Rare variant of misme syndrome A case report and review of literature

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Ashish Kumar Dwivedi
Shashi Kant Jain
Ashok Gandhi

Abstract

MISME syndrome, also known as neurofibromatosis type-2 (NF2), stands for multiple inherited schwannomas, meningiomas, and ependymomas (MISME) in the peripheral and central nervous system. It is a rare disorder of autosomal dominant inheritance due to mutations of a tumor-suppressor gene on the chromosome 22q12. Clinically, it is characterized by multiple benign tumors arising in both the central and the peripheral nervous system, particularly from the bilateral vestibular nerve in more than 90% of the patients and more than two thirds of them develop spinal tumors. Simultaneous occurrence of bilateral vestibular schwanoma with cervical and lumbar ependymoma without neuro cutaneous marker with weakness of limb as initial presentation is rare finding in single patient. Here, we are reporting a rare case of MISME syndrome harbouring bilateral vestibular schwanoma with cervical and lumbar ependymoma tumors in a 45 year old male patient having no other lesion and neurocutaneous marker with weakness of limb as initial presentation without posterior subcapsular cataract.

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Dwivedi, A. K., Jain, S. K., & Gandhi, A. (2016). Rare variant of misme syndrome: A case report and review of literature. Romanian Neurosurgery, 30(3), 426–431. Retrieved from https://www.journals.lapub.co.uk/index.php/roneurosurgery/article/view/939
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Author Biographies

Ashish Kumar Dwivedi

Department of Neurosurgery S.M.S. Medical College, Jaipur, INDIA

Shashi Kant Jain

Department of Neurosurgery S.M.S. Medical College, Jaipur, INDIA

Ashok Gandhi

Department of Neurosurgery S.M.S. Medical College, Jaipur, INDIA