Abstract
Background
Hemimegalencephaly with tuberous sclerosis complex is an uncommon association, usually
associated with intractable seizures that begin in the neonatal period or early infancy.
Typically, the seizures are managed with medications until the patient is older when
surgical treatment is considered safe.
Patient Description
We describe a 7-week-old infant with tuberous sclerosis (TSC1 mutation) and hemimegalencephaly who underwent a functional hemispherectomy for status
epilepticus. No clinical seizures have occurred since surgery nearly 5 years ago and
subsequent weaning of antiepileptic drugs 3 years ago. This is one of the youngest
patients with tuberous sclerosis complex treated with a hemispherectomy and one of
seven patients described in the literature.
Conclusions
Our patient, along with previously reported cases, suggests that a hemispherectomy
is a viable option in the very young. With evolution of this surgical process since
its inception nearly 6 decades ago, it may now be performed safely in early infancy,
engendering the possibility of seizure freedom in most and thus optimizing neurodevelopmental
outcome.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Pediatric NeurologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Tuberous Sclerosis Complex.in: Pagon R.A. Adam M.P. Ardinger H.H. GeneReviews. University of Washington, Seattle (WA)1993-2015
- Cognitive and behavioral correlates of tuberous sclerosis complex.J Child Neurol. 2004; 19: 847-852
- Hemimegalencephaly and tuberous sclerosis complex.Neurology. 2005; 64: 1634
- Hemimegalencephaly in tuberous sclerosis complex.J Child Neurol. 2002; 17: 677-680
- Intractable epilepsy in hemimegalencephaly and tuberous sclerosis complex.J Child Neurol. 2007; 22: 80-84
- Unilateral megalencephaly and tuberous sclerosis: related disorders?.J Child Neurol. 1994; 9: 443-446
- Hemimegalencephaly and focal megalencephaly in tuberous sclerosis complex.AJNR Am J Neuroradiol. 1998; 19: 1935-1938
- Hemimegalencephaly with tuberous sclerosis: a longitudinal imaging study.Australas Radiol. 2003; 47: 438-442
- Seizure outcome after functional hemispherectomy for malformations of cortical development.Neurology. 2001; 57: 331-333
- Hemispherectomy for treatment of refractory epilepsy in the pediatric age group: a systematic review.J Neurosurg Pediatr. 2015; 15: 34-44
- Epilepsy surgery outcome in children with focal epilepsy due to tuberous sclerosis complex.Neuropediatrics. 2002; 33: 255-261
- Bilateral decreased oxygenation during focal status epilepticus in a neonate with hemimegalencephaly.J Child Neurol. 2004; 19: 394-396
- Epidemiology of hemimegalencephaly: a case series and review.Am J Med Genet A. 2005; 139: 204-211
- Longitudinal MR findings in a patient with hemimegalencephaly associated with tuberous sclerosis.Brain Dev. 2005; 27: 458-461
- Hemimegalencephaly–a case for hemispherectomy?.Neuropediatrics. 1985; 16: 46-55
- Hemimegalencephaly and intractable epilepsy: benefits of hemispherectomy.Epilepsia. 1989; 30: 833-843
- Neuro-cognitive development and epilepsy outcome in children with surgically treated hemimegalencephaly.Neuropediatrics. 1999; 30: 307-313
- Longitudinal seizure outcome and prognostic predictors after hemispherectomy in 170 children.Neurology. 2013; 80: 253-260
- Clinical outcomes of hemispherectomy for epilepsy in childhood and adolescence.Brain. 2003; 126: 556-566
- The role of hemispherectomy in the treatment of holohemispheric hemimegaloencephaly.J Neurosurg. 1994; 81: 37-42
- Why would you remove half a brain? The outcome of 58 children after hemispherectomy-the Johns Hopkins experience: 1968 to 1996.Pediatrics. 1997; 100: 163-171
- Posthemispherectomy hydrocephalus: results of a comprehensive, multiinstitutional review.Epilepsia. 2013; 54: 383-389
- Hemimegalencephaly and intractable epilepsy: complications of hemispherectomy and their correlations with the surgical technique. A report on 15 cases.Pediatr Neurosurg. 2000; 33: 198-207
- Hemispherectomy in a premature neonate with linear sebaceous nevus syndrome.Pediatr Neurosurg. 2008; 44: 159-164
- Seizure and developmental outcomes after hemispherectomy in children and adolescents with intractable epilepsy.Childs Nerv Syst. 2013; 29: 475-488
Article info
Publication history
Published online: June 29, 2015
Accepted:
June 21,
2015
Received:
April 3,
2015
Identification
Copyright
© 2015 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
