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Epilepsy and Seizures: Overview

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Epilepsy is a chronic neurological disease marked by recurrent, unpredictable seizures. These seizures are caused by abnormal electrical discharges in the brain, leading to behavior, sensation, or consciousness alterations. They can also cause transient impairment of awareness, interfering with daily activities.
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Hemimegalencephaly with intractable epilepsy: A case report.

Prem Chand¹, Muhammad Faraz Raghib², Muhammad Sohail Salat³

¹Pediatric Neurology, The Aga Khan University, Karachi, Pakistan.

JPMA. the Journal of the Pakistan Medical Association

|September 20, 2017

Summary

Isolated Hemimegalencephaly (iHME) is a rare brain malformation causing severe seizures in infants. This case highlights diagnostic challenges and intractable epilepsy despite treatment, emphasizing the need for further research.

Keywords:

Hemimegalencephaly, Hemiparesis, Intractable epilepsy, Hemispherectomy.

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Area of Science:

Neurology
Developmental Neuroscience
Pediatric Neurology

Background:

Isolated Hemimegalencephaly (iHME) is a rare congenital malformation of cortical development.
It involves the enlargement of one cerebral hemisphere, often leading to significant neurological deficits.

Observation:

A 5.5-month-old infant presented with focal seizures on day 10 of life.
Electroencephalogram (EEG) revealed excessive sharp transients and epileptiform discharges predominantly over the right hemisphere.
Magnetic Resonance Imaging (MRI) confirmed asymmetric enlargement of the right cerebral hemisphere, consistent with iHME.

Findings:

Initial anti-epileptic treatment provided temporary seizure control.
Seizures became intractable despite polytherapy, indicating a severe form of the condition.
The case underscores the diagnostic features and clinical course of iHME.

Implications:

Early identification and detailed case studies of iHME are crucial for understanding its pathophysiology.
Further research can lead to improved diagnostic strategies and more effective therapeutic interventions for iHME.
This case contributes to the knowledge base for managing intractable epilepsy associated with rare brain malformations.