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Seizure Semiology and Presurgical Evaluation on October 1, 2021 by Guest 260 PRACTICAL NEUROLOGY Pract Neurol: first published as 10.1111/j.1474-7766.2004.00244.x on 1 October 2004. Downloaded from REVIEW Lateral Medial Precentral Precentral Lateral Premotor Medial Premotor Central Prefrontal Prefrontal Medial-dorsal Lateral-dorsal Ventral Lateral-ventral Medial-ventral Classification Medio-ventral Frontal lobe epilepsy: http://pn.bmj.com/ seizure semiology and presurgical evaluation on October 1, 2021 by guest. Protected copyright. Dr Aileen McGonigal† and Professor Patrick Chauvel* *Director of Neurophysiology and Neuropsychology and †Clinical Research Fellow in Epileptology, Service de Neurophysiologie Clinique, Hôpital de la Timone and Laboratoire de Neurophysiologie et Neuropsychologie, INSERM EMI 9926, Faculté de Médecine, Marseille, France; Email: [email protected] Practical Neurology, 2004, 4, 260–273 © 2004 Blackwell Publishing Ltd OCTOBER 2004 261 Pract Neurol: first published as 10.1111/j.1474-7766.2004.00244.x on 1 October 2004. Downloaded from INTRODUCTION no matter how sophisticated. Just as the pres- John Hughlings Jackson refl ected that the fron- ence of a right hemiparesis alerts the clinician to tal lobe is the brain’s ‘most complex and least look for a lesion in the contralateral motor path- organized centre’ (Jackson 1931) and, despite way, so too can the clinical features of a seizure subsequent advances in neuroscience, even by point to the activation (or inhibition) of certain the late 20th century the frontal lobe was still brain regions. At its simplest level, this allows us considered to be an ‘uncharted province of the to relate a sign such as a focal clonic contraction brain’ (Goldman-Rakic 1984). For epileptolo- in the hand with seizure activity in the contralat- gists today, frontal lobe epilepsy (FLE) remains eral motor cortex. However, in FLE the observed the most challenging of all the epilepsies, both symptoms or signs may be complex, subtle and in terms of understanding how seizures are or- often occur simultaneously or in rapid succes- ganized and how they should be treated. This sion, frequently refl ecting the activation of dif- is very evident in comparison to the now well- ferent structures within a dynamic system, with defi ned syndrome of mesial temporal lobe epi- rapid and unpredictable propagation patterns. lepsy (TLE). Not only is it challenging to determine from Important advances have been made in re- which part of the frontal lobe the seizure arises, cent decades, especially in correlations between but also it is often diffi cult to assess whether a the clinical and electrical expression of seizures, particular attack is indeed a frontal lobe sei- permitting better understanding of FLE. To- zure at all. Frontal lobe seizures are particularly gether with major developments in the fi eld of prone to misdiagnosis as psychogenic non-epi- neuroimaging, these advances are changing the leptic seizures, due to their sometimes bizarre approach to management, particularly in mak- or atypical appearance, as well as to the fact that ing curative surgery a real possibility for many surface EEG does not necessarily show interictal more patients than ever before. or ictal abnormalities (Bautista et al. 1998). Understanding FLE, it can be argued, will Another possible misdiagnosis of FLE is of a also help us understand more about the cer- sleep disorder, particularly as a large proportion ebral processes that underlie normal higher of frontal seizures arise from sleep. For example, brain functions such as the interaction between the nocturnal attack disorder originally identi- emotion and decision making (Damasio 1995). fi ed as a form of movement disorder – ‘paroxys- Indeed, frontal lobe epilepsy has been described mal nocturnal dystonia’ (Lugarasi & Cirignotta as ‘the next frontier’ (Niedermeyer 1998). From 1981) – was subsequently recognized to have an historical observations to futuristic develop- epileptic basis in most cases (Meierkord et al. ments: what does all this mean for our routine 1992). The syndrome of autosomal dominant http://pn.bmj.com/ clinical practice? nocturnal frontal lobe epilepsy (ADNFLE) was later described; this is a monogenic disorder AIMS OF THIS REVIEW with high penetrance, characterized by brief We have chosen to focus on the approach to hyperkinetic nocturnal seizures. the electroclinical diagnosis and localization Because of these diagnostic diffi culties, cau- of FLE, in other words the combined analysis tion must be exercised, and an epilepsy specialist on October 1, 2021 by guest. Protected copyright. of the clinical features of seizures (semiology) rather than a general neurologist or general phy- and electroencephalographic (EEG) data. This sician should ideally make the diagnosis. approach is particularly important when assess- The localizing value of specifi c semiological ing those patients who may be candidates for features is, in general, less well-understood in epilepsy surgery: the 20% or so of all patients FLE, compared with TLE. For this reason, as well with partial epilepsy who are pharmacoresist- as other issues related to the limitations of EEG ant. In addition we will briefl y discuss recent in FLE, diagnosis and localization are well rec- developments in other aspects of presurgical ognized to be more diffi cult than in other locali- evaluation. zation-related epilepsies (Manford et al. 1996). Indeed, it is likely that some epilepsy ‘surgical WHY ARE ELECTROCLINICAL failures’, including cases operated for presumed CORRELATIONS SO IMPORTANT IN TLE that do not become seizure-free post-op- FRONTAL LOBE EPILEPSY? eratively, refl ect incorrect presurgical localiza- In epilepsy, as in all neurological practice, the tion, rather than suboptimal resection (Walsh & history and physical signs are of paramount im- Delgado-Escueta 1984). FLE forms the second portance and cannot be replaced by a single test, largest group of potentially operable localiza- © 2004 Blackwell Publishing Ltd 262 PRACTICAL NEUROLOGY Pract Neurol: first published as 10.1111/j.1474-7766.2004.00244.x on 1 October 2004. Downloaded from tion-related epilepsies after TLE. The reliable area) within the premotor cortex, particularly electroclinical diagnosis of FLE, as well as the in the dominant hemisphere (area 44). identifi cation of subtypes, is therefore particu- • The frontal eye fi elds, which can contribute larly important in terms of optimal selection of to ictal versive head and eye movement, lie candidates for epilepsy surgery. within the dorsolateral cortex (area 8) in the Despite the complex nature of most fron- boundary where the premotor and precentral tal seizures, certain semiological patterns are cortex meet, and may therefore be involved in reproducible and can help to defi ne the likely seizures arising from either of these regions. region(s) involved. The occurrence of localized The organization of the prefrontal cortex, tonic posturing (face, upper limb, lower limb), which is predominantly made up of heteromo- emotional behaviour (such as fear) and com- dal association areas, is extremely complicated plex motor activity, may direct the clinician to- and incompletely understood. It has complicat- wards a particular part of the frontal lobe, as will ed and long association connections with other be discussed later. This becomes crucial when a brain regions, including limbic and paralimbic more precise sublobar understanding of locali- areas, which involve a continuum of temporal zation is required. and frontal lobe structures (particularly the cin- gulate gyrus and the posterior orbital region) FRONTAL LOBE ANATOMY AND RELATION (Fig. 2). Incoming sensory information from TO SEMIOLOGY these areas may be processed, taking account of The frontal lobe is the largest lobe in the brain motivational and emotional states, and used to (accounting for about 40% of cerebral cortex) infl uence decision-making and many aspects of (Fig. 1). This large size contributes to diagnostic behaviour (Pandya & Yeterian 1985). Patients diffi culties. There are multiple diverse propaga- with prefrontal epilepsy may demonstrate in- tion patterns, and there is the problem of lim- terictal behavioural or psychiatric abnormalities, ited EEG sampling, particularly from relatively such as lack of spontaneity and poor planning ‘hidden’ regions such as medial and basal (or- (frontal abulic syndrome), or impulsivity and bitofrontal) cortex (Bautista et al. 1998). The socially inappropriate behaviour (frontal dis- functional anatomical divisions of precentral, inhibition syndrome), which may improve fol- premotor and prefrontal cortex provide a use- lowing surgery (Devinsky et al. 1995). ful model for thinking about semiology and will be briefl y described: ATTEMPTS TO CLASSIFY FRONTAL LOBE • The precentral region consists of primary EPILEPSY motor cortex, Brodmann’s area 4. Although the approach of separating tempo- http://pn.bmj.com/ • The premotor cortex consists principally of ral from extra-temporal epilepsy is now estab- the lateral and medial components of area 6, lished, and most extra-temporal epilepsies have the latter corresponding to the supplemen- their origin in the frontal lobe, a widely accepted tary motor area (SMA). classifi cation of FLE has not yet been reached. • There is some representation of language (in- Indeed the nomenclature used by different cluding the region formally known as Broca’s groups to describe frontal seizure types has var- on October 1, 2021 by guest. Protected copyright. D E Figure 1 Cytoarchitectonic 8B 4 8Ad diagram of the frontal lobe
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