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3 results

  • Chapter 1.3 - Chapter

  • from 1 - Inflammatory Conditions
  • Edited by Derya Uluduz, Istanbul Üniversitesi
  • Anita Arsovska
  • Book:
    Rare Causes of Stroke
    Published online:
    06 October 2022
    Print publication:
    01 September 2022, pp 72-78

  • Summary

    Behçet’s syndrome (BS), originally described in 1937 by the Turkish dermatologist Hulusi Behçet, as a distinct disease with oro-genital ulceration and uveitis known as the “triple-symptom complex”, is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin. The condition that features primary neurological involvement is termed as neuro-Behçet syndrome (NBS). Based on the clinical and neuroimaging features, primary BS neurological involvement may be divided into two major forms: 1) parenchymal NBS (p-NBS); and, 2) vascular NBS. Cranial neuropathy, dysarthria, ataxia, hemiparesis, and headache are the major symptoms of p-NBS, with headache being the most prevalent (in patients with both p-NBS and neuro-vascular involvement). Lesions revealed by magnetic resonance imaging are most common in the mesodiencephalic junction followed by the pons/medulla oblongata. Vascular involvement (the second most common form of neurological involvement) is associated with a better prognosis than p-NBS, and the clinical manifestations vary by the site and extension of venous thrombosis. During the acute phase of p-NBS, the cerebrospinal fluid shows inflammatory changes in most cases of p-NBS with an increased number of cells, up to a hundred and sometimes more per ml, neutrophils being mostly the predominating cells and modestly elevated protein levels. Patients with BS-CVST do not exhibit any remarkable cerebrospinal fluid finding apart from an increased pressure. Currently, the only drug that have been shown to be effective based on the Class IV evidence is infliximab for the treatment of p-NBS

  • Skull Base Calcifying Pseudoneoplasms of the Neuraxis: Two Case Reports and a Systematic Review of the Literature

  • Kaiyun Yang, Kesava Reddy, Yosef Ellenbogen, Bill Hao Wang, Michel W. Bojanowski, Jian-Qiang Lu
  • Journal:
    Canadian Journal of Neurological Sciences / Volume 47 / Issue 3 / May 2020
    Published online by Cambridge University Press:
    17 December 2019, pp. 389-397
    • Article
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  • Background:

    Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumefactive lesion. CAPNONs can mimic calcified meningiomas at the skull base.

    Methods:

    Here, we report two cases of CAPNON and present a systematic review of the literature on skull base CAPNONs, to compare CAPNONs with calcified meningiomas.

    Results:

    Case 1: A 57-year-old man presented with right-sided lower cranial neuropathies and gait ataxia. He underwent a subtotal resection of a right cerebellopontine angle lesion, with significant improvement of his gait ataxia. However, his cranial neuropathies persisted. Pathological examination of the lesion was diagnostic of CAPNON, with the entrapped nerve fibers identified at the periphery of the lesion, correlating with the patient’s cranial neuropathy. Case 2: A 70-year-old man presented with progressive headache, gait difficulty, and cognitive impairment. He underwent a frontotemporal craniotomy for a near-total resection of his right basal frontal CAPNON. He remained neurologically stable 7 years after the initial resection without evidence of disease recurrence. We analyzed 24 reported CAPNONs at the skull base in our systematic review of the literature. Cranial neuropathies were present in 11 (45.8%) patients. Outcomes regarding cranial neuropathies were documented in six patients: two had sacrifice of the nerve function with surgical approaches and four had persistent cranial neuropathies.

    Conclusion:

    While CAPNON can radiologically and grossly mimic calcified meningiomas, they are two distinctly different pathologies. CAPNONs located at the skull base are commonly associated with cranial neuropathies, which may be difficult to reverse despite surgical intervention.