Subcortical Band Heterotopia Presented With Refractory Epilepsy and Reversible Aphasia

Reaz Mahmud

doi:10.7759/cureus.16990

Abstract

Subcortical band heterotopia (SBH) is a rare neurodevelopmental disorder due to mutation in the DCX or LIS1 gene. It is predominantly a disease of females. Its presentation varied widely, ranging from mild epilepsy and mental retardation to refractory epilepsy and severe mental retardation. Here, a case of a 22-year-old lady with refractory seizure is reported. She also had expressive aphasia which had reversed after adjustment of the anti-epileptic drugs and control of the seizure. Her MRI of the brain revealed a band of complete gray matter deep to the pachygyric cortex and an electroencephalogram (EEG) revealed bi-frontal slow waves.

Highlights

Subcortical heterotopia is a rare neurodevelopmental disorder of the human brain
The predominant cause of subcortical band heterotopia (SBH) is the mutations in the DCX or LIS1 gene [5]
Subcortical band heterotopia was defined by Dobyns et al [9] as a neurodevelopmental disorder that consists of “bilateral and symmetric ribbons of gray matter located in the centrum semiovale between the cortex and ventricular walls, which are separated from both by layers of white matter.”

Summary

Introduction

Subcortical heterotopia is a rare neurodevelopmental disorder of the human brain. There are three subtypes of subcortical heterotopia: (a) nodular, (b) laminar, and (c) subcortical band heterotopia (SBH) [1,2]. The presence of bilaterally symmetrical, heterotopic gray matter in between the ventricles and the cortex characterizes SBH [3] It is a classic example of deficient neuronal migration associated malformations [4]. She could speak normally prior to her onset of the seizure She had developed progressive speech regression since her 10 years of age and had become mute at her 12 years. Superficial and deep tendon reflexes were normal Her MRI of the brain (Figure 1) revealed a complete band of gray matter located deep and parallel to pachygyric overlying cortex. We did not make any dose adjustment and advised her strictly not to miss the dose of anticonvulsants further Her final drugs were sodium valproate 1500 mg, levetiracetam 1000 mg, and clobazam 10 mg per day

Discussion

Conclusions

Disclosures

Jacob H