement, especially in abduction of RE. C. One year after pulse therapy, expressive improvement of horizontal motricity and remaining palsy of vertical gaze, particularly downward.

Neurologic manifestations represent less than 10% of sarcoidosis cases[2]. Isolated neurosarcoidosis is even rarer[3].

Conflict of interest:

There is no conflict of interest to declare.

Authors’ contribution:

NTB: Conceptualization, data curation, formal analysis, investigation, resources, writing-original draft; BCRG: data curation, formal analysis, resources; MC: visualization, writing-review and editing; MS: visualization, writing-review and editing; ACAFF: Conceptualization, formal analysis, project administration, resources, writing-original draft, writing-review and editing

• References

• 1 Zarei M, Anderson JR, Higgins JN, Manford MR. Cavernous sinus syndrome as the only manifestation of sarcoidosis. J Postgrad Med. 2002;48(2):119-21.
• 2 Chang CS, Chen WL, Li C Te, Wang PY. Cavernous sinus syndrome due to sarcoidosis: A case report. Acta Neurol Taiwan. 2009 Mar;18(1):37-41.
• 3 Kidd DP. Sarcoidosis of the central nervous system: clinical features, imaging, and CSF results. J Neurol. 2018 Aug;265(8):1906-15. https://doi.org/10.1007/s00415-018-8928-2

Publication History

Received: 02 September 2020

Accepted: 02 November 2020

Article published online:
01 June 2023