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Intradural Extramedullary Lesions in the Cervical Spine in Neurofibromatosis

Rana Moshref, Abeer Mirdad

(Department of Neurosurgery, King Abdulaziz University Hospital, Jeddah, Saudi Arabia)

Am J Case Rep 2021; 22:e933090

DOI: 10.12659/AJCR.933090


BACKGROUND: Neurofibromatosis (NF) is categorized into 3 diseases: neurofibromatosis type 1, type 2, and schwannoma. NF2 is associated with a mutation in gene 22q11.2. It is present in about 1/25 000 to 33 000 births, and it is passed in an autosomal dominant fashion. Diagnosis is made based on clinical and radiological features. A few clinical features have been characterized and included in the Manchester criteria. A few neurofibromatosis type 2 patients have been diagnosed with over 25 cervical lesions. We report a case of an intradural extramedullary cervical lesion in a patient later diagnosed with neurofibromatosis type 2.
CASE REPORT: The patient was 30-year-old man admitted through the emergency unit, presenting with gradual onset and progressive spastic quadriparesis of 6 months duration. An MRI spine showed intradural extramedullary masses in the right side of C4 and left side of C6. He underwent cervical intradural excision of 2 masses under general anesthesia with neuromonitoring. The tumor was sent to histopathology and reported as neurofibromatosis 2.
CONCLUSIONS: Neurofibromatosis is a common entity, but the diagnosis of a cervical mass is judicious to avoid any misfortune in neurological function. It requires a multidisciplinary approach and screening modalities.

Keywords: Cervical Cord, Neurofibromatosis 2, Spinal Cord Neoplasms

This paper has been published under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0) allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially.
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