Evolution of concepts in epilepsy surgery.

At the time of the first meeting of the International League Against Epilepsy (ILAE) in 1909, surgical treatment for epilepsy had been accepted as an alternative therapy for over two decades, but was rarely practiced, considered a last resort for carefully selected patients. Localization was based on ictal semiology and identification of a structural lesion. Very few papers on epilepsy surgery were presented at ILAE meetings or published in Epilepsia during the first half of the 20th century. A modest explosion in interest in epilepsy surgery at mid-century resulted from recognition that "invisible" epileptogenic lesions could be identified by EEG, especially for temporal lobe epilepsy. Epilepsy surgery received a second boost in popularity toward the end of the 20th century with the advent of structural and functional neuroimaging, and the number of epilepsy centers worldwide doubled between the first Palm Desert conference in 1986 and the second Palm Desert conference in 1992. Neuroimaging also helped to increase application of surgical treatment to infants and young children with severe epilepsies. Epilepsy surgery was accepted as standard of care for drug-resistant focal epilepsy and was well-represented at international ILAE congresses and in Epilepsia. Advances continue into the 21st century with the introduction of laser ablation, and palliative neuromodulation approaches, which have greatly increased the population of patients who can benefit from surgery. Modern presurgical evaluation techniques have also made surgical treatment possible in many countries with limited resources. Three randomized control trials now have definitively proved the safety and efficacy of epilepsy surgery, however, this alternative therapy remains under-utilized even in the industrialized world, where less than 1% of potential candidates are being referred to epilepsy centers. Furthermore, those who are referred receive surgery an average of 20 years after onset of epilepsy, often too late to avoid irreversible disability. The major challenges in realizing the full potential of epilepsy surgery, therefore, are not as much in the continued improvement of the treatment itself, as they are in addressing the treatment gap that is preventing appropriate patients from being referred to full-service epilepsy centers.

A neurosurgeon`s view: Laser interstitial thermal therapy of mesial temporal lobe structures.

Stereotactic laser ablation of mesial temporal structures is a promising new surgical intervention for patients with mesial temporal lobe epilepsy (MTLE). Since this procedure was first used to treat MTLE in 2010, the literature contains reports of 37 patients that underwent MR-guided stereotactic laser amygdalohippocampotomy (SLAH) using Laser Interstitial Thermal Therapy (LITT) with at least 1year of follow-up. This early body of data suggests that SLAH is a safe and effective treatment for MTLE in properly selected patients. Moreover, SLAH is substantially less invasive when compared with open surgical procedures including standard anterior temporal lobectomy and its more selective variants, results in immediate destruction of tissue in contrast to radiosurgical treatments for MTLE, and can more readily ablate larger volumes of tissue than is possible with techniques employing radiofrequency ablation. Finally, evidence is accruing that SLAH is associated with lower overall risk of neuropsychological deficits compared to open surgery. Thus, LITT constitutes a novel minimally invasive tool in the neurosurgeon's armamentarium for managing medically refractory seizures that may draw eligible patients to consider surgical interventions to manage their seizures.

Scott Fitzgerald: famous writer, alcoholism and probable epilepsy / Scott Fitzgerald: famoso escritor, alcoolismo e provável epilepsia

ABSTRACT Scott Fitzgerald, a world-renowned American writer, suffered from various health problems, particularly alcohol dependence, and died suddenly at the age of 44. According to descriptions in A Moveable Feast, by Ernest Hemingway, Fitzgerald had episodes resembling complex partial seizures, raising the possibility of temporal lobe epilepsy.

RESUMO Scott Fitzgerald, reconhecido escritor americano, mundialmente famoso, apresentou vários problemas de saúde, particularmente a sua dependência ao álcool, tendo morte súbita aos 44 anos de idade. Relatos publicados no livro de Ernest Hemingway “Paris é uma festa” dão conta de que Scott Fitzgerald apresenta prováveis crises parciais complexas, sugerindo a hipótese de epilepsia do lobo temporal.

H.M. never again! An analysis of H.M.'s epilepsy and treatment.

On August 25, 1953, the patient H.M., aged 27, underwent a bilateral surgical destruction of the inner aspect of his temporal lobes performed by William Beecher Scoville with the aim to control H.M.'s drug refractory epileptic seizures and alleviate their impact on his quality of life. Postoperatively, H.M. presented for 55 years a "striking and totally unexpected grave loss of recent memories". This paper reports what we know about H.M.'s epilepsy before and after surgery and puts forward arguments supporting the syndromic classification of his epilepsy. We attempted to elucidate what could have been the rationale, in 1953, of Scoville's decision to carry out a bilateral ablation of H.M.'s medial temporal lobe structures, and we examined whether there was any convincing argument published before 1953 suggesting that bilateral hippocampal ablation could result in a permanent and severe amnesia. Our a posteriori analysis of H.M.'s medical history suggested that he was most probably suffering from idiopathic generalized epilepsy with absences and generalized convulsive seizures worsened by high dosage phenytoin treatment, or less probably from cryptogenic frontal lobe epilepsy. Importantly, he did not have temporal lobe epilepsy. Scoville based his proposal of bilateral mesial temporal lobe ablation on his experience as a psychosurgeon and on the assumption that the threshold of generalized epileptic activity could be lowered by some kind of hippocampal dysfunction potentially epileptic in nature. Given the scanty information on the link between amnesia and medial temporal lobe lesions that was available in humans in 1953, one can understand why Scoville was so surprised by the "striking and totally unexpected" memory loss he observed in H.M. after the bilateral ablation of his mesial temporal lobe structures.

Frederic Gibbs and his contributions to epilepsy surgery and electroencephalography.

Frederic Gibbs' (1903-1992) long research career was devoted to the understanding and treatment of epileptic phenomena and closely associated with the development of electroencephalography (EEG). After medical school, he joined the Harvard Neurological Unit at Boston City Hospital directed by Stanley Cobb. In the early 1930s, Gibbs developed a thermoelectric blood flow probe and, with William Lennox, proved in animals and humans that a seizure increases cerebral blood flow. By 1934, Gibbs became a pioneer in the field of EEG while working at Harvard with Hallowell Davis and Lennox, and was the first to convincingly record and report EEG findings in epilepsy and states of altered consciousness. Several years later, Gibbs and Lennox were the first to recommend cerebral excisions in several patients with uncontrolled epilepsy based on EEG. Moving to the University of Illinois at Chicago in 1944, Gibbs founded a consultation clinic for epilepsy, performed the first EEG depth recordings using pneumoencephalography-guided stereotaxy, and noted that sleep EEGs in patients with psychomotor seizures frequently disclosed temporal epileptic patterns. Gibbs convinced Percival Bailey to collaborate on patients with refractory temporal lobe psychomotor seizures without tumors. In 1947, the first nonlesional temporal lobe excisions based on EEG localization were performed in these patients, and, by 1948, anterior temporal lobectomy had become their procedure of choice. Gibbs and Lennox received the coveted Lasker Award among other honors as pioneers in establishing the modern era of epilepsy diagnosis and treatment.

Hippocampal sclerosis: progress since Sommer.

Hippocampal sclerosis (HS) continues to be the most common pathology identified in patients with refractory temporal lobe epilepsy undergoing surgery. Wilhelm Sommer described this characteristic pattern of neuronal loss over 120 years ago through his post-mortem studies on patients with epilepsy. Neuropathological post-mortem studies in the 20th century proceeded to contribute significantly to the understanding of this disease process, with regard to the varying patterns of HS and involvement of adjacent limbic structures. From studies of surgical temporal lobe specimens from the 1950s onwards it was recognized that an early cerebral injury could act as the precipitant for the sclerosis and epilepsy. Modern neuropathological studies have focused on aspects of neuronal injury, loss of specific neuronal groups and cellular reorganization to address mechanisms of epileptogenesis and the enigma of how specific hippocampal neuronal vulnerabilities and glial proliferation are both the effect and the cause of seizures.

From lateral to mesial: the quest for a surgical cure for temporal lobe epilepsy.

PURPOSE: A gap of more than a hundred years occurred between the first accounts of mesial temporal sclerosis and recognition of its role in the pathogenesis of psychomotor seizures. This paper reviews how the understanding and surgical treatment of temporal lobe epilepsy developed, particularly from the work of Penfield, Jasper, and their associates at the Montreal Neurological Institute (MNI). METHODS: Publications on EEG and surgery for temporal lobe seizures from 1935 to 1953 were reviewed and charts of selected patients operated on at the MNI in the same period were examined. Attention was focused on the evolution of surgical techniques for temporal lobe epilepsy. RESULTS: In the late 1930s, some EEG findings suggested deep-lying disturbances originating in the temporal lobe. However, it took another two decades before the correlation of clinical, neurophysiological, and anatomical findings provided evidence for the involvement of the mesial structures in psychomotor or temporal lobe seizures. From 1949 and onward, Penfield and his associates applied this evidence to extend the surgical resections to include the uncus and the hippocampus. CONCLUSION: The collaborative work of a team led by Penfield and Jasper at the MNI helped to define the role of neurophysiological studies in epilepsy surgery. As a result, the importance of removing the mesial structures in order to obtain better seizure control in patients with temporal lobe epilepsy became firmly established.

Ivan IV, el Terrible, ¿padeció de un síndrome del lóbulo temporal? / The possible temporal lobe syndrome of Ivan IV the Terrible

Ivan IV "The Terrible" was the first Tsar of all Russias and was crowned in 1547. He extended Russian territories and opened the route to Siberia in successful campaigns against Tartars. He increased his personal power to the point of generating an autocracy that isolated him progressively from the council of Boyars. He had a complex personality and his acts were impregnated by a profound religiousness, episodes of rage, mood changes and a sense of "personal fate". All these traits configure the controversial "temporal lobe personality". The possible association between these personality traits and the eventual epilepsy that Ivan IV could suffer, is discussed. This association is called "temporal lobe syndrome". Considering the mood changes, with severe irritability and episodes of control loss alternated with feelings of guilt, sadness and isolation, another possibility is that the Tsar had an affective bipolar disorder or, less probably, a personality disorder.

Hughlings Jackson and the role of the entorhinal cortex in temporal lobe epilepsy: from patient A to Doctor Z.

Hughlings Jackson's insightful bedside observations of patients with epilepsy paved the way for the first effective surgical epilepsy treatments. Jackson's most famous case, that of Doctor Z, concerned a medical doctor with partial complex seizures who was reported to have a discrete and circumscribed medial temporal lobe (mTL) lesion on autopsy. Although integral to Jackson's argument for mTL resection, the case remains controversial due to inadequate pathological descriptions of Doctor Z's lesion. This motivated us to describe the case of a patient, whom we call Patient A, who suffered from a form of epilepsy similar to that of Doctor Z, accompanied by a discrete and circumscribed mTL lesion in the exact same location. The lesion, a cavernous hemangioma, spared the hippocampus and was restricted to the lateral aspect of the entorhinal cortex. This finding validates Jackson's original description and suggests that the entorhinal cortex can play a role in seizure genesis.

Príncipe Liev Nikoláievitch Míchkin ("O Idiota", Fiódor Dostoevsky) e a síndrome de personalidade interictal na epilepsia do lobo temporal / Prince Liev Nikoláievitch Míchkin (

A obra do romancista russo Fiódor Dostoevsky (1821-1881), além de seu extraordinário valor literário, reveste-se de importância especial para neurologistas e epileptologistas. O escritor, que era portador da enfermidade, transmitiu através de seus textos o universo do epiléptico e a maneira como esse é percebido pela sociedade. Seus livros tiveram grande influência na maneira como a cultura ocidental percebe a doença. O romance "O Idiota" tem como protagonista o Príncipe Liev Nikoláievitch Míchkin, um epiléptico com personalidade marcante. Considerando a proposta feita por Geschwind-Waxman (1975) de síndrome de personalidade interictal na epilepsia do lobo temporal, este artigo pretende discutir as alterações comportamentais no paciente epiléptico a partir de Míchkin, personagem principal de "O Idiota", de Fiódor Dostoevsky.

[The ecstatic epilepsy of Teresa of Jesus]. / La epilepsia extática de Teresa de Jesús.

AIMS: Our aim is to define the ecstasies (raptures) of Teresa de Ahumada (Teresa of Jesus) and to link them with ecstasies recently identified as convulsive seizures (Dostoevsky s epilepsy, Saint Paul s disease, ecstatic epilepsy). DEVELOPMENT AND CONCLUSIONS: We review the Carmelite nun's pathography, with special attention given to the excellent detailed descriptions of her ecstasies. The stereotypic movements and comparison with similar cases leads us to consider them as being epileptic, possibly secondary to neurocysticercosis.

The uncinated crisis of George Gershwin.

George Gershwin, renowned composer and pianist, well known for his popular works, died on the 11th July 1937 due to a brain tumor. His neurological symptoms first appeared on that same year, in February, with a simple olfactory partial seizure, characterized by an unpleasant smell of burnt rubber (uncinated seizure). He later had a quick clinical descend, with severe headache that occurred in bouts, dizziness, coordination compromise and olfactory seizures, eventually lapsing into a coma on the 9th July 1937. It was then that a gliomatosus cyst was diagnosed, which on microscopic examination proved to be a "glioblastoma multiforme". Despite the surgical intervention, Gershwin died soon after the procedure without recovering his consciousness. We make a brief review of Gershwin's neurologic disease, with emphasis on the initial symptoms, namely the uncinated seizures.

Amnesia in temporal lobectomy patients: historical perspective and review.

Profound memory loss is a rare but serious complication of temporal-lobe surgery for the relief of medically intractable epilepsy. This paper examines the characteristics of the patients who have been reported to become amnesic following temporal-lobe surgery over the last four decades. The critical role of the hippocampi in memory function are implicated in autopsy studies and MRI investigations, but these cases suggest that a range of memory impairments result from bilateral hippocampal damage, rather than a pure amnesic syndrome in every case. There is some evidence that bilateral structural hippocampal abnormalities may not necessarily be associated with significant memory problems, if these abnormalities have a developmental basis. However, whilst not necessarily profound, any post-operative deterioration in memory function remains a significant consideration in the presurgical evaluation of temporal-lobe epilepsy patients.

Tratamiento quirúrgico de la epilepsia del lóbulo temporal / Surgical treatment of temporal lobe epilepsy

La epilepsia del lóbulo temporal (ELT) refractaria a tratamiento médico corresponde aproximadamente al 75 porciento del total de las cirugías de la epilepsia. La cirugía de la epilepsia se realiza sólo en pacientes refractarios a tratamiento médico con dósis máximas de drogas anti-epilépticas óptimas. El éxito de la cirugía depende del diagnóstico pre-quirúrgico. Este se basa en la historia clínica, el exámen neuropsicológico, el test de Wada, y las neuro-imagénes (básicamente resonáncia magnética). Así puede lograrce la localización precisa del foco epileptógeno y muchas veces se detectan lesiones acompañantes insospechadas. Lesiónes orgánicas tales como tumores de bajo grado de malignidad, fallas del desarrollo embrionario y esclerosis del hipocampo se asocian a epilepsia temporal en hasta el 90 porciento de los casos. El tipo de cirugía a realizar dependerá de la ubicación del foco epileptógeno, la presencia o no de una lesión asociada y la relación de estos dos factores con las áreas cerebrales elocuentes. Las intervenciones pueden ser: resecciones standard que incluyen los 2/3 anteriores del polo temporal más amígdalo-hipocampectomía, resecciones témporo-laterales sin inclusión de las estructuras témporo-mesiales, amígdalo hipocampectomía selectiva o diferentes variaciones de las técnicas anteriores, tales como las resecciones temporales a medida, las cuales son hoy las más utilizadas. La cirugía logra que hasta un 90 porciento de los pacientes queden libres o con una significativa reducción de sus crisis y un 10 porciento permanece sin cambios. Las complicaciones quirúrgicas son actualmente muy bajas, ellas se dividen en complicaciones generales a todo acto quirúrgico (infección, hemorragia, etc.) y complicaciones neurológicas directamente relacionadas con el tipo de cirugía realizada. Este artículo revisa las actuales indicaciones y técnicas para cada tipo de cirugía en ELT

Epilepsia del lóbulo temporal: alternativas quirúrgicas / Temporal lobe epilepsy: surgical alternatives

La epilepsia del lóbulo temporal (ELT) refractaria a tratamiento médico corresponde aproximadamente al 75 por ciento del total de las cirugías de la epilepsia. El tipo de cirugía a realizar dependerá de la ubicación del foco epileptógeno, la presencia o no de una lesión asociada y la relación de estos 2 factores con las áreas cerebrales elocuentes. Las intervenciones pueden ser: resecciones standard que incluyen los 2-3 anteriores del polo temporal más amigdalohipocampectomía, resecciones témporo-laterales sin inclusión de las estructuras témporo-mesiales, amigdalo hipocampéctomía selectiva o diferentes variaciones de las técnicas anteriores. tales como las resecciones temporales a medida, las cuales son hoy las más utilizadas. Las complicaciones quirúrgicas son actualmente muy bajas, ella se divide en complicaciones generales a todo acto quirúrgico (infección, hemorragia, etc.) y complicaciones neurológicas directamente relacionadas con el tipo de cirugía realizada. Las reoperaciones a causa de falla en el control de la epilepsia son infrecuentes y se realizan en pacientes que han sido sometidos previamente a una resección témporo-lateral y que posteriormente, al persistir las crisis, se ha requerido ir a resecar las estructuras témporo mesiales. Este artículo revisa las actuales indicaciones y técnicas para cada tipo de cirugía en ELT

Epilepsia do lobo temporal: um ensaio histórico e clínico / Temporal lobe epilepsy: a historical and clinical essay

Grande parte do avanço neurocientífico dos últimos anos pode ser caracterizado como conceitual, metodológico e tecnológico. No campo específico da epileptologia clínica, encontram-se exemplos claros destes ramos do progresso. O presente estudo faz ensaio histórico e clínico da epilepsia do lobo temporal como exemplo do resultado da aplicaçäo precisa de conceitos clínicos, metodologia cientifíca e tecnologia a uma questäo multidisciplinar