bluuebunny/crossref_metadata_2025 huggingface dataset:
Exceptionally long absence status: multifactorial etiology, drug interactions and complications
Authors: D'Agostino Maria Daniela , Andermann Frederick , Dubeau Francois , Fedi Marco , Bastos Alexandre
Published: March 2024

To our knowledge, petit mal status lasting longer than 2‐3 days has been documented only once [1]. We report a 66 year‐old man with well‐documented, idiopathic generalized epilepsy who developed petit mal status lasting 6 weeks. Valproate levels remained low because of interactions with phenytoin. When phenytoin was discontinued, valproate levels increased, and he progressively improved. Chronic ischemic changes in the white matter may have been an additional factor in the causation and, most likely, in the duration of his status.

Exceptionally long status and stupor increase the risk of medical complications. Valproate remains the medication of choice for the treatment of petit mal status. Despite the extraordinary course in this patient, complete recovery took place, confirming the benign nature of even such a prolonged episode.
Mesial temporal lobe epilepsy
versus
amygdalar epilepsy: Late seizure recurrence after initially successful amygdalotomy and regained seizure control following hippocampectomy
Authors: Wieser Heinz Gregor
Published: March 2024

We summarise the concept of mesial temporal lobe epilepsy and the
pros and cons
in order to define amygdala epilepsy. We present a patient with stereotactically proven right amygdalar seizure onset, associated with fear and vegetative autonomic signs and symptoms as the most prominent clinical ictal features. Following a right stereotactic amygdalotomy, the patient experienced an 11‐year seizure‐free period. Similar, but not identical, semeiology of complex partial seizures then recurred. A right‐sided selective hippocampectomy and excision of the previously lesioned amygdala was performed. Except for 2 complex partial seizures associated with withdrawal of antiepileptic drugs, the patient remained seizure‐free 9.5 years.

This case underscores the important role of the amygdala in generating the semiology, and raises several questions concerning the existence of “amygdalar epilepsy”. The 11‐year seizure‐free period following the stereotactic destruction of the amygdala is a strong argument for this notion. The late seizure recurrence requiring a second operation might, however, be seen as an argument for the important role of the hippocampal formation in the syndrome of mesial temporal lobe epilepsy even when the amygdala has been identified as the seizure onset zone. The role of stereotactic amygdalotomy is briefly reviewed.
Multifactorial pathogenesis of neonatal seizures – relationships to the benign partial epilepsies
Authors: Doose Hermann , Koudriavtseva Katharina , Neubauer Bernd A.
Published: March 2024

Elevated incidence of neonatal seizures (NS) in children with idiopathic partial epilepsies, as well as reports on the transition of NS into benign partial epilepsy, point to pathogenetic relationships between these conditions. Further insight into the nature of these relationships can be expected only from NS patients with long‐term follow‐up, which includes the age range of maximum manifestation of subsequent seizures or epilepsies (SSE), and age‐dependent genetic EEG traits. A sample of such cases will necessarily be selected and thus prohibit any quantitative inferences regarding the incidence of SSE and special EEG characteristics. Nevertheless, the data provide new aspects with regard to a possibly multifactorial pathogenesis of NS and SSE. Children in the present study were selected applying the following inclusion criteria: cerebral seizures during the first 14 days of life; follow up of more than two years with at least two EEG recordings beyond the age of two. Children with metabolic NS or subsequent West syndrome were not included. Seventy‐six cases were confirmed, 42 with SSE, 34 without. The incidence of EEG symptoms of a generalized genetic seizure liability (theta rhythms, generalized spikes and waves, photoparoxysmal response) was significantly elevated, equally in children with and without SSE. Beyond the age of two years, 50% of the probands had focal sharp waves foci characteristic of idiopathic partial epilepsy (e.g. rolandic epilepsy). Among SSE, febrile convulsions and partial epilepsies with benign course dominated. Idiopathic generalized epilepsies were not observed. These findings indicate that in a certain proportion of cases, NS have genetic factors in common with idiopathic partial epilepsies. Quantitative representative data cannot be obtained to determine the incidence of such pathogenetic mechanisms in NS and SSE.
Antimyoclonic effect of levetiracetam
Authors: Genton Pierre , Gélisse Philippe
Published: March 2024

Treatment of severe, incapacitating action myoclonus is difficult. Piracetam has been shown to be a very potent antimyoclonic agent, but only at very high, impractical doses, ranging from 24 to 40 g/d. Levetiracetam (LEV), a new antiepileptic drug, is a structurally related compound that has a distinct pharmacological profile and appears to be efficient at much lower doses. We gave LEV, 4,000 mg/d, without titration, to three volunteers with post‐anoxic myoclonus (PAM) (one case) and Unverricht‐Lundborg disease (two cases), over 2, 2 and 10 weeks, respectively. LEV produced a clear abatement of myoclonus, which is demonstrated on video for the patient with post‐anoxic myoclonus, without any unwanted side‐effects. These preliminary findings suggest that LEV may have interesting antimyoclonic properties that deserve further investigation.
Selection criteria and preoperative investigation of patients with focal epilepsy who lack a localized structural lesion
Authors: Duchowny Michael , Jayakar Prasanna , Koh Susan
Published: March 2024

Children with medically resistant epilepsy are often referred for surgical evaluation. The absence of a specific focal lesion on MRI may render the work‐up difficult. In response to the need to localize the primary area of epileptogenesis, surgery protocols are being developed which rely on clinical semiology, EEG and functional imaging data. In selected cases, intracranial EEG monitoring may be required. While testing more often depends on the convergence of modalities, it is possible to localize seizure origin in the majority of children, and fully excise the epileptogenic region. This review presents the etiology and preoperative modalities available for children with intractable, non‐lesional epilepsy.
Autonomic symptoms during epileptic seizures
Authors: Baumgartner Christoph , Lurger Stefanie , Leutmezer Fritz
Published: March 2024

Autonomic symptoms frequently occur during epileptic seizures either as an accompaniment to other seizure symptoms or as the predominant seizure manifestation. They do not represent simple reactions to motor manifestations of seizures, but are mediated by an activation of the central autonomic network. Autonomic symptoms can be divided into cardiovascular changes, respiratory manifestations, gastrointestinal symptoms, cutaneous manifestations, pupillary symptoms, genital and sexual manifestations as well as urinary symptoms. Due to a hemispheric‐specific representation of the central autonomic network, certain autonomic symptoms may provide lateralizing and sometimes localizing information on the seizure onset zone, although some of these signs may appear as a result of discharge spreading. Autonomic symptoms indicating a seizure onset in the non‐dominant hemisphere include ictal vomiting and retching, spitting automatisms and ictal urinary urge. Autonomic symptoms range from subtle seizure manifestations which become apparent only during meticulous seizure analysis, to severe, sometimes life‐threatening events. Cardiovascular and respiratory autonomic symptoms are discussed as the mechanisms underlying sudden unexplained death in epilepsy. When autonomic symptoms represent the sole seizure manifestation, they can pose problems for differential diagnosis of various nonepileptic conditions. Finally, autonomic seizure symptoms open a unique window on the functional organization of the central autonomic network and on brain function in general. (Published with videosequences.)
Functional MRI assessment of the hemispheric predominance for language in epileptic patients using a simple rhyme detection task
Authors: Baciu Monica , Kahane Philippe , Minotti Lorella , Charnallet Annik , David Danielle , Bas Jean‐François Le , Segebarth Christoph
Published: March 2024

This study assesses the interest of a simple fMRI rhyme detection paradigm to determine hemispheric predominance for language in epileptic patients. Nineteen patients were examined. The findings derived from the fMRI examinations were compared with those obtained on the same patients using the Wada test, stereotactic intracerebral EEG stimulations and recordings, and/or video‐EEG recordings. For the seventeen patients for whom language dominance could be assessed by means of at least one of the latter procedures, the fMRI examination provided concordant results in sixteen. In two patients, the hemispheric predominance for language could only be determined by fMRI. Nine patients underwent surgery subsequent to the fMRI examination. None of them exhibited any aphasic problems following surgery. The rhyme detection task used in the fMRI examination generates robust responses in the language areas, permits easy monitoring of the patient's task performance and can be easily undertaken by the epileptic patients. Thus, this study demonstrates that the fMRI rhyme detection paradigm is particularly well‐suited for determining hemispheric language predominance in epileptic candidates for surgery.
Is the left cerebral hemisphere more prone to epileptogenesis than the right?
Authors: Holmes Mark D. , Dodrill Carl B. , Kutsy Roman L. , Ojemann George A. , Miller John W.
Published: March 2024

Purpose

To determine if there is evidence that the left cerebral hemisphere is more prone to epileptogenesis than the right hemisphere.
Methods.
We examined 532 patients with localization‐related epilepsy, as documented by long‐term EEG‐video monitor studies. We identified those with interictal epileptiform patterns on EEG confined to one hemisphere, those with clinical seizures that arose only from one side, and individuals with neuropsychological deficits lateralizing more to one cerebral hemisphere than the other. These data were then related to the handedness of the subjects.

Results

For left‐handed patients, interictal discharges were significantly more likely to be confined to the left side, seizures more frequently arose from only the left hemisphere, and neuropsychological deficits lateralized more often to the left brain. In contrast, for right‐handed subjects, there were no significant differences in unilateral localization of either interictal discharges or of seizure onsets, nor did neuropsychological deficits lateralize more often to one side or the other. Although, as a group, left‐handers had an earlier age of onset of epilepsy than right‐handers, the lateralizing EEG and neuropsychological patterns seen in left‐handers were not related to age of onset of epilepsy, febrile seizures, family history of either epilepsy or left‐handedness, or other risk factors.

Conclusion

In localization‐related epilepsy, there is a significant association of left‐handedness with left hemispheric lateralization of epileptiform EEG patterns and neuropsychological deficits. These findings do not clearly reflect a greater left than right cerebral hemispheric vulnerability to epileptogensis, except possibly in left‐handers.
Efficacy of lamotrigine add‐on therapy in severe partial epilepsy in adults with drop seizures and secondary bilateral synchrony on EEG
Authors: Bisulli Francesca , Baruzzi Agostino , Rosati Anna , Riva Roberto , Avoni Patrizia , Cerullo Angelina , Tinuper Paolo
Published: March 2024

Objectives

To evaluate the efficacy of lamotrigine (LTG) add‐on therapy in drug‐resistant, partial epilepsy with epileptic drop attacks (EDA) and secondary bilateral synchrony (SBS) on EEG.

Methods

We carried out a single‐center, open‐label, prospective study on a restricted group of patients experiencing an EDA frequency of at least one/month during the previous year regardless of multiple antiepileptic drug (AED) trials. Study design consisted of three phases: a 3‐month baseline period, a 4‐month period in which LTG was titrated and a 9‐month maintenance dose observational period. LTG add‐on therapy depended on valproate (VPA) association, with a maximum of 200 mg/day with VPA and 600 mg/day in the absence of VPA. Every three months, patients underwent clinical, hematological and EEG evaluation including plasma level of AEDs. To assess the efficacy of LTG add‐on therapy, patients were required to keep a detailed seizure diary throughout the study.

Results

Fourteen patients (nine men and five women), aged from 21 to 51, were included in the study. All of them had complex partial seizures (CPS), besides EDA, and half of them had secondarily generalized seizures (SGS). Two of the 14 patients had to stop LTG due to side effects, although one of them was seizure‐free after LTG. Twelve patients completed the study. The improvement was more than 50% for every type of seizure. SGS disappeared in three cases and improved by more than 50% in another three cases. EDA disappeared in six patients; and improved with more than 50% EDA reduction in five patients. CPS disappeared in two patients and improved by more than 50% in eight. EEG improved in nine cases, with SBS disappearing in six patients.

Conclusions

We have demonstrated a good efficacy of LTG adjunctive therapy on EDA. Results include control of SGS and improvement of EEG tracing.
Atypical evolution in childhood epilepsy with occipital paroxysms (Panayiotopoulos type)
Authors: Caraballo Roberto Horacio , Astorino Francisco , Cersósimo Ricardo , Soprano Ana M. , Fejerman Natalio
Published: March 2024

We report, on two, school‐age girls with clinical and electroencephalographic features of early onset childhood epilepsy with occipital paroxysms (CEOP) of the “Panayiotopoulos type” that showed atypical evolution. Neurological examination and brain imaging were normal in both. One child presented at age 2.5 years episodes of oculocephalic deviation, and ictal vomiting during nocturnal sleep. The EEG showed left occipital spikes during wakefulness and sleep. One year later, frequent inhibitory seizures appeared in the lower limbs causing, “pseudoataxic gait”. At the same time she presented with behavioral disturbances and aphasia. EEG showed bilateral spike‐waves while awake and continuous spike‐waves during slow sleep (CSWSS). After switching AEDs to benzodiazepines, control of seizures along with improvement of behavior, and partial restoration of cognitive functions were achieved. The CSWSS disappeared and the last EEG at age 8 years only showed only isolated right occipital spikes.

The other girl had a personal and familial history of febrile seizures. At 4 years of age she presented the first non‐febrile seizures during sleep, with oculocephalic deviation and ictal vomiting, followed by a generalized tonic‐clonic seizure. Partial control of seizures was obtained with antiepileptic drugs. At age 7, the child began to have weekly episodes of oculocephalic version, occasionally with secondary generalization. Repeated inhibitory seizures and absences also appeared. EEG showed frequent bilateral spikes occupying predominantly the posterior regions while awake, and CSWSS. At 7.5 years the same electro‐clinical picture persisted. Ethosuximide was added to sodim valproate and clobazam. Fifteen days later, the seizures disappeared and the EEG showed less frequent bilateral occipital spikes. She is now 9 years old and she has been seizure‐free for 18 months. Her present neuropsychological profile shows mild mental retardation.

The two children with typical electroclinical features of “Panayiotopoulos Type” CEOP developed an atypical evolution which, to our knowledge, has not been described previously.
Nouveaux médicaments antiépileptiques dans les épilepsies de l'enfant: intérêts et limites
Authors: Kaminska Anna
Published: March 2024

Une des nouveautés de ces dernières années a été la découverte que les médicaments antiépileptiques avaient un profil d'efficacité et aussi d'aggravation, spécifiques en fonction des syndromes épileptiques. En raison de la multiplicité de syndromes et du peu d'études contrôlées réalisées chez l'enfant, le «spectre d'action» des antiépileptiques les plus récents n'est pas encore bien connu dans cette tranche d'âge. En effet, les essais contrôlés sont encore trop souvent réalisés d'abord chez l'adulte, puis lorsque le médicament est déjà commercialisé, ils débutent chez l'enfant. Pourtant certaines nouvelles molécules ont révolutionné le traitement des épilepsies de l'enfant, en particulier celui de syndromes épileptiques sévères (comme le syndome de West ou le syndrome de Lennox‐Gastaut).

Le vigabatrin a une efficacité remarquable dans les spasmes infantiles mais peut être responsable d'aggravation dans les épilepsies myocloniques, l'épilepsie‐absences et certaines épilepsies partielles surtout idiopathiques. La lamotrigine est particulièrement efficace dans l'épilepsie‐absences et les épilepsies généralisées symptomatiques et cryptogéniques comme le syndrome de Lennox‐Gastaut ou l'épilepsie myoclono‐astatique. En revanche, une aggravation quasi systématique a été observée dans l'épilepsie myoclonique sévère du nourrisson et des cas d'aggravation ont été notés dans les épilepsies partielles idiopathiques. Le felbamate peut être prescrit en troisième intention et sous surveillance hématologique dans le syndrome de Lennox‐Gastaut. L'efficacité et les indications de l'oxcarbazépine sont comparables à ceux de la carbamazépine. L'efficacité du topiramate a été démontrée dans les épilepsies partielles, les autres indications chez l'enfant restent à préciser. Les études pédiatriques concernant la gabapentine et la tiagabine dans les épilepsies partielles ont montré des résultats encourageants. L'histoire du stiripentol est un bon exemple de stratégie de développement d'un nouvel antiépileptique chez l'enfant; son efficacité en association au clobazam a pu être démontrée dans un syndrome particulier, l'épilepsie myoclonique sévère du nourrisson.
Épilepsie et troubles des apprentissages
Authors: Billard‐Daudu Catherine
Published: March 2024

L'indication dans certaines épilepsies de l'enfant, de choisir comme critère d'efficacité, non seulement les crises épileptiques mais aussi les paroxysmes intercritiques, est réelle dans des situations cliniques bien choisies comme les aphasies acquises avec épilepsies et les épilepsies avec pointe‐ondes continues dans le sommeil avec déficit cognitif acquis. Même si l'explication pathogénique reste mystérieuse, l'intérêt en pratique clinique de ces connaissances est fondamental dans ces pathologies sévères. Une meilleure connaissance des stratégies thérapeutiques s'impose, ainsi qu'une évaluation la plus rigoureuse possible des effets éventuellement bénéfiques des médicaments et enfin l'adjonction systématique à ces essais thérapeutiques d'un programme précis de réhabilitation.
Benign early infantile reflex absence seizures
Authors: Voskuil Piet H.A.
Published: March 2024

This case report, with videotape, focuses on reflex epileptic seizures of a girl who started to have seizures, provoked by tactile stimuli, at the age of twelve months. They were described as myoclonic jerks predominantly in the upper part of the body and the arms.

During video/EEG monitoring at the age of fifteen months, seizures provoked by the same tactile stimuli were recorded. For clinical and electro‐encephalographic reasons, these are best described as absence seizures (persistence of minimal myoclonic jerking). To our knowledge, such seizures at this age have not been documented before.

Treatment with antiepileptic agents was not initiated. Six months later the seizures stopped spontaneously and have not recurred. Two years later, psychomotor and behavioural development is normal [published with video‐sequences].
Small hypothalamic hamartomas and gelastic seizures
Authors: Striano Salvatore , Striano Pasquale , Grillo Sossio , Nocerino Cristoforo , Bilo Leonilda , Meo Roberta , Ruosi Patrizia , Boccella Patrizia , Briganti Francesco
Published: March 2024

Purpose

To describe the clinical history of patients with gelastic seizures (GSs) related to small‐size hypothalamic hamartomas (HHs), and to show some of these unusual seizures.

Material and methods

Patients with GSs and the MRI finding of HH < 1 cm diameter. Ictal EEG or video EEG are required.

Results

Three patients, among 6 with GSs and HH, had a small sessile HH. None of them had a history of precocious puberty, nor any relevant cognitive defects. All patients suffered from other seizure types, in addition to GSs. GSs were drug‐resistant in all cases.

Conclusion

since small, not easily recognizable HHs may be present in patients with GSs, a careful MRI study of the hypothalamic, infundibular and mammillary bodys areas is mandatory in these cases [published with videosequences].
Short duration outpatient video electroencephalographic monitoring: The experience of a southern‐Italian general pediatric department
Authors: Del Giudice Ennio , Crisanti Angela Francesca , Romano Alfonso
Published: March 2024

The authors assessed the event detection rate and clinical usefulness of short duration, outpatient video electroencephalographic monitoring (VEM), in the pediatric age group. The duration of monitoring was set at a two‐hour period. One hundred consecutive patients aged 0‐18 years were enrolled in the study. Patients belonged to one of the following groups: A) patients evaluated to differentiate between true epileptic seizures and nonepileptic events; B) patients with known epilepsy evaluated for a better definition of their seizure type; C) patients with isolated EEG abnormalities evaluated to identify unrecognised, subtle seizures. An additional group D, included patients with enhancement of spike activity induced by sleep. Eighty‐seven patients experienced at least one event per week and 13% had less than one event weekly. The event detection rate was 53% overall, and 61% in the first group of 87 patients. In patients who had events recorded and characterized, epileptic seizures were identified in 37 children (69.8%), and non‐epileptic events in 19 children (35.8%). Diagnostic yield was especially high in children with mental retardation who had predominantly non‐epileptic events. VEM was judged successful and/or informative in 73 cases (73%), and turned out to be useful even in patients with a low baseline frequency of clinical events.
Myoclonic encephalopathy caused by chronic bismuth abuse
Authors: Teepker Michael , Hamer Hajo M. , Knake Susanne , Bandmann Oliver , Oertel Wolfgang H. , Rosenow Felix
Published: March 2024

Bismuth (Bi) is used for the treatment of different gastrointestinal symptoms and disorders such as gastric ulcers. In Germany, Bi medication is available without prescription as over‐the‐counter‐medication even though it can cause severe myoclonic encephalopathy if ingested chronically in high doses.

We report a 49 year‐old woman with chronic gastric ulcers and 5 years of Bi abuse who developed the typical clinical course of Bi encephalopathy. She presented with progressive dementia, dysarthria and myoclonic jerks one week after increasing the Bi dosage. The EEG showed generalized spike‐wave complexes suggesting that the myoclonus was epileptic in nature. Bi intake was stopped and valproate was given, which decreased the frequency of the myoclonic jerks. Administration of the metal chelator D,L‐2,3‐dimercaptopropane‐1‐sulfonic acid (DMPS) led to increased urine excretion of Bi, but was accompanied by a clinical deterioration which resulted in it being discontinued. The subsequent clinical recovery of the patient was documented over 40 days by EEG, video and neuropsychological testing. A time lag of two weeks was observed between falling plasma levels and clinical improvement. In conclusion, Bi‐induced encephalopathy is a differential diagnosis for myoclonic encephalopathies. Treatment with metal chelators may aggravate the encephalopathy. The over‐the‐counter availabily of medications containing Bi should be questioned. (Published with video sequence.)
Epilepsy in children with congenital hemiplegia: correlation between clinical, EEG and neuroimaging findings
Authors: Panteliadis Christos , Jacobi Gert , Covanis Athanasios , Tzitiridou Maria , Kotzaeridou Urania , Arsos Georgios , Kardaras Panagiotis
Published: March 2024

Objectives

the aim of this retrospective, multicentre study was to investigate the relationship between epilepsy, clinical, electroencephalographic (EEG) and neuroimaging findings in children with congenital hemiplegia (CH).

Patients and methods

two hundred and three children with CH were assessed by history, neurological and developmental examination. Electroencephalogram (EEG) and CT/MRI brain imaging were performed in 150 of them (81/150 had an MRI and 69/150 had a CT scan). Patients were re‐evaluated every six months for, at least, a two‐year follow‐up period (range 2‐14 yrs).

Results

the EEG was abnormal in 76% of patients; epileptic seizures developed in 38.9% of them. The frequency of epilepsy paralleled the degree of EEG abnormality, approaching 85% in patients with severe EEG abnormalities and was also closely related to the extent of neuroimaging findings (up to 79% in patients with cerebral malformations). The prevalence of epilepsy in 12/62 patients (19.4%) with mild hemiplegia was significantly lower as compared to 67/141 (47.5%) of patients with moderate or severe hemiplegia. 36.7% of the children had their first seizure between the 1st and the 5th year of life, and 26.5% during the first year of life.

Conclusions

epileptic seizures developed in more than one third of patients with CH, although EEG abnormalities were evident in the majority of them. The prevalence of epilepsy is closely related to the severity of hemiparesis, the extent of neuroimaging findings and the degree of EEG abnormalities. The absence of EEG abnormalities and/or normal (or minor) neuroimaging findings was negatively related to the occurrence of epilepsy.
Ictal bradycardia followed by cardiac asystole: a case report
Authors: Mondon Karl , Charbonnier Bernard , Hommet Caroline , Corcia Philippe , Autret Alain , de Toffol Bertrand
Published: March 2024

We report on a patient with a 30‐year history of left temporal lobe epilepsy who presented with ictal bradycardia followed by cardiac asystole. The EEG during the ictal period was documented and analyzed. Clinical features and therapeutic considerations are discussed.
Investigating temporal pole function by functional imaging
Authors: Dupont Sophie
Published: March 2024

Although the role of the temporal lobe – especially its mesial part – is becoming clearer, that of the temporal pole remains more mysterious. Temporal pole function can be investigated in various ways: by comparing anatomical and clinical data; through experiments in animals; and by using functional imaging techniques. Anatomo‐clinical studies have suggested that the temporal pole is important in autobiographical memory; and studies in monkeys (conditioning and lesional experiments) have revealed a role for the temporal pole in a variety of functions, including taste and olfaction, face recognition, visual discrimination of two‐dimensional pictures, and the mnemonic functions of matching and learning. Functional imaging techniques, whether based on positron emission tomography (PET) or magnetic resonance imaging (MRI), make it possible to observe the functioning of the temporal pole
in vivo
in both healthy control subjects and patients. Initially, this type of approach was simply used to confirm observations made in animals. More recently it has shed light on other functions, especially with respect to processes associated with linguistic integration, which underlie the ability to make lexical and semantic links between different words and make it possible to understand a story in a general way without prior specific knowledge. Studies of epileptic, demented and schizophrenic patients have revealed that abnormal patterns of temporal pole function are typical in all these conditions.
The role of the temporal pole in the genesis of temporal lobe seizures
Authors: Kahane Philippe , Chabardès Stephan , Minotti Lorella , Hoffmann Dominique , Benabid Alim‐Louis , Munari† Claudio
Published: March 2024

Failure of selective amygdalo‐hippocampectomy strongly suggests that the so‐called mesio‐temporal lobe seizures do not always arise from the sole amygado‐hippocampo‐parahippocampal complex. Studies in temporal lobe epilepsies have shown that even in the presence of hippocampal sclerosis, the temporo‐polar region (TP) is often altered, both histologically, anatomically and functionally. These findings are in accordance with our last ten years experience in stereotactic intracerebral EEG recordings (SEEG), during which we frequently observed the simultaneous involvement of both the amygdala (A), the hippocampus (Hc) and the TP at the onset of temporal lobe seizures. Recently, we have reviewed the SEEG findings of 25 patients with a final diagnosis of temporal lobe epilepsy, in whom both mesial, lateral and polar temporal lobe structures were investigated. The analysis of the most representative seizure in each patient showed that a low voltage fast activity was recorded in the TP either initially or whitin the first 5 seconds in 13/25 patients (TP group, 52%), while in the remaining 12 cases, ictal discharges started in the Hc and/or amygdala with a later involvement of the TP (AH group, 48%). Preliminary results of a further study conducted in 48 patients have confirmed the high frequency of early TP involvement (2/3 of the cases) in temporal lobe seizures, the clinical characteristics of which did not differ from ictal clinical findings usually described in the mesio‐temporal lobe epilepsy syndrome. History of prolonged febrile convulsions and diagnosis of Hc sclerosis were more frequent in the AH group, while early consciousness impairment during seizures and anterior temporal white matter changes on MRI were more frequently observed in TP patients. However a clear‐cut distinction beween the two groups remains rather difficult in the absence of intracerebral recordings.
Rationale for treating epilepsy in children
Authors: Guerrini Renzo , Arzimanoglou Alexis , Brouwer Oebele
Published: March 2024

Growing evidence indicates that the effects of antiepileptic drugs on childhood epilepsies are partly linked to the specific type of epilepsy or epilepsy syndrome. Most (but not all) types of epilepsy can be classified into categories that are conceptually meaningful. It is likewise logical to set treatment targets and to estimate the risks according to the main syndromic groups, as they share common, electroclinical presentations and long‐term prognosis. Treatment should then be adjusted to each patient's clinical characteristics.

Treatment should be started soon, whenever there is indication that delay would harm the child. However, if seizures are not disabling, treatment may be delayed, in order to acquire more knowledge about the spontaneous expression of the disorder and the plan thoughtfully explained to the parents. In children presenting with partial symptomatic or cryptogenic epilepsy, it is important to assess the patient's response to several different drugs. However, in patients regarded as having refractory epilepsy, possibilities for a surgical solution must be evaluated early in the course of the disease. In severe epileptic encephalopathies, complete seizure control is impossible and, ideally, treatment should provide as much integration and autonomy, with alleviation of frequent seizures. Again, this should be carefully explained to the parents. In children with severe epileptiform EEG abnormalities coexisting with brain dysfunction (diffuse or specific), the extent of EEG‐related neurological dysfunction should be determined, and vigorous treatment should be started to abate its effects.

Finally, seizures could be worsened by inappropriate drugs, paradoxical reaction or intoxication. Severe childhood epilepsies are particularly at risk and mild idiopathic epilepsies may be transformed into severe disorders, priming a vicious circle of heavy treatment, whereby the original disorder is no longer recognizable.
Epilepsy in elderly people: Management issues
Authors: Tallis Ray , Boon Paul , Perucca Emilio , Stephen Linda
Published: March 2024

Epilepsy in elderly people is far more common than many doctors realise. The prevalence and incidence are frequently underestimated due to underdiagnosis or misdiagnosis of the condition. As the proportion of older people in the population grows, the management of epilepsy in this population will become increasingly important. Elderly people are especially susceptible to the consequences of seizures, particularly loss of self‐confidence, decreased independence, and “falls” resulting in physical injury.

Establishing the diagnosis of epilepsy in old age can be more difficult than in younger patients due to the extensive range of differential diagnoses and a far higher prevalence of concomitant disease. Treatment strategies are demanding; they must allow for the complexities of co‐morbidity, co‐medication, alterations in drug handling, and drug effects on an aging body. Together with a growing array of antiepileptic medication from which to choose, these factors make the management of epilepsy in elderly people particularly challenging.
PET imaging in epilepsy: basal ganglia and thalamic involvement
Authors: Semah Franck
Published: March 2024

Positron emission tomography imaging has revealed basal ganglia and thalamic abnormalities in cerebral blood flow, metabolism and neurotransmission in patients with partial epilepsy. Although these changes remain of uncertain significance, this involvement could be associated with the initiation, propagation or control of seizures. This paper will review the imaging data obtained using positron emission tomography in patients with partial and generalized epilepsy.
Ictal SPECT in a case of pure musicogenic epilepsy
Authors: Gelisse Philippe , Thomas Pierre , Padovani Raymond , Hassan‐Sebbag Nathalie , Pasquier Jacques , Genton Pierre
Published: March 2024

A 39‐year‐old, right‐handed woman had seizures for two years which were always triggered by exposure to various types of music: the first occurred while she listened to a tune she particularly liked,
Con Te Partiro
, by Andrea Boccelli. Other triggering factors were various types of music such as supermarket background music and polyphonic singing or instrumental music played by family members. The seizures had a stereotyped course: she felt anxious, tearful, then occurred slight obtundation, during which she smacked her lips and moved restlessly. There was no complete loss of consciousness, but some degree of amnesia. She never experienced a generalized tonic‐clonic seizure, but reported rare spontaneous feelings of
déjà‐vu
that had begun at the same time as the induced seizures. There were no other spontaneous attacks; only one seizure was apparently provoked, not by music but by a loud background noise in her office. She was a music lover and a singer. Interictal EEG showed independent slow waves over the temporal regions. Several seizures with EEG localisation over the right temporal region were elicited after several minutes of exposure to music. Monoauricular stimulation with the same music produced a seizure when applied to the left ear but was ineffective when applied to the right ear. Ictal SPECT demonstrated right temporal hyperperfusion. MRI was normal. On high dose of carbamazepine, seizure frequency decreased. The addition of topiramate resulted in full seizure control. Musicogenic epilepsy is a rare form of reflex epilepsy. Pure cases, when patients do not experience unprovoked seizures, are exceptional. Our report confirms the implication of the right temporal lobe in this epilepsy. [Published with video sequence].
Reflex epilepsy and non‐ketotic hyperglycemia
Authors: Ozer Feriha , Mutlu Aytul , Ozkayran Tufan
Published: March 2024

Epileptic seizures are paroxysmal events, and it is likely that many, if not most, of them are precipitated by known or unknown factors acting on a central nervous system that is predisposed to the production of epileptic discharges by the presence of an organic lesion, a genetically determined neuronal hyperexcitability, or both. Known precipitating influences are quite varied. We report the case of a 58 years‐old patient, followed for non‐ketotic hyperglycemia, who presented with focal seizures exclusively induced by specific, active or passive, postures and movements of the right arm or hand. MRI was normal. Following regulation of glucose blood levels the position‐induced seizures stopped and antiepileptics were not prescribed. The seizures are kept under control by regulating blood glucose. [Published with video sequences].
Hypothalamic hamartoma in adults
Authors: Mullatti Nandini
Published: March 2024

Epilepsy in adult patients with hypothalamic hamartoma has not been well studied. It is uncommon but merits recognition. In this paper, 14 adult patients with hypothalamic hamartoma and epilepsy, of whom three developed epilepsy only in adult life, are presented. The later onset of epilepsy appears to be associated with a milder epilepsy syndrome, less severe learning difficulties and behaviour problems, and better occupational and social status. Gelastic seizures are less prominent in this age group. Of particular interest, one patient had prominent sleep disturbances characterized by a reduction in total slow wave and REM sleep without daytime sleepiness.

The milder epilepsy and preserved cognitive and social functioning have implications for management. A number of patients were controlled by anticonvulsant medication, and were functioning satisfactorily. For such patients minimally invasive surgical procedures, or medical therapy should be considered.
The endocrinology of hypothalamic hamartoma surgery for intractable epilepsy
Authors: Freeman Jeremy L. , Zacharin Margaret , Rosenfeld Jeffrey V. , Harvey A. Simon
Published: March 2024

Intractable epilepsy has replaced central precocious puberty (CPP) as the main indication for surgery in patients with hypothalamic hamartoma (HH). However, concern about endocrine complications and the paucity of published endocrine data may dissuade clinicians from recommending HH surgery. We report the preoperative endocrine status and postoperative endocrine findings of patients undergoing HH surgery at our centre.

Twenty‐nine patients aged 4‐23 years (mean 10 years) underwent detailed clinical assessment and biochemical testing of the hypothalamic‐pituitary axis before and after transcallosal resection of their HH. The perioperative evaluation included comprehensive evaluation of pubertal status, growth, weight, thyroid and adrenal function, and osmoregulation.

Forty‐five percent of patients had CPP at presentation and this was not altered by HH surgery. Asymptomatic deficiencies in thyroid hormone, growth hormone and cortisol response were identified in several patients prior to surgery, and biochemical CPP was present in four, clinically prepubertal children. Free thyroxine fell after surgery in the majority, and to clinically significant levels prompting treatment in 5 patients. Low growth hormone was present in 5/8 patients who had had previous HH surgery and in 6/29 following transcallosal surgery at our centre; short stature did not result during the period of follow‐up. Hypernatraemia developed in most patients postoperatively with sodium > 150 mmol/L seen in 16 (55%) patients; however, this was asymptomatic, not often associated with polyuria, and transient; no patient required ongoing antidiuretic hormone replacement. Appetite stimulation and early postoperative weight gain occurred in 45% patients, but resolved in half.

Disturbance of endocrine function may be clinically silent and should be routinely evaluated prior to HH surgery for intractable epilepsy. Following surgery, hypernatraemia, low thyroxine, low growth hormone, and weight gain are the main endocrine problems encountered. Prior, unsuccessful surgery may be a risk factor for endocrinopathy. Except for weight gain in some patients, these postoperative endocrine disturbances appear to be transient, mild or asymptomatic, and easily treated where necessary. Long term follow‐up of growth and sexual development in a large series of patients is required.
Transcallosal resection of hypothalamic hamartomas in patients with intractable epilepsy
Authors: Harvey A. Simon , Freeman Jeremy L. , Berkovic Samuel F. , Rosenfeld Jeffrey V.
Published: March 2024

A variety of surgical treatments for intractable epilepsy with hypothalamic hamartoma (HH) are described, although most are derived from limited patient experience gathered from several centres. We describe the results of transcallosal resection of HH in 29 consecutive patients undergoing surgery at one centre.

Twenty‐nine patients aged 4‐23 years (mean 10 years) underwent HH surgery with a minimum of 12 months follow‐up. A comprehensive, presurgical epilepsy evaluation, supplemented with endocrine and ophthalmological assessments was performed in all cases. HH were resected via a transcallosal, interforniceal approach to the third ventricle, with the assistance of frameless stereotaxy, limiting the resection to the margins of the third ventricular walls and floor and minimising traction and diathermy.

Complete or near‐complete (> 95%) resection of the HH was achieved in 18/29 patients, 75‐95% resection was achieved in seven patients (four of whom had complete or near‐complete disconnection of residual HH) and less than 50% resection was achieved in four. Postoperatively (follow‐up 12‐70 months, mean 30 months), 15 became seizure‐free (nine off antiepileptic medication), seven had > 90% reduction in seizure frequency, three had 55‐80% reduction in seizure frequency, and four had less than 40% reduction in seizure frequency. Of 16 patients who had seizures in the early postoperative period, six became seizure. No patient or lesion characteristics were associated with postoperative seizure freedom, including features of symptomatic generalised epilepsy. Neurobehavioural improvement and resolution of EEG abnormalities were seen in the majority. Complications were transient hemiparesis in two, transient hypernatraemia in 17, short‐term memory impairment in 14 (persistent in four), weight gain in ten (persistent in five), need for supplemental thyroxine in five, and lowered growth hormone (uncertain clinical significance) in six.

Transcallosal resection of HH is an effective treatment for intractable epilepsy, with 76% patients in our seizures being seizure‐free or having > 90% seizure reduction. The operative risks include stroke, short‐term memory disturbance, weight gain and minor endocrine disturbances. Based on published data, the transcallosal approach appears to be safer and more effective than other operative strategies.
Hypothalamic hamartoma with refractory epilepsy: surgical procedures and results in 18 patients
Authors: Fohlen Martine , Lellouch Arielle , Delalande Olivier
Published: March 2024

Aim

to study the surgical procedures and results on seizures, in 18 patients with refractory epilepsy due to hypothalamic hamartoma.

Patients and methods

Eighteen patients aged from 9 months to 32 years underwent surgery between 1997 and 2002. The mean age at seizure onset was 15.5 months. Seventeen patients had gelastic seizures, 14 had partial seizures, two had infantile spasms, 10 had tonic or atonic seizures and three had generalized seizures. The mean seizure frequency was 21 per day. Four patients had borderline intellectual disability and the others were mentally retarded. Five patients had precocious puberty, one had acromegaly and four suffered from obesity. Brain MRI, performed at least twice in each patient showed the hamartoma as a stable, homogeneous interpeduncular mass implanted either on the mammilary tubercle or on the wall of the third ventricle, with variable extension to the bottom. Ictal SPECT, performed in four patients, showed hyperperfusion within the hamartoma in two.

Surgical procedure

twenty‐six operations were performed in 18 patients. The first patient underwent a total removal of the hamartoma, whereas the following patients underwent a disconnection, either through open surgery (14 patients) or endoscopy (10 patients).

Results

regarding the seizure outcome with a mean follow up of three years four months (one year to 4.5 years), nine patients are seizure‐free, one patient has only brief gelastic seizures and eight are dramatically improved. Surgery was uncomplicated in all but two patients: one had transitory hemiplegia and paresis of the third cranial nerve, the other presented with hemiplegia due to ischemia of the middle cerebral artery territory. The quality of life, behavior and school performance were greatly improved in most of the patients.

Conclusion

our series illustrates the feasibility and relative safety of disconnecting surgery of hypothalamic hamartomas, with seizure relief in 50% and a dramatic improvement in the others. Endoscopic disconnection seems to be a very safe way to treat the intra‐ventricular part of hamartomas.
Stereotactic surgical approach to hypothalamic hamartomas
Authors: Kuzniecky Ruben I. , Guthrie Barton L.
Published: March 2024

Hypothalamic hamartomas are often associated to a progressive epileptic encephalopathy. Non‐invasive data indicates that destruction or isolation of the hamartoma may stop seizures. We present data on patients who underwent stereotactic radiofrequency with or without endoscopy. Epilepsy improved in 60% of patients with minimal morbidity. This approach was less succesful in large lesions. These results indicate that this approach should be considered in patients with small/medium lesions.
Antiepileptogenesis, neuroprotection, and disease modification in the treatment of epilepsy: focus on levetiracetam
Authors: Klitgaard Henrik , Pitkänen Asla
Published: March 2024

The search for antiepileptic drugs (AEDs) using drug screens that test for the ability to suppress paroxysmal events has primarily resulted in the discovery of AEDs that inhibit neuronal excitability. While profoundly reducing expression of epileptic seizures, current pharmacologic treatments have not been able to completely control seizures in all patients, and can impair normal neuronal excitation underlying cognition. A new approach to drug screening, including the process of epileptogenesis, may yield new classes of drugs that not only suppress seizures but also specifically act to protect against the neurobiological changes that contribute to the development of epilepsy. By preventing or reversing the neuronal circuit reorganizations that produce lowered seizure thresholds following brain insults such as head trauma or status epilepticus, these antiepileptogenic drugs could prevent, or reverse, progressive worsening of the epileptic process. It is likely that antiepileptogenic drugs will have mechanisms of action distinct from traditional AEDs, as the molecular mechanisms underlying epileptogenesis and ictogenesis probably differ. One new AED with potential antiepileptogenic properties is levetiracetam, which was discovered using non‐conventional drug screens. It markedly suppresses kindling development at doses devoid of adverse effects, with persistent suppression of kindled seizures even after termination of treatment. Further design and implementation of antiepileptogenic drug screens are needed for the discovery of other novel disease‐modifying agents.