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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

Electroencephalogram at 1-year of age showing right-sided unihemispheric abnormalities and subclinical seizure discharges
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Figure 3: Electroencephalogram at 1-year of age showing right-sided unihemispheric abnormalities and subclinical seizure discharges

Mentions: However, after 6 months postoperatively, spasms recurred with further regression of milestones. EEG showed unihemispheric abnormalities [Figure 3]. She was given a trial of vigabatrin, but seizure control was less than 50%. Frequency of partial seizures also increased to multiple times per day. In view of epileptic encephalopathy, it was decided to complete the functional hemispherotomy. A second surgery was done with complete callosotomy at 18 months of age. She withstood the procedure well. Postsurgery her spasms stopped but occasional partial seizures continued. Repeat neuroimaging (MRI brain) showed complete disconnection between the two hemispheres [Figure 4]. EEG revealed multiple subclinical seizures from right hemisphere. Post second surgery there was more than 75% seizure reduction; there were no spasms, but subclinical seizures still persisted along with left-sided hemiparesis.


Hemispherotomy in an infant with hemimegalencephaly.

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

Electroencephalogram at 1-year of age showing right-sided unihemispheric abnormalities and subclinical seizure discharges
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Electroencephalogram at 1-year of age showing right-sided unihemispheric abnormalities and subclinical seizure discharges
Mentions: However, after 6 months postoperatively, spasms recurred with further regression of milestones. EEG showed unihemispheric abnormalities [Figure 3]. She was given a trial of vigabatrin, but seizure control was less than 50%. Frequency of partial seizures also increased to multiple times per day. In view of epileptic encephalopathy, it was decided to complete the functional hemispherotomy. A second surgery was done with complete callosotomy at 18 months of age. She withstood the procedure well. Postsurgery her spasms stopped but occasional partial seizures continued. Repeat neuroimaging (MRI brain) showed complete disconnection between the two hemispheres [Figure 4]. EEG revealed multiple subclinical seizures from right hemisphere. Post second surgery there was more than 75% seizure reduction; there were no spasms, but subclinical seizures still persisted along with left-sided hemiparesis.

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