Paper Information

Journal:   IRANIAN JOURNAL OF CHILD NEUROLOGY (IJCN)   SUMMER 2016 , Volume 10 , Number 3; Page(s) 86 To 90.
 
Paper: 

HEMISPHERECTOMY PROCEDURE IN PROTEUS SYNDROME (CASE REPORT)

 
 
Author(s):  GUNAWAN PRASTIYA INDRA*, LUSIANA LUSIANA, SAHARSO DARTO
 
* AIRLANGGA UNIVERSITY COLLEGE OF MEDICINE, JL. PROF DR MOESTOPO 6-8 SURABAYA INDONESIA
 
Abstract: 

Objective: Proteus syndrome is a rare overgrowth disorder including bone, soft tissue, and skin. Central nervous system manifestations were reported in about 40% of the patients including hemimegalencephaly and the resultant hemicranial hyperplasia, convulsions and mental deficiency. We report a 1-month-old male baby referred to Pediatric Neurology Clinic Soetomo Hospital, Surabaya, Indonesia in 2014 presented recurrent seizures since birth with asymmetric dysmorphic face with the right side larger than the left, subcutaneous mass and linear nevi. Craniocervical MRI revealed hemimegalencephaly right cerebral hemisphere. Triple antiepileptic drugs were already given as well as the ketogenic diet, but the seizures persisted. The seizure then was resolved after hemispherectomy procedure.

 
Keyword(s): INTRACTABLE EPILEPSY, LINEAR NEVI, HEMIMEGALENCEPHALY, HEMISPHERECTOMY, PROTEUS SYNDROME
 
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