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Hemispherotomy in an infant with hemimegalencephaly.

Kulkarni SD, Deopujari CE, Patil VA, Sayed RJ - J Pediatr Neurosci (2015 Apr-Jun)

Bottom Line: Hemimegalencephaly (HME) is a rare hamartomatous congenital malformation of the brain.Hemispheric disconnection has been reported to be efficient in seizure control and prevents further cognitive injury and developmental delay.She had more than 90% reduction of seizures with good developmental outcome on follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Neurosciences, Bai Jerbai Wadia Hospital, Parel, Mumbai, Maharashtra, India.

ABSTRACT
Hemimegalencephaly (HME) is a rare hamartomatous congenital malformation of the brain. The epilepsy pattern in HME can be partial seizures or may present as spasms as in epileptic encephalopathy. Epilepsy associated with HME is usually resistant to antiepileptic drugs and requires surgical intervention. Hemispheric disconnection has been reported to be efficient in seizure control and prevents further cognitive injury and developmental delay. We report a case of HME, who underwent a two-stage hemispherotomy due to complications in the first surgery. She had more than 90% reduction of seizures with good developmental outcome on follow-up. Thus, despite risks of the procedure, early surgery should be preferred in infants with HME.

No MeSH data available.


Related in: MedlinePlus

(a) Magnetic resonance imaging (MRI) brain (T1-weighted image) at 2 months of age showing widespread cortical dysplasia in the right hemisphere. (b and c) MRI brain (T2-weighted and T1-weighted images) at 9 months of age showing right sided hemimegalencephaly. (d) Initial electroencephalogram showing gross asymmetry between two hemispheres with occasional epileptiform discharges from right frontotemporal region with right-sided theta delta bursts
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Figure 1: (a) Magnetic resonance imaging (MRI) brain (T1-weighted image) at 2 months of age showing widespread cortical dysplasia in the right hemisphere. (b and c) MRI brain (T2-weighted and T1-weighted images) at 9 months of age showing right sided hemimegalencephaly. (d) Initial electroencephalogram showing gross asymmetry between two hemispheres with occasional epileptiform discharges from right frontotemporal region with right-sided theta delta bursts

Mentions: Her first magnetic resonance imaging (MRI) (at 2 months of age) showed widespread cortical dysplasia in the right hemisphere; however myelination was incomplete [Figure 1a]. Follow-up scans were advised which revealed right-sided HME [Figure 1b and c]. Initial electroencephalogram (EEG) depicted gross asymmetry between the two hemispheres with occasional epileptiform discharges from right frontotemporal region with right-sided theta delta bursts [Figure 1d]


Hemispherotomy in an infant with hemimegalencephaly.

Kulkarni SD, Deopujari CE, Patil VA, Sayed RJ - J Pediatr Neurosci (2015 Apr-Jun)

(a) Magnetic resonance imaging (MRI) brain (T1-weighted image) at 2 months of age showing widespread cortical dysplasia in the right hemisphere. (b and c) MRI brain (T2-weighted and T1-weighted images) at 9 months of age showing right sided hemimegalencephaly. (d) Initial electroencephalogram showing gross asymmetry between two hemispheres with occasional epileptiform discharges from right frontotemporal region with right-sided theta delta bursts
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4489073&req=5

Figure 1: (a) Magnetic resonance imaging (MRI) brain (T1-weighted image) at 2 months of age showing widespread cortical dysplasia in the right hemisphere. (b and c) MRI brain (T2-weighted and T1-weighted images) at 9 months of age showing right sided hemimegalencephaly. (d) Initial electroencephalogram showing gross asymmetry between two hemispheres with occasional epileptiform discharges from right frontotemporal region with right-sided theta delta bursts
Mentions: Her first magnetic resonance imaging (MRI) (at 2 months of age) showed widespread cortical dysplasia in the right hemisphere; however myelination was incomplete [Figure 1a]. Follow-up scans were advised which revealed right-sided HME [Figure 1b and c]. Initial electroencephalogram (EEG) depicted gross asymmetry between the two hemispheres with occasional epileptiform discharges from right frontotemporal region with right-sided theta delta bursts [Figure 1d]

Bottom Line: Hemimegalencephaly (HME) is a rare hamartomatous congenital malformation of the brain.Hemispheric disconnection has been reported to be efficient in seizure control and prevents further cognitive injury and developmental delay.She had more than 90% reduction of seizures with good developmental outcome on follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Neurosciences, Bai Jerbai Wadia Hospital, Parel, Mumbai, Maharashtra, India.

ABSTRACT
Hemimegalencephaly (HME) is a rare hamartomatous congenital malformation of the brain. The epilepsy pattern in HME can be partial seizures or may present as spasms as in epileptic encephalopathy. Epilepsy associated with HME is usually resistant to antiepileptic drugs and requires surgical intervention. Hemispheric disconnection has been reported to be efficient in seizure control and prevents further cognitive injury and developmental delay. We report a case of HME, who underwent a two-stage hemispherotomy due to complications in the first surgery. She had more than 90% reduction of seizures with good developmental outcome on follow-up. Thus, despite risks of the procedure, early surgery should be preferred in infants with HME.

No MeSH data available.


Related in: MedlinePlus