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Transcallosal endoscopic resection of hypothalamic hamartoma in a case with Pallister-Hall syndrome.
MedLine Citation:
PMID:  21636348     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Pallister-Hall syndrome (PHS) is a very rare syndrome characterized by hypothalamic hamartoma (HH), polydactyly, panhypopituitarism, imperforate anus and other visceral anomalies. Contrary to patients with isolated HH, neurological dysfunction and precocious puberty are uncommon and seizures are usually well controlled with anticonvulsant medication. Therefore, conservative management of HH is advised. To the best of our knowledge, seven cases of PHS with surgical resection of the HH have so far been reported. Five patients were either seizure-free or had >90% seizure reduction postoperatively. Here, we present a case of PHS of a patient who also underwent transcallosal endoscopic resection of the HH with a subsequent 70% reduction in seizure frequency.
Authors:
Nese Dericioglu; Serap Saygi; Nejat Akalan
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-6-3
Journal Detail:
Title:  Epileptic disorders : international epilepsy journal with videotape     Volume:  -     ISSN:  1294-9361     ISO Abbreviation:  -     Publication Date:  2011 Jun 
Date Detail:
Created Date:  2011-6-3     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100891853     Medline TA:  Epileptic Disord     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
University Institute of Neurological Sciences and PsychiatryUniversity Institute of Neurological Sciences and Psychiatry.
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