bluuebunny/crossref_metadata_2025_split huggingface dataset:
Nouveaux médicaments antiépileptiques dans les épilepsies de l'enfant: intérêts et limites
Authors: Kaminska Anna 
Published: March 2024

RÉSUMÉ
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

RÉSUMÉ
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

ABSTRACT
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

ABSTRACT

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

ABSTRACT
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

ABSTRACT
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

ABSTRACT

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

ABSTRACT
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

ABSTRACT

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

ABSTRACT
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

ABSTRACT
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

ABSTRACT
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

ABSTRACT
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.
Hypothalamic hamartoma in adults
Authors: Mullatti Nandini 
Published: March 2024

ABSTRACT
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

ABSTRACT
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.