Amusia and musicogenic epilepsy

Amusia and musicogenic epilepsy are clinical disorders that provide a window into the localization of music in the brain. Classic clinical studies of patients with these disorders, coupled with more recent studies employing modern neuroimaging and sophisticated neuropsychologic paradigms, have converged in helping to elucidate the complex neural systems that are utilized in decoding music. The notion of cerebral dominance for music has been replaced by a concept of modular but interconnected networks that have wide bilateral localization in the brain and that are molded both by genetics and experience. These disorders also provide insight into the important interface between music and emotion.     

A case of musicogenic epilepsy

1) A case of musicogenic epilepsy or psychomotor seizures supervening whenever the patient hears a certain tume has been presented. 2) The EEG features of the seizure are such as are often seen in psychomotor seizures. 3) Auditory evoked response may be left out of consideration. 4) There is no appreciable relationship between the disease and the life history of the patient. The attempt of our patient to cure herself by making use of conditioned reflex proved to be a failure. The patient is more liable to the disease when she is on the strain while hearing a tune. 5) Our consideration of the mechanism of the disease has led to the presumptive conclusion that hyperacusis, conditioned reflexes and the impact of life history may be involved, and the fragility of the memory function of the temporal lobe underlie the genesis of the disease.     

Surgical treatment for epilepsy

Nearly one-third of patients with newly diagnosed epilepsy will develop medically refractory seizure disorders. The initial response to antiepileptic drug therapy is highly predictive of long-term outcome. Patients with intractable epilepsy may have a progressive disorder that is medically, physically, and socially disabling. Surgical resection of the epileptogenic zone or lesional pathology, or both, may significantly reduce seizure tendency in selected patients. The present review supports the position that early and effective epilepsy surgery may not only render the patient with intractable partial epilepsy seizure-free, but also allow the individual to become a participating and productive member of society. Patients with surgically remediable epileptic syndromes should be identified early in the evaluation and treatment of their seizure disorders. Favorable candidates for focal cortical resection include individuals with medial temporal lobe epilepsy and partial seizures related to selected lesional pathology, e.g. primary brain tumor or vascular anomalies. In conclusion, surgical treatment of intractable partial epilepsy has been shown to compare favorably to antiepileptic drug therapy. Individuals rendered seizure-free may experience a significant improvement in quality of life. Patients who fail to respond to initial antiepileptic drug therapy should be “triaged” to a presurgical evaluation. Ictal semiology combined with structural magnetic resonance imaging and the electroclinical correlation may permit identification of candidates for early and effective surgical treatment.     

A young infant with musicogenic epilepsy

Musicogenic epilepsy is a relatively rare form of epilepsy. In its pure form, it is characterized by epileptic seizures that are provoked exclusively by listening to music. The usual type of seizure is partial complex or generalized tonic-clonic. Precipitating factors are quite specific, such as listening to only one composition or the actual playing of music on an instrument. However, simple sound also can be a trigger. We report a 6-month-old infant with musicogenic epilepsy. She manifested right-sided focal seizures with occasional generalization. The seizures were frequently triggered by loud music, especially that by the Beatles. The interictal electroencephalography results were normal. Ictal spikes were present throughout the left temporal area during continuous electroencephalograpic monitoring. Brain magnetic resonance imaging results were normal, whereas single-photon emission computed tomography of the brain revealed hypoperfusion of the left temporal area. The young age and epileptogenic left temporal lobe lesion in this patient with musicogenic epilepsy were unusual characteristics. Theoretically, three levels of integration are involved in music processing in the brain. The involved integration of this infant’s brain may be the sensory level rather than the emotional level. Nevertheless, the personal musicality and musical style of the Beatles might play an important role in this patient’s epilepsy.     

外伤后癫痫的外科治疗

目的评价并总结外伤后癫痫（PTE）手术治疗的方法及治疗情况,为PTE的手术治疗提供经验。方法回顾性分析2008年1月至2010年1月在我院行开颅手术治疗的PTE病例42例,总结这些病例的术前定位技术、手术治疗方法以及0.5～2年期的随访情况。结果手术结果EngelⅠ级17例,Ⅱ级22例,Ⅲ级3例,IV级0例,术后出现暂时性轻度偏瘫28例,一过性动眼神经麻痹2例,暂时性运动性失语3例,无死亡病例。结论 PTE发作形式多为部分性发作,一般影像学检查可发现明确病理改变,致痫灶多位于其附近,药物难以控制病例行手术治疗效果较好。     

继发性癫痫的手术治疗

继发性癫痫病因复杂．治疗方法和手段也不尽相同。自2000年7月至2006年5月我们对37例继发性癫痫患者根据病因不同进行不同手术治疗．取得了较好的疗效，现报告如下。     

fMRI of triggerable aurae in musicogenic epilepsy

The authors studied a patient with musicogenic epilepsy triggered by one specific musical piece using 3D PRESTO fMRI. During epileptic aurae initiated by the stimulus, signal increases were found in the left anterior temporal lobe, correlating with ictal EEG and SPECT showing a left anterior temporal focus, and the right gyrus rectus. Because fMRI indicated a cascade of recruitment of the ventral frontal lobes by epileptogenic music, left anterior temporal lobe activity could be secondary to a right gyrus rectus focus, possibly triggered by emotional processing of music.     

额叶癫痫的手术治疗

目的总结并分析顽固性额叶癫痫患者手术治疗的效果、手术方式和经验。方法回顾性分析2003年3月至2009年4月在我院接受手术治疗的116例顽固性额叶癫痫患者的临床及脑电图特征、手术方式和疗效。结果随访1～6年,平均2.6年。疗效根据Engel的标准进行评定,其中I级（癫痫发作消失）49例（42.2%）;Ⅱ级（癫痫发作频率减少≥90%）36例（31.0%）;Ⅲ级（癫痫发作频率减少≥75%）19例（16.4%）;Ⅳ级（癫痫发作频率减少〈75%或与术前相近）12例（10.3%）。本组总有效（Ⅰ、Ⅱ、Ⅲ级）率为89.7%;效果优良（Ⅰ、Ⅱ级）率为73.3%。此外,患者术前所伴有的精神和行为异常在术后多数患者有所改善。术后无严重并发症及手术死亡。结论对于顽固性额叶癫痫,准确定位原发致痫灶并采用合理的手术方式彻底处理痫灶是手术成功的关键。必要时应采用颅内埋藏电极来寻找原发致痫灶。     

颞叶癫痫的手术治疗(附34例分析)

目的　探讨颞叶癫痫的术前评估和术式选择策略。方法　对 34例颞叶癫痫患者应用无创和有创方法进行术前综合评估 ,采用扩大额颞问号式手术切口 ,经外侧裂入路、皮层脑电监测引导下实施手术。对术前评估方法、术中脑电监测的意义和手术方式选择进行了分析。结果　 2 1例患者根据长程蝶骨电极脑电图和MRI等无创检查定位了致痫灶 ,13例根据颅内埋置电极脑电图定位了致痫灶。术后随访 15 .6± 6 .0月。 2 9例 (85 .3% )癫痫发作消失 ,3例 (8.8% )发作减少 75 % ,2例 (5 .9% )发作减少 5 0 %以上。 8例曾出现短期并发症 ,无永久性并发症发生。手术 6个月后 ,患者的日常生活能力评分与手术前相比明显改善 (P <0 .0 5 )。结论　长程视频脑电图和MRI检查是颞叶癫痫致痫灶定位可靠的无创性检查方法 ;颅内埋置电极检查是术前准确定位致痫灶必要的手段。颅内电极脑电图监测对设计切除方式有重要参考价值 ;改良的手术切口和经外侧裂入路有利于安全有效的切除前颞叶或选择性切除颞叶内侧结构     

Surgical treatment for intractable epilepsy]

Epilepsy surgery can be divided into two categories, resective and disconnective procedures. The former includes lesionectomy, corticectomy, and lobectomy. The latter comprises MST (multiple subpial transection), corpus callosotomy, and hemispherotomy. In this presentation, the preoperative diagnosis and surgical outcomes of temporal lobectomy and MST will be illustrated. Temporal lobe epilepsy is one of the most common seizures in adult intractable epilepsy. Noninvasive preoperative evaluation, including analysis of seizure semiology, repetitive scalp EEG, and MR imaging, can definitely localize the seizure focus, without depending on invasive monitoring, in 70% of the cases. Seizure outcome after temporal lobectomy is generally satisfactory, with 70% seizure-free and > 90% significantly improved. However, verbal amnesia is an unavoidable sequela when the focus is on the speech-dominant side and preoperative MRI reveals little or no hippocampal atrophy. MST is an epoch-making surgical technique by which surgical treatment of eloquent cortex has become possible. In cases with neocortical epilepsy treated by MST alone or combined with corticectomy, 80% showed significant improvement, that is compatible with reported outcomes of corticectomy. MST can be also applied to treatments of extensive epileptic foci, Rasmussen's encephalitis, or Landau-Kleffner syndrome.     

New‐onset musicogenic epilepsy after temporal lobe epilepsy surgery

Musicogenic epilepsy is a reflex epilepsy provoked by listening to or playing music. The epileptogenic network involves temporal regions, usually mesiotemporal structures. We present a 31‐year‐old female patient who experienced musicogenic seizures after a right temporal lobectomy with amygdalohippocampectomy that was performed in order to treat preexisting right mesio‐temporal epilepsy.     

Surgical treatment of epilepsy

Approximately 30-40% of patients with focal epilepsy continue to have seizures despite appropriate medical therapy. Surgical treatments should be considered in this important subset of patients. Recent advances in neuroimaging technology have revolutionized the identification and evaluation of surgical candidates. The goal of the presurgical evaluation (video EEG monitoring, neuroimaging, and neuropsychological assessment) is to delineate the epileptogenic zone. Surgery is recommended when this has been adequately identified and the proposed procedure is expected to result in a high likelihood of seizure freedom and a low risk of neurologic and cognitive morbidity.     

Surgical treatment of epilepsy

In this article the possibilities, indications, methods and results of surgery in epilepsy are summarized in general with the Hungarian experience emphasized. Surgery may provide effective treatment in about 5-10% of the epileptic population. Surgical solution nowadays became an essential treatment in medial temporal epilepsy, if hippocampal sclerosis or other lesion is present, in therapy resistant lesional extratemporal epilepsies and in catastrophic childhood epilepsies if the epileptic disorder is restricted to one hemisphere (Rasmussen syndrome, hemimegalencephaly, Sturge-Weber disease and posttraumatic or postencephalitic hemispherial epilepsies). The algorithms of the presurgical evaluation and the current methods for study the pacemaker area, forbidden zones, and hemispherial functions are treated. The currently used type and techniques of surgery, such as lesionectomy, temporal lobe resections, hemispherotomy, callosotomy, multiple subpial transsections and their indications are described. The newest surgical approaches, as deep brain stimulation, vagal nerve stimulation, and irradiation techniques are also briefly touched. Lastly, we deal with prognostical factors of the surgical outcome, reasons of surgical failures and complications. In a brief chapter the importance of postsurgical rehabilitation is emphasized.     

Neural correlates of musicogenic epilepsy: SISCOM and FDG-PET

To localize the neural correlates of musicogenic epilepsy, subtraction ictal SPECT coregistered with MRI (SISCOM) and (18)F-fluorodeoxy glucose positron emission tomography (FDG-PET) were performed in a woman who had suffered from frequent musicogenic seizures. She had complex partial seizures consisting of palpitation and an unpleasant feeling, which were followed by staring and oroalimentary automatisms. Ictal EEG showed rhythmic theta waves originated from the right temporal lobe, and SISCOM showed ictal hyperperfusion on right insula, amygdala, hippocampal head, and anterior temporal lobe, whereas interictal FDG-PET showed interictal hypometabolism in the same brain regions, suggesting dysfunction and abnormal activation of right temporo-limbic structures related to an emotional response to music.     

Surgical treatment for extratemporal lobe epilepsy]

We analyzed the seizure outcome of 140 patients who underwent resective surgeries of extratemporal lobes including multilobar resection and hemispherectomy. The patients were followed for 2.0 to 16.7 years after surgery (mean, 6.2 years). The overall seizure outcome was Engel's class I in 87 patients (62%), class II in 14 (10%), class III in 13 (9%), and class IV in 26 (19%). The proportion of class I cases was 76% in 71 patients with discrete lesions under 5 cm in diameter on MRI, whereas the proportion was 52% in 46 patients with widespread lesions over 5 cm, and 39% in 23 patients with no MRI-detectable lesion. The seizure-free rates of extratemporal lobe resection was slightly lower compared with temporal lobe resection. Even in patients with extratemporal lobe epilepsies, clinicians should consider the option of surgical intervention from the early stage of disease.     

神经节细胞胶质瘤相关癫痫的外科治疗

目的 探讨神经节细胞胶质瘤（GG）相关癫痫手术治疗方法及效果。方法 2015年1月至2018年12月前瞻性收集GG相关癫痫共21例。根据癫痫病史、神经心理评估、长程视频脑电监测、头颅MRI及PET-CT等多模态Ⅰ期评估,15例在术中皮层脑电图（ECoG）监测下行裁剪式切除,6例行长程视频立体定向脑电图（SEEG）颅内深部电极植入术Ⅱ期评估后再行裁剪式切除术。结果 21例中,肿瘤等同于癫痫灶14例,肿瘤及边缘组织共为致痫灶5例,肿瘤周边组织为致痫灶2例。21例术后病理均为GG,合并局灶性皮质发育不良Ⅲb型7例（6例位于肿瘤边缘,1例位于肿瘤后缘2.6 cm处）。术后随访6~42个月,中位数为24个月,Engel分级Ⅰ级20例,Ⅱ级1例。结论 GG相关癫痫,肿瘤并不等同致痫灶。正确认识肿瘤与致痫灶的关系,致痫灶并肿瘤切除术是有效的手术方法。