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CASE REPORT
Year : 2013  |  Volume : 19  |  Issue : 1  |  Page : 36-38

MISME syndrome: A rare clinical entity


Department of Otorhinolaryngology, Head and Neck Surgery, SMHS Hospital, Srinagar, Jammu and Kashmir, India

Correspondence Address:
Mirza Aneesa
Department of Otorhinolaryngology, Head and Neck Surgery, SMHS Hospital, GMC Srinagar, Jammu and Kashmir
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-7749.108169

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We report a case of a 16 year-old male patient diagnosed as MISME syndrome (MISME: Multiple inherited schwannomas, meningiomas, and ependymomas) which is a rare clinical entity. The disease is autosomally hereditary, without gender predilection and having a clinical penetrance rate of nearly 90%.The gene defect is in chromosome 22 ( NF II gene) and is distinguished from NF I where the gene defect is in chromosome 17.


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