Sleep-related hypermotor seizures in aspartylglucosaminuria: A case report

From the age of 24 years a young man with a definitive diagnosis of aspartylglucosaminuria (AGU) presented short-lasting sleep-related paroxysmal events characterized by sudden awakening with a frightened look, hyperventilation, and complex bilateral motor activity. Nocturnal video-polysomnography recorded several events consistent with a diagnosis of hypermotor epileptic seizures. This pattern of sleep-related epileptic seizures has been reported in rare familial cases, more often in cryptogenic and symptomatic patients in the spectrum of nocturnal frontal lobe epilepsy. Epilepsy and sleep-related nonepileptic problems are common in patients with AGU, but no case of hyperkinetic nocturnal frontal lobe seizures has been reported to date. Differential diagnosis of abnormal paroxysmal motor events in sleep is frequently a challenge for the clinician: Video-polysomnographic recordings might serve to identify the possible epileptic origin of some of the excessive motor activities during sleep referred in patients with AGU.     

Surgical treatment of patients with drug‐resistant hypermotor seizures

Purpose: To describe the clinical, electrophysiologic, neuroradiologic, and histologic findings in our patients with drug‐resistant hypermotor seizures (HMSs) and to evaluate the outcome of their surgical treatment. Methods: Twenty‐three patients were identified by criteria for drug‐resistant HMS. Surgical treatment and presurgical evaluation modalities including semiology, magnetic resonance imaging (MRI), interictal/ictal scalp video‐EEG (electroencephalography), and intracranial recording were analyzed retrospectively. Results: The common seizure frequency of 60–300 per month was observed among 15 patients. Sixteen patients (69.6%) experienced auras such as fear and palpitation. Marked agitation was observed in 12 patients and mild agitation in 11 patients. Groaning/shouting and asymmetric posturing were common accompanying symptoms. Asymmetric posturing was observed more often in patients with mild agitation than in those with marked agitation (p = 0.027). MRI detected focal abnormalities in six patients. Intracranial recording was conducted in 16 patients. The origins of seizures were localized in mesial frontal cortex in four patients, dorsolateral frontal cortex in four patients, and mesial temporal cortex in two patients. The epileptogenic zones (EZs) were resected from the frontal lobe in 21 patients and from the temporal lobe in 2 patients. The follow‐up ranged from 12–60 months. Seventeen patients (73.9%) had been seizure‐free, 11 of whom had presented with marked agitation (11 of 12) and 6 with mild agitation (6 of 11) (p = 0.069). Histologic examinations demonstrated focal cortical dysplasia (FCD) in 18 patients. Discussion: The HMSs can originate from both the mesial and dorsolateral frontal cortex, and occasionally from the temporal lobe. Patients with drug‐resistant HMSs should be recommended for resective surgical treatment.     

癫痫过度运动发作的症状分析

目的 分析过度运动发作(hypermotor seizure)的癫痫临床症状特征及手术预后。方法 8例运动过度发作的患者，分析癫痫37次发作期间的临床症状，临床发作的起始时间，癫痫病灶的位置，手术结果及病理结果。结果 过度运动发作动作包括：撞头、上肢舞动、前后出拳动作、踢脚、肢体甩动、下肢蹬车动作、躯体翻滚、躯体晃震、骨盆前后甩动等反复激烈动作。癫痫发作中其他发作症状包括：匝眼、扮鬼脸、紧握和抓挠、怪声、紧张姿势。过度运动发作在一次癫痫发作开始的l～53秒(平均12．2秒)时出现，持续时间l～40秒(平均18．6秒)。癫痫灶位置：内侧额叶5例，外侧额叶2例，外侧颞叶l例。8例中，7例术后癫痫发作消失。病理：8例均为皮质发育异常。结论 过度运动发作的产生主要来源于躯体和近体关节的反复激烈运动，常与其他类型发作混合发作；额叶癫痫容易产生此类发作，尤其是额叶内侧起源癫痫；外科的手术效果较好。     

Analysis of clinical patterns and underlying epileptogenic zones of hypermotor seizures

Background: Hypermotor seizures (HMS) are characterized by complex movements involving the proximal segment of the limbs and trunk. Although they are primarily reported in mesial frontal or orbitofrontal epilepsies, they have also been described in patients with temporal or insular epilepsies, questioning the localizing value of HMS in patients contemplating epilepsy surgery. Furthermore, HMS can include different forms of HM behaviors. Whether these clinical variations may be systematized and may reflect different locations of the epileptogenic zone (EZ) has not been evaluated yet. Methods: We have retrospectively analyzed ictal signs and intracerebral EEG data in 11 patients presenting with HMS who became seizure free following epilepsy surgery with a minimum follow‐up of 24 months. Clinical phenomena were reviewed blinded to seizure onset zone. Results: Two types of HMS could be distinguished in this population: HMS1, observed in six patients, mainly consisted of marked agitation that either included body rocking, kicking, or boxing, associated with a facial expression of fear. HMS2, observed in the five other patients, mainly consisted of mild agitation that included either horizontal movements or rotation of trunk and pelvis while lying on the bed, usually associated with tonic/dystonic posturing. SEEG findings showed that the EZ associated with HMS1 was mainly centered on the ventromesial frontal cortex. Conversely, HMS2 was primarily associated with an EZ localized within the mesial premotor cortex. Conclusions: Although these findings remain to be confirmed by larger studies, they may help optimize the placement of intracerebral electrodes in patients contemplating epilepsy surgery.     

Surgical treatment of hypermotor seizures originating from the temporal lobe

PurposeTo describe the characteristics of electroclinical manifestations in patients with hypermotor seizures (HMSs) originating from the temporal lobe.MethodsWe retrospectively reviewed the data of patients who underwent surgical treatments for seizure to identify patients with HMSs of temporal origin. We systematically reviewed patient seizure histories, imaging reports, video-EEG monitoring data, operative records and pathological findings.ResultsEight of the 9 patients reported auras. The ictal behavior included marked agitation in 5 patients and mild agitation in 4 patients. All of the 9 patients exhibited stiffness or dystonia of the upper limb or contralateral limbs during ictus. Seven of the 9 patients completed intracranial recording and at least 3 seizures were recorded for each patient. The intracranial recordings showed ictal activity originating from mesial temporal lobe in 6 patients and the lateral temporal lobe in 1 patient. The time interval of ictal propagation from the temporal to frontal lobe was 15.0 ± 8.3 s. While the time interval from EEG origination to the beginning of hypermotor behavior was 21.0 ± 8.1 s. Brain MRIs revealed hippocampal sclerosis in 3, neoplastic lesion in 1, and normal images in the remaining 5 patients. Patients were followed for 1–5 years after the anterior temporal lobectomy; 7 patients remained seizure-free throughout follow-up.ConclusionSome HMSs can originate from the temporal lobe. In carefully selected patients, surgical resection may lead to good outcomes.     

A case of post‐leptospirosis autoimmune epilepsy presenting with sleep‐related hypermotor seizures

This video‐illustrated case report concerns a 49‐year‐old woman who presented with sleep‐related hypermotor seizures. The antecedent history of leptospirosis, high frequency of new‐onset seizures, presence of an unclassified anti‐neuronal antibody, and dramatic response to steroids strongly supported post‐infectious immune‐mediated pathogenesis in our patient. To the best of our knowledge, post‐leptospirosis autoimmune epilepsy presenting as sleep‐related hypermotor seizures has not hitherto been reported. [Published with video sequence on www.epilepticdisorders.com ].     

125例颞叶癫手术远期疗效分析

目的评价颞叶癫手术的远期疗效。方法回顾性分析125例颞叶癫手术患者,术前、术后进行全面评估。结果本组125例无手术死亡及严重并发症,术后随访2～3a,疗效按Engel的标准评定,Engel’s效果分级：I级（术后无癫发作）81例（64.80%）,Ⅱ级（极少发作,1～2次/a）20例（16.00%）,Ⅲ级（发作频率减少75%以上）16例（12.80%）,Ⅳ级（发作频率减少75%以下）8例（6.40%）,总有效率93.6%。结论外科手术治疗颞叶癫是一种安全、有效的方法,疗效理想。     

Selection of adult patients for surgical treatment of epilepsy

ABSTRACT— The ideal treatment for epilepsy would produce complete control without toxicity and without side effects.
The physician would like to have surgical therapy as an option for those patients who are not completely free of seizures and for those who are free of seizures but suffer toxic side effects.
Surgery for epilepsy has now become an option that should be considered along with various medical treatment options. The success rates have increased and the complication rates decreased dramatically at those centers performing surgery on a substantial number of cases a year. Patients with complex partial seizures with a clear-cut unilateral temporal lobe focus and highly stereotyped seizures are by far the best candidates for surgery. In the hands of several major epilepsy programs significant improvement in seizure frequency is achieved in greater than 90 per cent of patients.
Patients with sudden drop attacks or uncontrolled generalized tonic-clonic seizures can benefit from section of the corpus callosum. Good results are seen in 85 per cent of the cases.     

Sudden unexpected death in epilepsy: people with nocturnal seizures may be at highest risk

Purpose: Most people with epilepsy who die suddenly and whose death is attributed to sudden unexpected death in epilepsy (SUDEP) are found in or by the bed for unknown reasons. We assessed whether those with sleep‐related SUDEP were more likely to have nocturnal seizures, and whether seizure patterns (diurnal vs. nocturnal) differed from people dying suddenly and living controls with epilepsy. Methods: Seizure patterns in a cohort of 154 people with epilepsy who died suddenly and after autopsy conformed to the definition of SUDEP and 616 controls living with epilepsy were classified as having “exclusively diurnal” or “nocturnal seizures.” Comparisons were made between the groups. SUDEP was classified as sleep‐related or non–sleep‐related based on eyewitness accounts and the circumstances surrounding death. Key Findings: SUDEP was primarily a sleep‐related (58%) and unwitnessed (86%) event. If sleep‐related, SUDEP was more likely to be unwitnessed [odds ratio (OR) 4.4, 95% confidence interval (CI) 1.6–12]. Those with sleep‐related SUDEP were more likely to have a history of nocturnal seizures than those who had non–sleep‐related SUDEP (OR 3.6, 95% CI 1.4–9.4). Those who died were more likely to have a history of nocturnal seizures than living controls (OR 3.9, 95% CI 2.5–6.0). After correction for previously established SUDEP risk factors ( Langan et al., 2005 ), the presence of nocturnal seizures remained significant (OR 2.6, 95% CI 1.3–5.0). Significance: Nocturnal seizures seem to be an independent risk factor for SUDEP. These findings underscore the importance of preventive measures, which may include night supervision.