bluuebunny/crossref_metadata_2025_split huggingface dataset:
Functional organization of the circuits connecting the cerebral cortex and the basal ganglia: implications for the role of the basal ganglia in epilepsy
Authors: Slaght Seán Joseph , Paz Tamar , Mahon Séverine , Maurice Nicolas , Charpier Stéphane , Deniau Jean‐Michel 
Published: March 2024

ABSTRACT
The basal ganglia are composed of a set of forebrain structures implicated in the adaptive control of behaviour. These structures process information originating from the entire cerebral cortex, as well as from nonspecific thalamic nuclei and the amygdala. In turn, they redistribute the integrated signals toward thalamic and brainstem nuclei related to motor, premotor, prefrontal and limbic cortical areas. During the two last decades, there has been increasing experimental evidence that the basal ganglia circuitry may be part of a remote control system influencing the spread of epileptic seizures. In the present article, we review the basic principles of the functional organization of the basal ganglia and provide experimental data on the activity that is transmitted by the cerebral cortex to the input stage of the basal ganglia during absence seizures. The functional organization of the basal ganglia supports the current hypothesis that these structures can dynamically control generalized seizures through their input‐output relationships.

RÉSUMÉ – Organisation fonctionnelle des circuits connectant le cortex cerebral aux ganglions de la base: implication pour le rôle des ganglions de la base dans l'épilepsie.
Les ganglions de la base sont constitués d'un ensemble de structures sous corticales impliquées dans le contrôle adaptatif du comportement. Ces structures intègrent des informations issues de l'ensemble du cortex cérébral ainsi que des noyaux thalamiques non spécifiques et de l'amygdale. En sortie, les ganglions de la base exercent leur influence sur des noyaux du thalamus et du tronc cérébral en relation avec les aires corticales motrices, prémotrices, préfrontales et limbiques. Au cours de ces vingt dernières années, diverses données expérimentales ont étayé l'hypothèse que les ganglions de la base font partie d'un système de contrôle de la propagation des crises d'épilepsie. Cet article résume les principes fondamentaux de l'organisation fonctionnelle des ganglions de la base et décrit l'activité transmise par le cortex cérébral à l'étage d'entrée du système des ganglions de la base pendant les crises d'épilepsie de type absence. L'organisation fonctionnelle des ganglions de la base est cohérente avec l'hypothèse que ce système exerce un contrôle dynamique sur les crises d'épilepsie généralisées par l'intermédiaire de ses relations entrée‐sortie.
Psychiatric aspects of patients with hypothalamic hamartoma and epilepsy
Authors: Savard Ghislaine , Bhanji Nadeem H , Dubeau François , Andermann Frederick , Sadikot Abbas 
Published: March 2024

ABSTRACT
Uncontrolled rage, while long associated with hypothalamic hamartoma, has not been as extensively studied as the epilepsy. Rage can be more detrimental to quality of life than seizures. It is now realized that behavior and aggression improve after a complete resection of the hypothalamic hamartoma correlating with a good seizure control post‐surgically.
We report on the longitudinal psychiatric history of a patient with hypothalamic hamartoma and rage whose severe and refractory epilepsy was ultimately treated by thalamic and intrahamartoma chronic stimulation. Our patient did not exhibit sham rage typical of hypothalamic lesions, but rather multifactorial aggressive bouts typical of challenging behaviors seen with mental retardation. The anxious and social features of the aggression suggest that psychiatric interventions, which have been neglected as the emphasis has been on seizure control, are worthwhile in the overall management of this difficult case.
Heritable syndromes with hypothalamic hamartoma and seizures: using rare syndromes to understand more common disorders
Authors: Biesecker Leslie G. 
Published: March 2024

ABSTRACT
Manifestations of non‐syndromic hypothalamic hamartomas are different from those of the syndromic hamartomas, and the syndromic ones are rare. In spite of the rarity of the syndromic hamartomas, they can be a powerful biological tool to help us understand the pathophysiology of these heritable disorders. Several disorders that include hypothalamic hamartomas are reviewed here and the current understanding of the biology of the lesions is summarized.
Resective surgery for hypothalamic hamartoma
Authors: Polkey Charles E 
Published: March 2024

ABSTRACT
Hypothalamic hamartoma presents with precocious puberty, epilepsy or both. There are two epileptic syndromes, one presenting initially in infancy with gelastic seizures evolving rapidly into a syndrome with multiple seizures, developmental delay and a moderate to severe behaviour disorder. The other presents later with a milder epileptic syndrome, again usually including gelastic seizures, but with normal intellect and behaviour. Magnetic resonance imaging identifies and gives a detailed anatomical picture of these lesions. Direct surgery, using microsurgical techniques and neuronavigation guidance has been used for these lesions. Three surgical approaches have been used, one lateral pterional, another midline frontal through the lamina terminalis and a third is a transcallosal interforniceal approach. In addition a disconnection procedure, usually pterional, aims to disconnect the lesion without the risks of major resection. The transcallosal interforniceal approach is the most successful with 69% of patients seizure‐free. There are complications in about 24% of patients, the same as other approaches, but the complications are milder and include fewer neurological deficits than the other routes. Alternate strategies include stereotactic radiosurgery and radiofrequency ablation under stereotactic control.
Efficacy of levetiracetam in partial seizures
Authors: Devinsky Orrin , Elger Christian 
Published: March 2024

ABSTRACT
Controlled clinical trials and routine clinical practice demonstrate that levetiracetam is effective as add‐on therapy and appears to allow for withdrawal to monotherapy in patients who respond well in the add‐on setting. In pivotal clinical trials of adjunctive therapy with levetiracetam 1000 to 3000 mg/day (pooled data), 40% to 54% of patients experienced a 50% or greater reduction in seizure frequency, compared with 18% to 28% of patients treated with placebo. The median percent reduction from baseline in seizure frequency ranged from 36% to 68% for levetiracetam, versus 10% to 23% for placebo. Seizure freedom was achieved by 11% to 35% of those in the levetiracetam treatment group, compared with 3% to 18% of those in the placebo group. (All comparisons statistically significant versus placebo for simple partial, complex partial, and secondarily generalized seizures except for percentage of seizure‐free patients with simple partial seizures.) Clinical observations are consistent with these findings.
Role of levetiracetam in the treatment of epilepsy
Authors: Brodie Martin J. , French Jacqueline A. 
Published: March 2024

ABSTRACT
Physicians treating patients with epilepsy have a host of therapeutic options. Drug choice is dictated first by the seizure(s) and/or epilepsy syndrome. Age is also a factor. Special considerations apply to women, particularly during their childbearing years, and to patients who are learning‐disabled. Drug selection is further influenced by such characteristics as spectrum of activity, rapid response, low potential for drug‐drug interactions, and ease of use. In addition to clinical trial data, postmarketing assessments of the new antiepileptic drugs provide useful clinical information on efficacy and safety. Levetiracetam has specific characteristics that make it an optimal choice for many patient populations.
Benign idiopathic occipital epilepsy: report of a case of the early benign type
Authors: Thomas Pierre , Arzimanoglou Alexis , Aicardi Jean 
Published: March 2024

ABSTRACT
We report the electro‐clinical characteristics of a case with Gastaut type idiopathic occipital epilepsy. The visual seizures, a cardinal feature of the syndrome, are described and illustrated by video sequences. A number of stereotyped seizures are shown where the child, an 11 years old boy, clearly describes the visual phenomena experienced. Prognosis of this common form of partial idiopathic epilepsy is relatively good but, as in our case, seizures may be very frequent during a long period of time. In such cases the term ‘benign’ is probably not the most appropriate [published with video sequences].
Morphological neuroimaging of malformations of cortical development
Authors: D'Incerti Ludovico 
Published: March 2024

ABSTRACT
Malformations of cortical development are classified on the basis of imaging features and stages of cortical development. They are grouped by causes of the malformation: abnormal glial and neuronal proliferation, abnormal neuronal migration and abnormal cortical organisation. Focal or multifocal and generalised forms are recognised in each of these groups. In the first group, generalised forms include microlissencephalies. Among focal‐multifocal abnormalities, neoplastic forms include ganglioglioma and dysembryoplastic neuroepithelial tumours. Non‐neoplastic forms include focal cortical dysplasia and tuberous sclerosis. Malformations due to abnormal migration include lissencephalies; cortical heterotopias are recognised in both focal and generalised forms. Abnormal cortical organisation includes polymicrogyria, in generalised or focal forms, and schizencephalies among the focal forms.
The mortality of epilepsy revisited
Authors: Gaitatzis Athanasios , Sander Josemir W 
Published: March 2024

ABSTRACT
Epilepsy carries a significant mortality that, on average, is 2‐3 times higher than in the general population. Causes of death in epilepsy are presented. Mortality in epilepsy is assessed by means of particular parameters; the mortality rate, the standardised mortality ratio, and the proportional mortality rate. An overview of their use and significance is given here. A number of epidemiological studies have assessed mortality in people with epilepsy in the general population and in populations from hospitals, out‐patient departments, and epilepsy centres. Methodological issues concerning the study of mortality in these populations are discussed. Epidemiological data are presented to describe the overall and cause‐specific mortality, as well as determinants of mortality in epilepsy, such as epilepsy and seizure types, time from diagnosis, and age. It has become clear from population studies with long‐term follow‐up that epilepsy has a higher mortality in the first few years after diagnosis that tends to decrease over time. The pattern of mortality in epilepsy can reflect the underlying conditions causing epilepsy or be associated with the effect of seizures. Emphasis is given to preventable causes of death in epilepsy, such as sudden unexpected death in epilepsy and suicide, which are discussed more extensively. The size of the problem and measures to avoid more deaths in epilepsy are discussed in the light of recently published data.
Unruptured intracranial aneurysm as a cause of focal epilepsy: an excellent postoperative outcome after intra‐arterial treatment
Authors: Kuba Robert , Krupa Petr , Okáčová Lenka , Rektor Ivan 
Published: March 2024

ABSTRACT
This report involves a patient suffering from focal epilepsy caused by an unruptured, intracranial aneurysm (UIA), and her treatment using intravascular embolisation, with an excellent postoperative outcome in terms of the epilepsy. A 52 year‐old, right‐handed woman had been suffering for three years from focal seizures, characterized by loss of consciousness, and oroalimentary and hand automatisms. The epilepsy was not controlled by carbamazepine monotherapy. Magnetic resonance imaging and digital subtraction angiography revealed a saccular aneurysm at the bifurcation of the middle cerebral artery, in contact with the cortex of the temporal opercular region. An intra‐arterial embolisation, using a detachable, mechanical spiral (MDS, Bold Platinum), was performed, with no complications. The purpose of embolisation was the treatment of the aneurysm. The patient had two more, complex partial seizures (CPS) in the early postoperative period, but was seizure‐free between September 1998 and September 2002. This is the first case report that presents the successful treatment of UIA‐related epilepsy by means of intravascular embolisation.
Rasmussen's encephalitis
Authors: Hart Yvonne 
Published: March 2024

ABSTRACT
Rasmussen's encephalitis, a syndrome characteristically presenting in children with the onset of partial motor seizures followed by progressive hemiparesis and cognitive impairment, and accompanied by unilateral cerebral atrophy, was described nearly 50 years ago, yet the cause and optimum treatment remain unclear. Although it was originally presumed to have a viral aetiology, the possible roles of antibody‐mediated mechanisms and more recently cell‐mediated immunity in its pathogenesis have come under increasing scrutiny in the last ten years. These developments are discussed, together with a review of the clinical features. The advances in treatment which have accompanied these changes are also assessed.
An unusual case of neurocutaneous melanosis
Authors: de Andrade Daniela Oliveira , Dravet Charlotte , Raybaud Charles , Broglin Dominique , Laguitton Virginie , Girard Nadine 
Published: March 2024

ABSTRACT
The neurocutaneous melanosis (NCM) is a rare, neuroectodermal dysplasia defined by the association of giant or multiple, nonmalignant pigmented cutaneous nevi with leptomeningeal melanosis or melanoma. As a rule, the cerebral pathological substratum is characterized by a melanocytic infiltration of the leptomeninges, often leading to hydrocephalus. The most frequent clinical symptomatology starts early in life, with convulsive seizures, psycho‐motor delay, intracranial hyperpression: the prognosis is severe. Malignant melanomas can also occur. One 21 years‐old patient affected by NCM with a giant bathing nevus and epilepsy is reported. Her psychomotor development was slightly delayed. Academic progress was disturbed by the frequency of seizures and the multiple dermatological surgeries, and she remained at the elementary school level. Her epilepsy appeared at seven years and became pharmacoresistant. It was a focal, left temporal epilepsy. Neuroimaging investigations were performed repeatedly, and demonstrated the progressive appearance of parenchymal lesions with T1 and T2 shortening, without contrast enhancement, at the pons (11 years), the two hippocampi (14 years), and of an atrophy of the cerebellum and the brainstem (19 years). No hydrocephalus, tumoral aspect, or meningeal involvement were demonstrated. This patient's case is peculiar because her neurological symptomatology consists only of focal epilepsy, unrelated to a tumor, with moderate cognitive impairment despite a rather long course of the disease. Her evolution raises the question of condidency to surgical treatment. [Published with videosequences]
The roles of electroencephalography and neuroimaging in children with holoprosencephaly
Authors: Yang Ming‐Tao , Lee Wang‐Tso , Peng Steven Shinnforng , Lin Haung‐Chi , Tseng Chao‐Lin , Liang Jao‐Shwann , Wang Peng‐Jung , Shen Yu‐Zen 
Published: March 2024

ABSTRACT
We analyze the respective roles of neuro‐imaging and EEG in the assessment of 11 children with holoprosencephaly and epilepsy. Seizures were present in seven patients (64%) ; six were treated with antiepileptic drugs ; five had intractable epilepsy. Two of the patients with intractable epilepsy became seizure‐free under polytherapy. Fourteen EEG recordings were performed in eight patients. The abnormal EEG findings included slow waves, focal epileptiform discharges, slow spike‐and‐wave complexes, hypsarrhythmia, frontal fast activity, fronto‐occipital gradients of amplitudes (posterior amplitude attenuation), lack of photic driving, periodic discharges, and extremely large amplitudes. A fronto‐occipital gradient was found only in alobar and semilobar holoprosencephaly (HPE), while hypsarrhythmia only in lobar HPE. Lack of photic driving was found only in alobar HPE. All EEGs showed diffuse slow waves, and all patients had severe developmental delay. The Deep Gray Score (DGS) in neuroimaging studies, thought to predict clinical outcome, was irrelevant given the presence and intractability of the epilepsies. Patients with higher DGS, nonetheless, tended to have higher mortality rate. In conclusion, EEG evaluation provides additional functional information to neuroimaging studies in the assessment of neurological outcome in patients with HPE. With a more mature and well‐formed cerebrum, as found in the lobar and semilobar types, the possibility of hypsarrhythmia and photic driving increased, while that of fronto‐occipital gradients decreased.
Motor impairment on awakening in a patient with an EEG pattern of “unilateral, continuous spikes and waves during slow sleep”
Authors: Veggiotti Pierangelo , Cardinali Simonetta , Granocchio Elisa , Avantaggiato Paolo , Papalia G. , Cagnana Antonio , Lanzi Giovanni 
Published: March 2024

ABSTRACT

Movement disorders are rarely described in association with the “continuous spikes and waves during slow sleep (CSWS)” EEG pattern. We report the case of a young girl affected by an epileptic encephalopathy who, from the age of seven years and four months, has twice presented a movement disorder affecting the right arm, manifesting on awakening and disappearing by early afternoon. Sleep EEG during these periods showed continuous, highamplitude, diphasic spikes and slow waves over the left hemisphere. Association of clobazam, valproic acid and, on the second occasion, ethosuccimide led to disappearance of the above‐described EEG picture and associated motor symptoms. Neurophysiological investigations excluded other possible aetiologies. In view of this, and of the close relationship between the EEG picture and clinical course, we interpret the patient's impairment as “motor neglect” secondary to the continuous electrical activity recorded during sleep over the left hemisphere and involving the associative areas. This electrical activity in sleep, may be regarded as a “functional lesion” whose clinical consequences can be correlated with the site of the abnormalities.
[Published with video sequences]
Spike frequency is dependent on epilepsy duration and seizure frequency in temporal lobe epilepsy
Authors: Janszky Jozsef , Hoppe M. , Clemens Z. , Janszky I. , Gyimesi C. , Schulz R. , Ebner A. 
Published: March 2024

Objectives
We wanted to investigate factors that are associated with frequency of interictal epileptiform discharges by investigating 303 patients with temporal lobe epilepsy (TLE).

Methods
We included all patients who consecutively underwent the adult presurgical evaluation program at our center and who had intractable, medial TLE with complex partial seizures due to unilateral medial temporal lobe lesions. The interictal EEG samples were automatically recorded and stored on computer. The location and frequency of interictal epileptiform discharges were assessed by visual analysis of interictal EEG samples of 2‐minute duration every hour.

Results
There were 303 patients (aged 16‐63) who met the inclusion criteria. The median interictal epileptiform discharge frequency was 15 IED/h, the median seizure frequency was 4 seizures/month. According to univariate analyses, we found that age at monitoring, epilepsy duration, and higher seizure frequency were associated with higher interictal epileptiform discharge frequency. In the logistic regression analysis, we found that higher seizure frequency (p < 0.001) and longer epilepsy duration (p = 0.007) were independently associated with higher spike frequency, while the age at monitoring was not.

Conclusions
Seizure frequency and epilepsy duration (years of patient's life with seizure activity) were independently associated with IED frequency, suggesting that IED are modulated by seizures.
Identifying baseline characteristics of placebo responders
versus
nonresponders in randomized double‐blind trials of refractory partial‐onset seizures
Authors: Niklson Ida , Edrich Pascal , Verdru Peter 
Published: March 2024

ABSTRACT

In add‐on studies of partial‐onset seizures, the placebo response, defined as a 50% decrease from baseline in seizure frequency, ranges from 0‐19%. Reasons for this significant difference between placebo groups in different trials are not given in the literature. This exploratory analysis was undertaken to compare the baseline characteristics of placebo responders and nonresponders, in an attempt to identify common features. The pooled statistical analysis was performed on the database for three pivotal studies of levetiracetam (n = 904). Using the 50% response definition, we found that 45.6% of placebo nonresponders were on one antiepileptic drug at baseline, compared with 69% of placebo responders. The difference in number of baseline antiepileptic drugs was almost statistically significant (p = 0.056). Placebo nonresponders also tended to have epilepsy for longer than responders. The mean age at onset of epilepsy was consistently different between placebo nonresponders and responders (15.2
versus
20.8 years, respectively; p = 0.019). These findings suggest that the placebo response is higher in patients with partial‐onset seizures who are taking only one antiepileptic drug at baseline and have later onset and shorter duration of epilepsy than in patients on more than one antiepileptic drug at baseline with earlier onset and longer duration of epilepsy.
Herpes
encephalitis as a cause of nonconvulsive status epilepticus
Authors: Gunduz Aysegul , Beskardes Ali Fuat , Kutlu Ayse , Ozkara Cigdem , Karaagac Naci , Yeni Seher Naz 
Published: March 2024

ABSTRACT

Nonconvulsive status epilepticus is a specific form of status epilepticus characterized by alteration in mental status and persistent seizure activity on EEG, with or without motor phenomena. Recognition of the consequences of nonconvulsive status epilepticus has attracted greater attention to this condition. We present a 24‐year‐old woman with epilepsy who developed nonconvulsive status epilepticus during pregnancy. Despite treatment with antiepileptic drugs, the seizures persisted and confusion deepened. Further workup to explain the etiology revealed the diagnosis as herpes encephalitis. She recovered completely by the third day of parenteral acyclovir administration.
Herpes simplex
encephalitis causing nonconvulsive status epilepticus in a pregnant, epileptic woman is an unfortunate and unusual condition, which was simultaneously complicated by the presence of multiple etiological factors.
Uncommon, exceptional and atypical: a case of frontal lobe epilepsy due to cortical dysplasia
Authors: Grant Arthur C. , Kim Richard B. 
Published: March 2024

ABSTRACT

We report a case of frontal lobe epilepsy due to focal cortical dysplasia that included three independent unusual features. The patient, a 45‐year‐old, right‐handed woman, had her first seizure at age 29, well into adulthood. Seizures had been easily controlled with medication for 15 years, then without provocation they became medically intractable during a single, identifiable day. Resection of the dysplastic tissue in the posterior dorsolateral
right
frontal cortex rendered the patient seizure‐free, but produced a significant, non‐fluent, Broca type, crossed aphasia. In addition, the seizure semiology was striking and remarkable, a common finding in frontal lobe epilepsy.
[Published with video sequences]