Treatment of Epilepsy Associated with Periventricular Nodular Heterotopia
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(2020) 20:59
EPILEPSY (C.W. BAZIL, SECTION EDITOR)
Treatment of Epilepsy Associated with Periventricular Nodular Heterotopia Hui Ming Khoo 1
&
Jean Gotman 2 & Jeffery A. Hall 2 & François Dubeau 2
Accepted: 19 October 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review Epilepsy associated with periventricular nodular heterotopia (PNH), a developmental malformation, is frequently drug-resistant and requires focal therapeutic intervention. Invasive EEG study is usually necessary to delineate the epileptogenic zone, but constructing an accurate hypothesis to define an appropriate electrode implantation scheme and the treatment is challenging. This article reviews recent studies that help understanding the epileptogenicity and potential therapeutic options in PNH. Recent Findings New noninvasive diagnostic and intracerebral EEG analytic tools demonstrated that cortical hyperexcitability and aberrant connectivity (between nodules and cortices and among nodules) are likely mechanisms causing epilepsy in most patients. The deeply seated PNH, if epileptogenic, are ideal target for stereotactic ablative techniques, which offer concomitant ablation of multiple regions with relatively satisfactory seizure outcome. Summary Advance in diagnostic and analytic tools have enhanced our understanding of the complex epileptogenicity in PNH. Development in stereotactic ablative techniques now offers promising therapeutic options for these patients. Keywords Periventricular nodular heterotopia . Epilepsy . Treatment . SEEG . Radiofrequency thermocoagulation . Laser ablation
Introduction Heterotopia (heterotopion for singular and heterotopia for plural) literally means normal-looking neurons displaced in an abnormal location; they are classified as malformations due to abnormal neuron migration. There are many forms of heterotopia, with the neurons deposited anywhere between the ventricle and the pia, and are usually classified into subcortical, which can be laminar (band heterotopia) or nodular, or into subependymal or periventricular, which are usually nodular [1, 2]. Periventricular heterotopia is the most common form
among all heterotopia. Because of the difference in embryogenesis with other heterotopia, periventricular heterotopia is currently subcategorized as malformations with neuroependymal abnormalities [3]. Periventricular heterotopia can be further categorized into nodular and ribbon-like forms based on appearance [3]. Because patients with periventricular nodular heterotopia (PNH)-related epilepsy are most often resistant to antiepileptic drugs and require focal therapeutic interventions, this review will focus on PNH. The diagnostic and analytic tools as well as recent therapeutic options will be discussed.
This article is part of the Topical Collection on Epilepsy * Hui Ming Khoo [email protected] Jean Gotman [email protected] Jeffery A. Hall [email protected] François Dubeau [email protected]
1
Department of Neurosurgery, Osaka Universi
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