Association of ictal aphasia with hypoperfusion in language areas in temporal lobe epilepsy patients

Tipologia: CLINICAL COMMENTARY

2023-04-25

Abstract

Ictal clinical phenomenology, including aphasia, is usually associated with increased regional cerebral perfusion. We present an unusual pattern of ictal cerebral perfusion in three patients with pharmacoresistant, lesional temporal lobe epilepsy and ictal/postictal aphasia studied with prolonged video-EEG, ictal, and interictal SPECT and MRI for pre-surgical evaluation. Subtraction of ictal-interictal SPECT images co-registered with MRI (SISCOM) showed ictal hyperperfusion in the temporal epileptogenic area in all patients. In addition, hypoperfusion of Broca's area in one case, Wernicke's area in other patient, and both areas in the remaining one were observed. Ictal aphasia in these patients may be explained by functional inhibition of a primary language area, driven by the epileptogenic network. This pattern can contribute to understand the pathophysiology of some ictal signs, with an impact on the evaluation of individual surgical risks.

Fonte: Epileptic Disorders, Andrés Damian, Mariana Legnani, Patricia Braga, Rodolfo Ferrando

Semiology characteristics and location of epileptogenic zone in motor seizures of axial and shoulder girdle muscles assessed by video stereoelectroencephalography study in 65 patients

Tipologia: ORIGINAL ARTICLE

2023-04-24

Abstract

Objective

To study the semiology characteristics of motor seizures of axial and shoulder girdle muscles (ASMs) by stereoelectroencephalography (SEEG) and its value in determining location of epileptogenic zone.

Methods

A total of 598 patients underwent SEEG assessment in Sanbo Brain Hospital were reviewed; 65 patients with ASMs selected. Thirteen semiology feature items were extracted according to the location and symmetry of involved axial muscles, direction of movement, etc. Seizures were grouped with items, and the k-means was used to analyze association between ASMs semiology characteristics and seizure-onset zone (SOZ).

Results

The SOZs of ASMs involved 23 combinations of seven different brain regions: 31 patients (47.7%) had one brain region, 19 (29.2%) had two, 14 (21.5%) had three, and one patient (1.5%) had four. One hundred and fifteen brain regions were analyzed. Seven brain regions accounted for a significant difference in chi-square test, χ² = 62.79, p < 0.0001, with the highest proportion of insular and perisylvian. The k-means method identified two clusters: cluster 1 had a high degree of agreement with temporal lobe epilepsy (12/15), characterized by less shrug-like movement, later involvement of axial shoulder girdle muscles, longer duration, and lower seizure frequency; cluster 2 had a high degree of agreement with posterior cortex epilepsy (14/18), characterized by earlier involvement of axial shoulder girdle muscles, shorter duration, and higher seizure frequency. In frontal lobe, insular and perisylvian, anterior and middle cingulate gyrus, are the two categories accounted for similar proportion. Seizure-onset lateralized at the contralateral of unilateral cervical tonic, with rate of seizure-free was 73.7%.

Significance

The incidence of ASMs is high in insular and perisylvian. Unilateral cervical tonic seizures have good lateralizing value. Based on semiology characteristics, ASMs can be roughly clustered into two categories, which can only effectively distinguish the origins of temporal lobe and posterior cortex, with low discrimination for the seizure-onset of other lobes.

Fonte: Epileptic Disorders, Zhang Liping, Wang Jing, Yang Yujiao, Deng Qinqin, Yan Zhaofen, Liu Xinshan, Zhang Shidan, Zhou Jian, Guan Yuguang, Liu Changqing, Luan Guoming, Wang Mengyang

New‐onset refractory status epilepticus responsive to VNS: a case report

Tipologia: CASE VIGNETTE

2023-04-23
Epileptic Disorders, Accepted Article.

Fonte: Epileptic Disorders, Sancho Rodriguez‐Villar, Mohamed Ahmed Ahmed, Saif Haddad, Laura Mantoan, Neeraj Singh, Mohamed Mahdi‐roger, Ioannis Stavropoulos, Richard Selway, Antonio Valentin

A super‐elderly autopsy case of benign adult familial myoclonus epilepsy with a heterozygous mutation

Tipologia: CASE VIGNETTE

2023-04-21
Epileptic Disorders, EarlyView.

Fonte: Epileptic Disorders, Maya Tojima, Katsuya Kobayashi, Takefumi Hitomi, Haruka Ishibashi, Daisuke Yoshii, Makoto Sainouchi, Takashi Ayaki, Akihiro Shimotake, Kiyohide Usami, Takakuni Maki, Akiyoshi Kakita, Ryosuke Takahashi, Akio Ikeda

Epilepsia partialis continua and unilateral cortical–subcortical FLAIR‐hyperintense lesion in Rasmussen's encephalitis: Is it diagnostic?

Tipologia: ORIGINAL ARTICLE

2023-04-21

Abstract

Objective

Rasmussen encephalitis (RE) is a focal encephalitis, characterized by epilepsia partialis continua (EPC) with or without seizures and progressive unilateral deficits. Imaging characteristics of RE have been rarely described in detail in relation to EPC. So, the study aimed to explore if any relationship exists between the imaging characteristics and the presence or evolution of EPC in patients with RE.

Methods

This retrospective study included 11 patients with RE fulfilling the European consensus statement on RE followed between 2015 and 2020.

Results

The mean age for onset of seizures was 12 years (range 2.5–24 years). Seven patients had limb EPCs, two had face EPCs, face, and limb EPC in one, and lingual EPC in one patient. The first MRI was done within 1 day to 1 month of the onset of seizures. It was normal in two patients and showed only cortical atrophy, focal or hemispheric in four patients, caudate atrophy in two, and cortical or subcortical hyperintensity (HI) in six patients. Follow-up MRI, within 3 weeks to 6 months of the onset of EPC (mean 1.6 months) showed paramedian frontal HI with limb EPC in six patients. Insular HI in four patients; two had facial EPCs while lingual EPC and limb EPC with facial EPC was observed in one patient each.

Significance

Fluid-attenuated inversion recovery (FLAIR) HI and focal cortical atrophy on MRI is the most common finding in the early course of RE. T2 and FLAIR hyperintensity in the paramedian frontal or insular cortex may antedate the onset of EPC or may occur simultaneously with EPC.

Fonte: Epileptic Disorders, Sujit A. Jagtap, Yogeshwari Deshmukh, Aniruddha Joshi, Sandeep Patil, Nilesh Kurwale, Sujit Nilegaonkar

Sawtooth waves: An EEG normal variant

Tipologia: MULTIMEDIA TEACHING MATERIAL

2023-04-20
Epileptic Disorders, EarlyView.

Fonte: Epileptic Disorders, Fábio A. Nascimento, Sándor Beniczky

Frontal lobe epilepsy and MOGHE: Further observations on electroclinical & imaging phenotypes and surgical perspectives

Tipologia: ORIGINAL ARTICLE

2023-04-18

Abstract

Objective

Mild Malformation with Oligodendroglial Hyperplasia (MOGHE) is a recently described clinicopathologic entity, associated with drug-resistant epilepsy and extensive epileptogenic networks. Knowledge is accumulating about particular electroclinical phenotypes, correlations with imaging and potential prognostic significance for surgical outcome. The study adds relevant information by documenting the presence of a Hyperkinetic Frontal Lobe seizure phenotype in adolescents and an Epileptic Encephalopathy phenotype in young children.

Methods

Five cases subjected to a structured presurgical evaluation protocol, including EEG-FMRI, chronic and acute invasive EEG, subjected to frontal lobe surgery with post-operative follow-up between 15 months and 7 years.

Results

In the two adult cases, surface EEG demonstrated lateralized widespread Frontal Lobe epileptogenicity and hyperkinetic semiological features. MRI demonstrated cortical-white mater blurring and deeper white matter abnormalities. EEG-FMRI suggested concordant Frontal Lobe involvement. iEEG demonstrated a widespread frontal lobe epilepsy network. The three young children demonstrated a diffuse epileptic encephalopathy phenotype, with non-localizing, non-lateralizing surface EEG and “spasms” as the main seizure type. MRI demonstrated extensive frontal lobe subcortical gray and white matter abnormalities, consistent with MOGHE literature for this age, while EEG-FMRI, in 2/3, demonstrated concordant Frontal Lobe involvement. They did not undergo chronic iEEG and the resection was assisted by acute intraoperative ECoG. All cases were subjected to extensive frontal lobectomies with Engel class IA (2/5), IB (1/5) and IIB (2/5) outcomes.

Significance

The study confirms the presence of Frontal Lobe Epilepsyand Epileptic Encephalopathy Phenotypes, in accordance to epilepsy phenotypes already described in MOGHE literature. Presurgical Evaluation Studies, including EEG-FMRI, can provide strong lateralizing and localizing evidence of the Epileptogenic Networks involved. All responded favorably to extensive Frontal Lobe Resections, despite widespread epileptic activity recorder by surface and intracranial EEG pre- and prostoperatively; an Epileptic Encephalopathy Phenotype, in the first years of life, should not discourage such a resection.

Fonte: Epileptic Disorders, Kyriakos Garganis, Kostakis Gkiatis, Jelena Maletic, Jean Harushukuri, Nikolaos Kondylidis, Argiris Dinopoulos, Pelagia Vorgia, Roland Coras, Ingmar Bluemcke, Basilios Zountsas

Role of EEG as a Monitoring Tool in Patients with Glucose Transporter Type I Deficiency Syndrome (GLUT1 ‐DS) on Ketogenic Diet

Tipologia: ORIGINAL ARTICLE

2023-04-18

Abstract

Rationale

Glucose transporter type I deficiency syndrome (GLUT1-DS) is the fourth most frequent single-gene epilepsy refractory to standard antiepileptic drugs. Multiple seizures types and variable electrographic findings are reported. Ketogenic diet is expected to result in the complete resolution of the epileptiform activity.

Methods

A retrospective chart review of patients with GLUT1-DS on ketogenic diet between December 2012 and February 2022 was done. Analysis of the EEGs prior to and during the ketogenic diet was done.

Results

34 patients on ketogenic diet were reviewed. Ten had clinical diagnosis of GLUT1-DS, seven of them had genetic confirmation. 71% were female. Average age at seizure onset was 13.85 m.o. (range: 3-60, SD±20.52), at diagnosis was 44.57 m.o (range: 19-79), and at the onset of Ketogenic Diet was 46.43 m.o. (range: 20-83). 29 months (range: 13-38) delay between symptoms onset until diagnosis was noticed. At the diagnosis 100% reported seizures: 71% myoclonic, 57% generalized motor, 57% absence, 28% atonic, and 14% focal motor. Also, 71% abnormal eyes movements, 57% ataxia and 28% intolerance to fasting. 86% had normal brain MRI. 71% had abnormal EEG. All were on ketogenic diet, four on classical (1.75:1 – 2.25:1 ratio). Six were clinically seizure-free after ketogenic diet.

EEG features included notch delta, focal spike and wave, and generalized spike/polyspike and wave. One patient had bilateral independent centrotemporal spikes. Spikes showed high and very high amplitude in all of them (>200 μV). The variation of the spike index decreased in three patients but increased in two.

Conclusion

Ketogenic diet is the choice treatment for patients with GLUT1-DS. Electrographic features could show worsening after initiation of the ketogenic diet even with seizure control. EEG did not prove to be reliable tool for adjusting KD in our cohort. Centrotemporal spikes have not been reported in patients with GLUT-1 DS.

Fonte: Epileptic Disorders, Juan Toro‐Perez, Sarah Healy, Erick Sell, Srinivas Bulusu, Sharon Whiting

Clinical and electroencephalographic findings prior to the onset of juvenile myoclonic epilepsy: A case series

Tipologia: CLINICAL COMMENTARY

2023-04-17

Abstract

The purpose of this study was to investigate the timing of generalized electroencephalographic abnormalities in patients with juvenile myoclonic epilepsy who were followed up long term before the onset of juvenile myoclonic epilepsy. We enrolled juvenile myoclonic epilepsy patients whose course of epilepsy had been observed for >5 years before the onset of juvenile myoclonic epilepsy, those who had undergone electroencephalogram recording more than twice before the onset of juvenile myoclonic epilepsy, and those who had terminated antiseizure medications for at least 2 years before the onset of juvenile myoclonic epilepsy. Patients who had transitioned from childhood absence epilepsy to juvenile myoclonic epilepsy were excluded. We retrospectively reviewed the medical records and neurophysiological data of the patients. Four patients met the inclusion criteria. One patient was diagnosed with febrile seizures during childhood, and the remaining three had transitioned to juvenile myoclonic epilepsy from other epileptic disorders, such as self-limited epilepsy with autonomic seizures, genetic epilepsy with febrile seizure plus, or nonspecific genetic generalized epilepsy. All patients exhibited generalized spike–wave discharges or photoparoxysmal responses for >2 years before the onset of juvenile myoclonic epilepsy. The four patients had transitioned to juvenile myoclonic epilepsy from other epileptological preconditions. Patients with juvenile myoclonic epilepsy may show generalized electroencephalographic abnormality many years prior to the onset of symptoms. Generalized spike–waves on the electroencephalogram during the course of any type of epilepsy or febrile seizure may be a risk factor for developing juvenile myoclonic epilepsy.

Fonte: Epileptic Disorders, Akiko Hiraiwa, Yu Kobayashi, Moemi Hojo, Jun Tohyama

Highly purified cannabidiol improves stability and postural tone in adult patients with Lennox–Gastaut syndrome: A case series

Tipologia: CLINICAL COMMENTARY

2023-04-17

Abstract

Lennox–Gastaut syndrome (LGS) is a severe developmental epileptic encephalopathy associated with numerous neurological signs and symptoms. Altered postural tone and the need for a caregiver-assisted wheelchair are features characterizing patients with LGS. Highly purified cannabidiol (CBD) is a novel antiseizure medication (ASM) recommended for seizure treatment, in combination with clobazam, in patients with LGS. Adding CBD to the previous ASM treatment helps in reducing seizure frequency, specifically drop seizures, in patients with LGS in both clinical trials and real-world studies. However, no data about drug effects on postural tone, motor activity, gait, and stability are available. In this case series, three adult patients diagnosed with LGS were treated with CBD as an add-on. During the follow-up, a slight improvement in seizure frequency was observed. Unexpectedly, an amelioration in postural tone and stability, measured using the validated Gross Motor Function Classification System, was also detected. Our case series suggests that CBD may help in managing patients with LGS regarding seizure control and in improving other aspects of the clinical spectrum of the disease, such as postural tone and stability. The mechanisms at the basis of this improvement may be related, other than seizure reduction, to the drug's effect on the brain locomotor centers, as demonstrated in animal model studies.

Fonte: Epileptic Disorders, Carmen Calvello, Mariana Fernandes, Clementina Lupo, Fabio Placidi, Francesca Izzi, Ciro Bianco, Maria Grazia Celeste, Nicola Biagio Mercuri, Claudio Liguori