Orbital apex syndrome associated with diffuse hypertrophic pachymeningitis in isolated neurosarcoidosis

Síndrome da fissura orbitária superior associada a paquimeningite hipertrófica difusa na neurosarcoidose isolada

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 syndrome1. 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 cases2. Isolated neurosarcoidosis is even rarer3.

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
  » https://doi.org/10.1007/s00415-018-8928-2

Publication Dates

• Publication in this collection
  11 June 2021
• Date of issue
  Aug 2021

History

• Received
  02 Sept 2020
• Reviewed
  19 Oct 2020
• Accepted
  02 Nov 2020