Neurofibromatosis II: A Disease of Multicentric Central Nervous System Masses
Arvinder Singh, Manjeet Kaur, Sumanjeet Kaur
Citation Information :
Singh A, Kaur M, Kaur S. Neurofibromatosis II: A Disease of Multicentric Central Nervous System Masses. Curr Trends Diagn Treat 2021; 5 (2):110-113.
Neurofibromatosis (NF) is an autosomal dominant disorder with two reported types. Neurofibromatosis type I presents with the cutaneous manifestations and involves astrocytes and the neurons. Neurofibromatosis II involves meninges and the Schwann cells in the central nervous system with presentation as bilateral schwannomas, multiple meningiomas, and cord ependymomas.
Our present case was presented with bilateral hearing loss and chronic headache. MRI of the brain showed bilateral vestibular schwannomas extending up to bilateral cerebellopontine angle cisterns, mass lesion in relation to the right trigeminal nerve, multiple extra-axial dura-based mass lesions, posterior fossa mixed signal lesion, optic nerve sheath tumors, and two intradural intramedullary cervical cord ependymomas with surrounding cord edema.
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