Literature DB >> 21630043

Palliative functional hemispherectomy for treatment of refractory status epilepticus associated with Alpers' disease.

Svetlana Lupashko1, Saleem Malik, David Donahue, Angel Hernandez, M Scott Perry.   

Abstract

PURPOSE: Palliative epilepsy surgery is considered for patients that would benefit from surgical therapy for intractable epilepsy but are not candidates for curative procedures. In many cases, the goals of therapy focus on improved quality of life more than seizure freedom. We discuss the use of epilepsy surgery for refractory status epilepticus, as well as the rationale and ethical considerations for employing a palliative procedure in otherwise fatal diseases.
METHODS: We present a child with Alpers' disease presenting with refractory status epilepticus which was treated with functional hemispherectomy after failure of multiple typical therapies. Hemispherectomy allowed for the child to be extubated and ultimately discharged to home with her family. Unfortunately, the child died several months later after developing new-onset liver failure in the setting of a viral illness.
CONCLUSION: Functional hemispherectomy was effective for the treatment of refractory status epilepticus in Alpers' disease. We believe that the procedure resulted in improved quality of life which was the primary outcome goal. Palliative procedures should be considered in diseases with ultimate fatal outcome when the short-term benefits outweigh the risks. The ethical aspects of treatment must be carefully considered to insure treatment is provided in the best interest of the patient.

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Mesh:

Year:  2011        PMID: 21630043     DOI: 10.1007/s00381-011-1495-9

Source DB:  PubMed          Journal:  Childs Nerv Syst        ISSN: 0256-7040            Impact factor:   1.475


  8 in total

1.  Status epilepticus in children with Alpers' disease caused by POLG1 mutations: EEG and MRI features.

Authors:  Nicole I Wolf; Shamima Rahman; Bernhard Schmitt; Jan-Willem Taanman; Andrew J Duncan; Inga Harting; Gabriele Wohlrab; Friedrich Ebinger; Dietz Rating; Thomas Bast
Journal:  Epilepsia       Date:  2008-11-19       Impact factor: 5.864

2.  Alpers syndrome with prominent white matter changes.

Authors:  Xinhua Bao; Ye Wu; Lee-Jun C Wong; Yuehua Zhang; Hui Xiong; Ping-Chieh Chou; Cavatina K Truong; Yuwu Jiang; Jiong Qin; Yun Yuan; Qing Lin; Xiru Wu
Journal:  Brain Dev       Date:  2007-10-17       Impact factor: 1.961

3.  Neurosurgical treatment of refractory status epilepticus.

Authors:  D G Gorman; W D Shields; D A Shewmon; H T Chugani; R Finkel; Y G Comair; W J Peacock
Journal:  Epilepsia       Date:  1992 May-Jun       Impact factor: 5.864

Review 4.  Alpers syndrome: progressive neuronal degeneration of children with liver disease.

Authors:  Neil Gordon
Journal:  Dev Med Child Neurol       Date:  2006-12       Impact factor: 5.449

Review 5.  Resective surgery to treat refractory status epilepticus in children with focal epileptogenesis.

Authors:  A Alexopoulos; D K Lachhwani; A Gupta; P Kotagal; A M Harrison; W Bingaman; E Wyllie
Journal:  Neurology       Date:  2005-02-08       Impact factor: 9.910

6.  Life-saving epilepsy surgery for status epilepticus caused by cortical dysplasia.

Authors:  Pavel Krsek; Michal Tichý; Tomás Belsan; Josef Zámecník; Lukás Paulas; Ludvika Faladová; Premysl Jiruska; Alice Maulisová; Vladimír Komárek
Journal:  Epileptic Disord       Date:  2002-09       Impact factor: 1.819

7.  POLG1 mutations cause a syndromic epilepsy with occipital lobe predilection.

Authors:  Bernt A Engelsen; Charalampos Tzoulis; Bjørn Karlsen; Atle Lillebø; Liv M Laegreid; Jan Aasly; Massimo Zeviani; Laurence A Bindoff
Journal:  Brain       Date:  2008-01-30       Impact factor: 13.501

8.  Expanding the role of surgery in intractable extratemporal pediatric epilepsy.

Authors:  Monisha Goyal; Shenandoah Robinson
Journal:  Neurosurg Focus       Date:  2008-09       Impact factor: 4.047

  8 in total
  6 in total

Review 1.  Hemispherectomy in the treatment of seizures: a review.

Authors:  Sean M Lew
Journal:  Transl Pediatr       Date:  2014-07

2.  Fifty consecutive hemispherectomies: outcomes, evolution of technique, complications, and lessons learned.

Authors:  Sean M Lew; Jennifer I Koop; Wade M Mueller; Anne E Matthews; Julianne C Mallonee
Journal:  Neurosurgery       Date:  2014-02       Impact factor: 4.654

3.  Comment to the paper: Palliative functional hemispherectomy for treatment of refractory status epilepticus associated with Alper's disease.

Authors:  Brian S Carter
Journal:  Childs Nerv Syst       Date:  2011-06-04       Impact factor: 1.475

4.  Effective application of corpus callosotomy in pediatric intractable epilepsy patients with mitochondrial dysfunction.

Authors:  Ji-Hoon Na; Heung Dong Kim; Young-Mock Lee
Journal:  Ther Adv Neurol Disord       Date:  2022-04-21       Impact factor: 6.430

Review 5.  Management of Status Epilepticus in Children.

Authors:  Douglas M Smith; Emily L McGinnis; Diana J Walleigh; Nicholas S Abend
Journal:  J Clin Med       Date:  2016-04-13       Impact factor: 4.241

Review 6.  POLG-related disorders and their neurological manifestations.

Authors:  Shamima Rahman; William C Copeland
Journal:  Nat Rev Neurol       Date:  2019-01       Impact factor: 42.937

  6 in total

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