Epilepsy and Behavior Reports最新文献

Exercise is a well-established component in the management of chronic illness both as a primary prevention and secondary intervention. The assumption that in otherwise healthy individuals, higher socioeconomic status (SES) is positively associated with physical activity (PA) has been debated. We report the influence of SES on adherence to home-based exercise program in people with epilepsy (PWE) from a developing country. Participants’ response to self-reported Social Needs Screening Tool of the American Academy of Family Physicians was collected. The current study is a secondary follow-up and post-hoc analysis of data from patients we have previous published. The average age of the study population was 26.93 ± 10.20 years with 57.8 % men. Among the 116 study participants, 31 (26.72 %) were adherent to the exercise program. Unemployment (14.1 % vs. 0.0 %; p = 0.034) was higher, fewer people had least high school education (76.6 % vs 93.5 %; p = 0.050) in PWE who did not adhere to exercise program. A significantly higher number of PWE who were not adherent to exercise reported that their family members or anyone else never physically hurt them (97.6 % vs 80.6 %; p = 0.05), never threaten (94.1 % vs 74.2 %; p = 0.007) and/or never scream at them (90.6 % vs 74.2 %; p = 0.011). In PWE education and employment are associated with adherence to home-based exercise programs. The role of family support and personal safety in adherence to exercise should be evaluated in detail.

Prosopagnosia, a neurological condition affecting perception and differentiation of faces, is categorized as either acquired or developmental (present since birth). Acquired cases of prosopagnosia are usually caused by right hemisphere or bilateral damage. We present a right-handed 17-year-old male with a history of focal epilepsy and a new diagnosis of prosopagnosia due to a perinatal stroke affecting the left lingual gyrus, a structure in close proximity to the fusiform face area. In addition to showing that early acquired cases of prosopagnosia may go unrecognized, this case shows that left hemisphere lesions may also affect facial recognition. It is important to screen for prosopagnosia via comprehensive neuropsychological evaluation in patients with lesions proximal to the fusiform face area.

Responsive neurostimulation (RNS) is a valuable tool in the diagnosis and treatment of medication refractory epilepsy (MRE) and provides clinicians with better insights into patients’ seizure patterns. In this case illustration, we present a patient with bilateral hippocampal RNS for presumed bilateral mesial temporal lobe epilepsy. The patient subsequently underwent a right sided LITT amygdalohippocampotomy based upon chronic RNS data revealing predominance of seizures from that side. Analyzing electrocorticography (ECOG) from the RNS system, we identified the frequency of high amplitude discharges recorded from the left hippocampal lead pre- and post- right LITT amygdalohippocampotomy. A reduction in contralateral interictal epileptiform activity was observed through RNS recordings over a two-year period, suggesting the potential dependency of the contralateral activity on the primary epileptogenic zone. These findings suggest that early targeted surgical resection or laser ablation by leveraging RNS data can potentially impede the progression of dependent epileptiform activity and may aid in preserving neurocognitive networks. RNS recordings are essential in shaping further management decisions for our patient with a presumed bitemporal epilepsy.

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutations in the tumor suppressor genes TSC1 or TSC2. TSC is characterized by the formation of multiple tumors in various organs. The most common neurological manifestation of the disorder is epilepsy present in 79–90% of cases. At least one-third of TSC patients develop drug-resistant epilepsy (DRE) which remains a great challenge for clinicians. Neuromodulation is an option in cases of multifocal epilepsy, epilepsy originating in eloquent areas, or the inability to identify the ictal onset zone. Deep brain stimulation of the anterior thalamic nucleus (ANT-DBS) may be used in the treatment of multifocal DRE. Here, we present a case of a patient with multifocal DRE caused by TSC, who was treated with ANT-DBS. A follow-up period of eight months showed that the patient's multifocal DRE was successfully treated by ANT-DBS.

Developmental and epileptic encephalopathies (DEE) are conditions in which a mutated gene may cause abnormal functioning of the central nervous system, resulting in both encephalopathy and epileptogenesis. We present a case of a girl with a DEE characterized by a Rett-like phenotype in association with febrile and afebrile clusters of focal seizures. The girl presented typical development until the age of 18 months, followed by regression. The first febrile bilateral tonic-clonic seizure was observed at 30 months of age, and the following month seizures recurred in clusters of several episodes per day every 10 days. These seizures were characterized by behavioural arrest, emotional symptoms, head turning, and followed by bilateral tonic-clonic seizures. The administration of valproic acid and levetiracetam led to prolonged seizure control. However, from the age of 7 years, she had monthly recurrent clusters of focal seizures and non-convulsive status epilepticus which occurred at different ages. Brain and spinal cord MRI showed mild non-progressive hemispheric cerebellar atrophy. A next generation sequencing panel for epilepsy identified the de novo splicing mutation c.2973+1G>A of the SMC1A gene.

Juvenile Myoclonic Epilepsy (JME) is an idiopathic generalized epilepsy associated with a characteristic sleep/wake rhythm, with the tendency to go to bed later at night, to get up later in the morning. In the pediatric population, we have previously observed specific circadian and sleep/wake patterns of generalized seizures (6 am-12 pm) and myoclonic seizures (in wakefulness, 6 am to noon). Delayed Sleep-Wake Phase Disorder (DSWPD) is characterized by sleep initiation insomnia when attempting sleep at conventional times and difficulty waking at the required time. Here we present the case of a 20-year-old man with JME, diagnosed DSWPD (sleep schedule 3 am to 11 am), presenting with nocturnal seizures out of sleep, always between 5 and 6am. Improvements in seizure control (seizure frequency from 8 per month to 0 per month) were achieved with timed evening melatonin, combined with behavioral sleep-wake scheduling (sleep schedule 10 pm to 6 am) and morning light therapy. Recognition and characterization of DSWPD in JME, together with assessment of circadian and diurnal seizure patterns, may offer therapeutic consideration for better control of seizures.

Stereotypic neural networks are repeatedly activated in drug-refractory epilepsies (DRE), reinforcing the expression of certain psycho-affective traits. Geschwind syndrome (GS) can serve as a model for such phenomena among patients with temporal lobe DRE. We describe stereo-electroencephalogram (SEEG) exploration in a 34-year-old male with DRE and GS, and his treatment by SEEG-radiofrequency (SEEG-RF) ablation. We hypothesized that this approach could reveal the underlying epileptic network and map eloquent faculties adjacent to SEEG-RF targets, which can be further used to disintegrate the epileptic network. The patient underwent a multi-modal pre-surgical evaluation consisting of video EEG (VEEG), EEG source localization, 18-fluorodexyglucose-PET/MRI, neuropsychological and psychiatric assessments. Pre-surgical multi-modal analyses suggested a T4-centered seizure onset zone. SEEG further localized the SOZ within the right amygdalo-hippocampal region and temporal neocortex, with the right parieto-temporal region as the propagation zone. SEEG-RF ablation under awake conditions and continuous EEG monitoring confirmed the abolishment of epileptic activity. Follow-up at 20 months showed seizure suppression (Engel 1A/ILEA 1) and a significantly improved and stable psycho-affective state. To the best of our knowledge this is the first description of the intracranial biomarkers of GS and its further treatment through SEEG-RF ablation within the scope of DRE.

We report a 60-year-old woman who presented to the emergency department after experiencing a witnessed unknown onset bilateral tonic clonic seizure (GTCS) that culminated in cardiac arrest. A neurology consultant uncovered a years-long history of frequent episodic staring followed by confusion and expressive aphasia, which strongly suggested that she suffered from epilepsy. Thus, her cardiac arrest and subsequent resuscitation met criteria for a near-sudden unexpected death in epilepsy (SUDEP) diagnosis. Serial bloodwork demonstrated transient troponin I elevations and leukocytoses, while a brain MRI revealed global cerebral anoxic injury and a small acute right cerebellar ischemic infarction. A review of her medical record uncovered a hospitalization sixteen months earlier for a likely GTCS whose workup showed similar troponin I elevations and leukocytoses, and surprisingly, a different small acute right cerebellar ischemic infarction in the same vascular territory. To our knowledge, this is the first report of subcortical ischemic infarctions occurring concurrently with GTCSs in a near-SUDEP patient. Aside from illustrating the key role of inpatient neurologists in the diagnosis of near-SUDEP, this manuscript discusses the potential significance of postictal ischemic infarctions, transient asymptomatic troponin elevations, and transient non-infectious leukocytoses in epilepsy patients with cardiovascular risk factors.

To investigate the quality of epilepsy care in a region in Japan that lacked specialised care, we retrospectively evaluated patients who visited our newly established epilepsy division between April 2018 and March 2021, and had been treated with anti-seizure medications (ASMs) for at least 1 year prior.

Of the 231 patients included, 169 had ongoing seizure episodes at first visit (seizure-persist group) and 62 had no seizure episodes for more than a year (seizure-free group). Eighty-three patients in the seizure-persist group had not received specialised epilepsy care, 15 had been treated with unnecessary medications, and seven had experienced side effects from ASMs. Twelve patients in the seizure-free group had been treated with unnecessary ASMs, 10 had been treated with ASMs with teratogenic potential and four had experienced ASM side effects. These patients could be classified as having an advanced epilepsy treatment gap (ETG) because they had not previously received necessary specialised care. The progressive decline in the number of patients with advanced ETG suggests that our new epilepsy division has addressed this issue.

This study highlights that a significant number of patients with advanced ETGs exist in Japan and that proper countermeasures are required to address this gap.

Several studies have suggested the epileptogenic potential of temporal encephaloceles. However, there is limited literature describing the results of intracranial EEG monitoring for patients with temporal encephaloceles. We describe a 19 year-old right-handed woman with drug-resistant epilepsy who presented with seizure onset at age 16 in the setting of a left temporal encephalocele where the seizure onset zone was confirmed to be the encephalocele via stereo EEG (sEEG). She had focal impaired awareness seizures occurring weekly that would progress to focal to bilateral tonic-clonic seizures monthly. Imaging showed a left anterior inferior temporal lobe encephalocele and a left choroidal fissure cyst that were stable on repeat imaging. Prolonged scalp recorded video EEG recorded seizures that showed either near simultaneous onset in the bitemporal head regions or a transitional left temporal sharp wave followed by maximum evolution in the left temporal region. Invasive monitoring with sEEG electrodes targeting primarily the left limbic system with one electrode directly in the encephalocele captured seizures with onset in the left temporal pole encephalocele. A limited resection was performed based on the results of the sEEG and except for one seizure in the immediate postop period in the setting of infection, patient remains seizure free at her 4 month follow up. This report describes a case of drug-resistant focal epilepsy where sEEG monitoring confirmed a temporal encephalocele to be the seizure onset zone without simultaneous onset at mesial temporal or other neocortical structures that were sampled. Our findings support the potential for epileptogenicity within an encephalocele with direct intracranial monitoring.