Review
Focal extratemporal epilepsy: clinical features, EEG patterns, and surgical approach

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Abstract

The objective of this review is a summary of the clinical and electrographic findings in those forms of epilepsy to which the term ‘extratemporal’ (ExT) can be applied. They form a group that differs in many ways from the better known temporal lobe epilepsies. Seizure foci are difficult to localize by clinical semiology alone but modern imaging now often allows a precise definition of the epileptogenic area. The most common causes of ExT epilepsy are tumors and cortical dysgenesis. The concept of ‘dual pathology’ implies the coexistence of two or more distinct lesions, typically mesial temporal sclerosis and cortical dysplasia. Electroencephalography (EEG) and electrocorticography (ECoG) are valuable tests in the definition of the epileptogenic area beyond the structural lesion, and surgical removal must be guided by the nature of the lesion and the extent of the epileptogenic zone.

Introduction

Electroencephalography (EEG), neuroimaging, and long-term monitoring have aided in the characterization of many types of focal epilepsy. ‘Typical’ temporal lobe seizures originating in the hippocampi the parahippocampal gyri, and the amygdaloid nuclei constitute the majority of focal epilepsies and gain the most benefit from surgery [36]. In contrast, extratemporal (ExT) epilepsy constitutes a more heterogeneous group. Different types of focal ExT epilepsies manifest themselves by a wide range of epileptic seizures and show highly variable responses to therapy.

Extratemporal epilepsy can be classified according to the presumed site of seizure origin. Frontal, parietal, and occipital focal epilepsies exist, and multiple territorial overlaps may occur [96], [98]. The ExT epilepsies are common disorders. According to the National General Practice Study of Epilepsy [75] at the Chalfont Centre for Epilepsy in England, 26.9% of all patients with definite epilepsy had seizures of identifiable focal origin. Within this group 22.5% had frontal, 32.5% central, 5.6% frontotemporal, and 6.3% each parietal and occipital foci, with only 27% of the patients having temporal lobe abnormalities. A larger more recent study [109] at the Epilepsy Unit of the La Salpêtrière Hospital in Paris included 1369 patients with partial epilepsy of whom 814 had identifiable lesions by magnetic resonance imaging (MRI). One hundred and seventy-nine (22%) had frontal, 20 (2.5%) occipital, and 15 (2%) parietal epilepsies. It is not always clear how the type of epilepsy affects the outcome of medical or surgical therapy. This retrospective review attempts a correlation of clinical findings, EEG and electrocorticography (ECoG), and surgical success rates, and the goal is to guide the neurologist toward the most rational management in their patients with ExT epilepsy.

Section snippets

Clinical features

The manifestations of ExT epilepsy are variable, and localization by clinical signs alone remains difficult. A few types of ExT seizures are stereotypical and characteristic of certain epileptogenic sites. Generally, ictal semiology is diagnostic when seizures arise from a primary cortical area, such as the primary motor, visual, or sensory cortices.

Interictal EEG abnormalities

Several types of interictal patterns may be seen in patients with ExT epilepsy. They include: (1) focal interictal epileptiform discharges (IEDs) localized to the site of seizure origin; (2) regional, poorly localized, but lateralized IEDs; (3) falsely localizing focal IEDs; (4) multifocal IEDs; (5) bilateral or generalized, bilaterally synchronous IEDs; and (6) no IEDs. Different degrees of focal or diffuse non-epileptiform electrographic changes may also be present.

Causes of extratemporal epilepsy

Jackson [55] recognized that structural brain pathology, such as a tumor, may cause epilepsy. With progress in neuroimaging, the proportion of ExT epilepsy cases without an identifiable focal brain lesion decreased significantly and now constitutes probably 20–40% of all ExT epilepsy cases [34], [83]. Table 2 presents the rate of occurrence of different radiological and histopathological findings in ExT epilepsies. Brain tumors and developmental cortical disorders are the two largest

Surgical outcome

Long-term outcome after surgery for ExT seizures depends on many factors. Generally, the benefit of surgical treatment for ExT epilepsy is less impressive than operations for medial temporal epilepsy. However, recent studies in pediatric epilepsy did not find a statistical difference between the outcomes after surgical treatment of temporal and ExT epilepsy [33], [134]. Seizure-free outcome rates were 10% for selected temporo-parietal and occipital cases [38], 26% in a large retrospective

Conclusion

Epilepsy is no longer a single clinical entity because it shows a perplexing abundance of clinical symptoms and signs and abnormal electrophysiological and radiological features. Extratemporal epilepsy stands out as a fairly well-circumscribed disorder. However, it may be further divided into subtypes based on the site of seizure origin and the presence of etiological factors. Clinical semiology of the seizures may be helpful in establishing the type of epilepsy but is not always reliable. A

Acknowledgements

The author wishes to acknowledge the assistance of Dr A. Koeppen, review editor, in the completion of the manuscript.

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