Viscous Leptomeningeal Pseudotumoural Masses and Multiple Cranial Neuropathy – Severe Presentation of Neurosarcoidosis
  • Rita Ribeiro Dias
    Serviço de Medicina interna do Centro Hospitalar e Universitário do Porto, Portugal
  • Inês Henriques Ferreira
    Serviço de Medicina interna do Centro Hospitalar e Universitário do Porto, Portugal
  • Raquel Faria
    Unidade de Imunologia Clínica, Centro Hospitalar do Porto, Porto, Portugal; Unit for Multidisciplinary Research in Biomedicine (UMIB) – ICBAS Universidade do Porto, Porto, Portugal

Keywords

Neurosarcoidosis, cranial neuropathy, leptomeningeal mass

Abstract

We present a case of a 56-year-old man with a history of episcleritis (left) and cluster headache (left) who had a penetrating trauma of the left eye leading to amaurosis 1 month previously. Since then, he developed multiple cranial neuropathy of the right side (V, VII, VIII, IX, X, XI and XII cranial pairs). Magnetic resonance imaging (MRI) revealed an infiltrative lesion of the base of the skull which extended to the retropharyngeal and jugular space, which progressed to multiple leptomeningeal masses extending to the clivus, despite aggressive immunosuppression. Rebiopsy of 1 meningeal mass supported the diagnosis of neurosarcoidosis. The patient finally responded to high-dose prolonged infliximab therapy, with complete remission.

VIEW THE ENTIRE ARTICLE

References

  • Nozaki K, Judson MA. Neurosarcoidosis: clinical manifestations, diagnosis and treatment. Presse Med 2012;41(6 Pt 2):e331–e348.

  • Allen RK, Sellars RE, Sandstrom PA. A prospective study of 32 patients with neurosarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2003;20(2):118–125.

  • Yacoub H, Al-Qudah Z, Souayah N. Cranial neuropathies in sarcoidosis. World J Ophthalmol 2015;5:16–22.

  • Wengert O, Rothenfusser-Korber E, Vollrath B, Bohner G, Scheibe F, Otto C, et al. Neurosarcoidosis: correlation of cerebrospinal fluid findings with diffuse leptomeningeal gadolinium enhancement on MRI and clinical disease activity. J Neurol Sci 2013;335:124–130.

  • Gelfand JM, Bradshaw MJ, Stern BJ, Clifford DB, Wang Y, Cho TA, et al. Infliximab for the treatment of CNS sarcoidosis: a multi-institutional series. Neurology 2017;89(20):2092–2100.

    • Views: 727
    HTML downloads: 65
    PDF downloads: 331

    Published: 2020-02-06
    Issue: 2020: Vol 7 No 3 (view)

    How to cite:
    1.
    Dias RR, Henriques Ferreira I, Faria R. Viscous Leptomeningeal Pseudotumoural Masses and Multiple Cranial Neuropathy – Severe Presentation of Neurosarcoidosis. EJCRIM 2020;7 doi:10.12890/2020_001453.