Correction to: 40th EUROPEAN SOCIETY OF NEURORADIOLOGY Diagnostic and Interventional Annual Meeting.

We note that the following data error was published in regard to patient numbers in the Results section 1.

Pascual-Castroviejo type II syndrome (P-CIIS). Importance of the presence of persistent embryonic arteries.

INTRODUCTION: Cutaneous hemangioma and vascular malformation are two vascular abnormalities frequently associated with absence or hypoplasia of one or both carotid and/or vertebral arteries, presence of persistent embryonic arteries, especially the trigeminal, cerebellar malformations, and coarctation of the aortic arch and/or congenital cardiopathy. This disease is known as Pascual-Castroviejo type II syndrome (P-CIIS) and by the acronym PHACE. MATERIAL AND METHODS: Three patients (two females and one male) with facial hemangioma are studied during the first years of age by magnetic resonance angiography (MRA) and their vascular evolution to adult age followed through several MRA controls. RESULTS: All the three patients showed persistence of the trigeminal artery associated to other intra- and extracranial vascular abnormalities of type hemangioma or hemangiomatous arteries that presented progressive involution with decreased arterial caliber without appearing cerebrovascular stroke or hypoxic zones because, at the same time, collateral vascularization appeared through connections between the embryonic arteries and the peripheral branches of the internal carotids or connections between branches of the external and internal carotids. Only one patient had obstruction of a branch of the left middle cerebral artery after 3 days, with gastroenteritis with elevated fever at 17 months of life that caused parenchymal infarct in the left cerebral region supplied by the obstructed artery. CONCLUSIONS: The presence of embryonic arteries, especially the trigeminal, and connections between branches of the internal and external carotids, mainly through the internal maxillary and ophthalmic arteries, ensure the cerebral supply in the P-CIIS despite the progressive involvement of the cerebral arteries.

Posterior fossa tumors in children with neurofibromatosis type 1 (NF1).

BACKGROUND: Tumours of the posterior fossa associated with neurofibromatosis type 1 (NF1) are very infrequent. Series studying this association are seldom reported. PERSONAL EXPERIENCE: In a series of 600 NF1 patients studied during 39 years (1965-2004) only five (0.83%) had posterior fossa tumours. They were studied clinically, radiologically by computerized tomography (CT) or magnetic resonance (MR) and histologically. Four of them had astrocytomas but only in one case was the tumour primarily cerebellar while the tumour was primarily of the brain stem with invasion of the adjacent regions of one or both cerebellar hemispheres in three patients. The fifth tumour was a medulloblastoma that had a survival of 3 years following treatment. The patient with primary cerebellar astrocytoma is apparently cured 7 years after the removal of the tumour. The patients with the brain stem tumours extending to the cerebellum, showed a chronic slowly progressive cerebellar disease, but remain alive at age of more than 20 years (one was lost to follow-up). DISCUSSION AND CONCLUSION: The aim of this study was to present five children (one male and four females) less than 16 years of age when they were initially seen in our service, who had NF1 associates with posterior fossa tumours. This location is very uncommon in patients with NF1, in contrast with those located in other regions, such as pathway optic tumours and brain stem tumours. Most of these tumours are histologically benign (low grade astrocytomas). Only one patient in this series had a medulloblastoma, an exceptionally rare tumour seldom reported in patients with NF1.

Sturge-Weber syndrome: study of 55 patients.

PURPOSE: To review the clinical and neuroimaging features of a large series of patients with Sturge-Weber syndrome (SWS) seen over a 40-year period. METHODS: Fifty-five patients with SWS (30 males and 25 females), were studied between 1965 and 2004. Results of neurological and ophthalmological examinations, electroencephalographic, and neuroimaging studies were reviewed. All patients were seen by one of the authors (I. P-C). RESULTS: Epilepsy, hemiparesis, mental retardation and ocular problems were the most frequent and severe features of patients with Sturge-Weber syndrome in this series. The facial nevus flammeus was unilateral in 35 (63.5%) patients, bilateral in 17 (31%) and absent in 3 (5.5%) of the patients with leptomeningeal angiomas. Seven (41%) of the 17 patients with bilateral nevus flammeus had unilateral leptomeningeal angiomas. Seizures occurred in 47 patients (85.5%). Complete seizure control was obtained in 20 patients (42.5%), but in 2 of these 20 patients seizures were controlled only after lobectomy. All patients with unilateral or bilateral upper eyelid nevus flammeus had ipsilateral, unilateral or bilateral choroid-retinal angiomas. Only 20 (36%) of the 55 patients had low-normal or borderline intelligence (IQs < 70). No relationship was observed between the size of the facial nevus flammeus and the severity of the brain lesion. CONCLUSIONS: Epilepsy, hemiparesis, mental retardation and ocular problems were the most frequent and severe features of patients with Sturge-Weber syndrome in this series. Cerebral lesions followed a progressive course during early childhood, but not later. Early surgical treatment controlled the seizures but other neurological problems such as hemiparesis and intellectual deficits showed a less satisfactory response. Early onset of seizures and poor response to medical treatment, bilateral cerebral involvement and unilateral severe lesions were indicative of a poor prognosis. Limited intelligence and social skills, poor aesthetic appearance and seizures complicated the integration of SWS patients. These features must be addressed in order for the patients improve social interactions, obtain gainful employment and achieve a better quality of life.

Diplegia due to transcranial knife-blade injury in a 20-month-old child.

Transcranial stab wounds are uncommon among both adults and adolescents and rarely occur in children, particularly when caused by another child. A 20-month-old girl was injured by a 3-year-old cousin, who introduced a knife blade into the brain through the left parietal region. The trajectory of the wound penetrated at least 5 cm, crossed the falx cerebri, and involved both motor cortical areas. The clinical sequela was a severe symmetric spastic diplegia.

Termocoagulación por radiofrecuencia de ganglio de Gasser para el tratamiento de neuralgia trigeminal, resultados de 11 años / Gasser's ganglion radiofrequency thermocoagulation for the treatment of trigeminal neuralgia, 11-year results

Objetivos: Describir resultados de los últimos 11 años en el tratamiento de neuralgia del trigémino con termocoagulación por radiofrecuencia, analizar variables relacionadas a complicaciones y resultados. Material y Métodos: Estudio retrospectivo, descriptivo, longitudinal, comparativo y analítico. Se analizaron los resultados de los últimos 11 años de nuestro servicio evaluando las temperaturas de las lesiones armando dos grupos, de 65°C-70°C y 71°C-75°C para analizar su relación con resultados y complicaciones. Resultados: Se trataron 59 pacientes en los cuales se realizaron 74 procedimientos, la edad media fue 59.22 años (±13,45). Se observó recidiva en 23 procedimientos con una tasa global de 31%. El tiempo medio de recidiva fue de 28,19 meses (±26,21). El tiempo medio de seguimiento fue de 33,10 meses (±33,49). El tiempo medio de evolución del dolor, previo al primer procedimiento, fue de 5,35 años (±4,37). Analizando los grupos se observó que no existía relación significativamente estadística (p = 0,74) entre el grupo de pacientes de 65ºC-70ºC y el grupo de 71ºC-75ºC y recidiva. No se observó relación estadísticamente significativa entre el grupo de 65ºC-70ºC y el grupo de 71ºC-75ºC y tiempo de recidiva (p=0,12). Se observó más pacientes con hipoestesia inmediata en el grupo de pacientes de 65ºC-70ºC, sin significación estadística (p=0,47). Conclusión: La termocoagulación por radiofrecuencia de ganglio de Gasser es un procedimiento accesible, mínimamente invasivo que demostró buenos resultados y buen manejo del dolor con bajo índice de complicaciones.

Objectives: Describe results of the last 11 years in the treatment of trigeminal neuralgia with radiofrequency thermocoagulation, analyze variables related to complications and results. Methods: Retrospective, descriptive, longitudinal, comparative and analytical study. The results of the last 11 years of our service were analyzed by assessing the temperatures of the lesions by assembling two groups, 65° C-70° C and 71 ° C-75° C to analyze their relationship with results and complications. Results: 59 patients were treated in which 74 procedures were performed; the mean age was 59.22 years (± 13.45). Recurrence was observed in 23 procedures with an overall rate of 31%. The average recurrence time was 28.19 months (± 26.21). The average follow-up time was 33.10 months (± 33.49). The average time of pain evolution, prior to the first procedure, was 5.35 years (± 4.37). Analyzing the groups, it was observed that there was no significant statistical relationship (p = 0.74) between the group of patients from 65ºC-70ºC and the group from 71ºC-75ºC and recurrence. No statistically significant relationship was observed between the 65ºC-70ºC group and the 71ºC-75ºC group and recurrence time (p = 0.12). More patients with immediate hypoaesthesia were observed in the group of patients from 65ºC-70ºC, without statistical significance (p = 0.47). Conclusion: Gasser's ganglion radiofrequency thermocoagulation is an accessible, minimally invasive procedure that demonstrated good results and good pain management with a low complication rate

Cobb syndrome: case report.

We present a 7-year-old boy in whom cutaneous hemangioma and intramedullary and paraspinal arteriovenous malformations were associated. Magnetic resonance arteriography revealed the presence of the two arterovenous malformations, and the selective intercostal arteriography demonstrated that the intraspinal and paraspinal arteriovenous malformations were supplied by the same intercostal arteries. Rubbing the back was required to detect the cutaneous changes, which were only suspected by casual inspection.

[Voluminous plexiform neurofibromas of the neck region in neurofibromatosis 1]. / Neurofibromas plexiformes voluminosos de cuello en la neurofibromatosis tipo 1.

AIM. To present the clinic, imaging and evolutive characteristics of a series of patients with neurofibromatosis 1 with voluminous plexiform neurofibromas in the neck (VPNFN) during childhood. PATIENTS AND METHODS. Nine patients (five females and four males) who were diagnosed as VPNFN at ages between 3 and 15 years. The VPNFN widespread to the posterior fossa or the upper thoracic region in some cases. The diagnosis was based on the clinical, imaging and histological findings. RESULTS. One of the tumors was intralaryngeal and caused respiratory difficulties. The other eight patients had the origin of the tumor in several spinal roots of one or both sides and could growth to the posterior fossa and to the upper thoracic region in some cases with displacement of the surrounding organs, especially in three patients, all girls, in whom the tumor reached a voluminous size on one side, that was observed only until 10 to 11 years when the growth ceased. CONCLUSIONS. The VPNFN are histologically benign tumors. Those located in the larynx must be removed because of the respiratory problems, but it is not necessary in cases with other locations despite the voluminous size that can reach in some patients with great displacement of the surrounding organs. The analysis of the results of our series may demonstrate that al least the extralaryngeal tumors only grow to 11-12 years of age. This possibility may make recommendable to retard the surgical treatment as much as possible in cases that it is not necessary.

TITLE: Neurofibromas plexiformes voluminosos de cuello en la neurofibromatosis tipo 1.Objetivo. Presentar las caracteristicas clinicas, de imagen y evolutivas de una serie de pacientes con neurofibromatosis tipo 1 que desarrollaron durante la infancia neurofibromas plexiformes voluminosos en el cuello (NFPVC). Pacientes y metodos. Nueve pacientes (cinco mujeres y cuatro varones) con edades entre 3 y 15 años en el momento del diagnostico de los tumores, que podian extenderse tambien a la fosa posterior y a la zona toracica superior. El diagnostico estuvo basado fundamentalmente en la clinica, la imagen y la histologia. Resultados. Un tumor era intralaringeo y causaba problemas respiratorios. Los otros ocho casos tenian su origen en varias raices espinales de uno o de ambos lados y podian crecer tambien hacia el interior de la fosa posterior y de la region toracica en algunos pacientes, y desplazaban a las estructuras anatomicas vecinas, especialmente en tres casos, todos niñas, en las que el tumor crecio hasta alcanzar gran volumen, especialmente por un lado, parandose el crecimiento entre los 11 y 12 años y no volviendo a crecer mas tarde. Conclusiones. Los NFPVC son tumores histologicamente benignos. La extirpacion es necesaria cuando estan localizados en la laringe por los problemas respiratorios que causan, pero no en los de las otras regiones, pese a que el voluminoso tamaño que alcanzan en algunos casos puede causar exagerados desplazamientos de las estructuras vecinas. El estudio de nuestra serie parece indicar que al menos los tumores extralaringeos solo crecen hasta los 11-12 años. Puede ser recomendable retrasar la cirugia tanto como sea posible si no existe sintomatologia aguda que la haga necesaria.

[Corpus callosum tumor as the presenting symptom of neurofibromatosis type 1 in a patient and literature review]. / Tumor del cuerpo calloso como presentación de neurofibromatosis tipo 1 en un paciente y revisión de la bibliografía.

INTRODUCTION: Neurofibromatosis type 1 (NF1) is one of the most frequent neurocutaneous syndromes. NF1 can be associated with intracranial tumors in any location, but only rarely in the corpus callosum. AIMS: To describe a case of NF1 presenting as a tumor of the corpus callosum and to carry out a review of the incidence of the tumors of corpus callosum in our series and in the literature. CASE REPORT: We present a child who was studied since 3 years of age because of complete NF1 clinical diagnostic criteria (without genetic study). He was studied by MR and magnetic resonance spectroscopy (MRS). MR study showed neurofibromatosis bright objects distributed over several regions of the cerebral hemispheres and cerebellum, a possible brain stem tumor (bulbar zone) and the splenium of the corpus callosum. The MRS of the brain stem tumor showed changes consistent with a low grade glial tumor. The patient was followed until 19-years of age without demonstrating any changes in the clinical features or the tumor size in both locations Only six cases of corpus callosum tumor in patients with NF1 have been published to date. CONCLUSIONS: We present a new case with tumor of the corpus callosum and NF1. The imaging characteristics and the clinical course were in favour of the benign nature of this type of tumor.

Bilateral spinal neurofibromas in patients with neurofibromatosis 1.

Neurofibromatosis 1 (NF1) is a neurocutaneous syndrome that can be inherited as autosomal dominant or may appear due to a de novo mutation. We present 8 patients (5 M and 3 F) with sporadic or non-familial spinal neurofibromatosis 1 (non-FSNF1) associated with bilateral spinal neurofibromas involving all of the paraspinal nerves. To our knowledge, this is the first series of such association described in the literature. Their ages ranged from 6 months to 20 years (average 9.8 years) at the time of radiological diagnosis. This presentation appears to be earlier than in familial spinal neurofibromas in NF1 (FSNF1). Predisposition to malignancy probably is greater in the non-FSNF1 type. MRI studies were performed routinely in all patients with NF1 and these were complemented with MRI enhanced with gadolinium and repeated at different ages in cases with paraspinal tumors. Coronal views provided the best evidence for the presence of neurofibromas in every spinal nerve. The size of the tumors and the clinical complications increased with advancing age in most patients. Giant plexiform tumors were often seen in the cervico-thoracic region. Malignant peripheral nerve sheath tumors (MPNST) were found in one patient with a sciatic tumor and another patient died suddenly at home without necropsy or pathological study. Voluminous paraspinal neurofibromas can be at risk for malignancy. More frequent neuroimaging studies may be necessary for an earlier detection. Early surgical treatment to anticipate the occurrence of MPNST during surveillance could be an option. Bilateral spinal neurofibromas are found in both patients who inherited the NF1 and in those due to de novo mutations.

[Surgery of intractable epilepsy in infants and adolescents. Case report]. / Cirugía de epilepsia refractaria en niños y adolescentes. Serie de casos.

Our objective is to present a series of 20 cases of children under 15 years operated on of intractable epilepsy in the last 12 years in Sanatorio Allende of Córdoba, based on medical records and follow up. Were included 8 patients with cortical dysplasias, 6 with tumors, 2 with Rasmüssen syndrome, 1 with mesial gliosis, 1 with porencephalic cyst, 1 with gliosis due to traumatic brain injury, and 1 with encephalitis history. Localization was temporal in 9 cases and extra-temporal in 11 cases (4 were frontal, 4 occipital, and 3 parietal). There was no surgical mortality. Patients with brain tumors present a satisfactory control over convulsive crises after tumor resection (Engel IA in 100%). Good results were also achieved in nontumor pathologies: 8 cases Engel IA, 1 Engel I B; 3 cases Engel III A, one IV A and other IV C.

Experiencia en el tratamiento de los meningiomas del ángulo pontocerebeloso del Sanatorio Allende / Experience in the treatment of meningiomas of the pontocerebellar angle of the Allende Sanatorium

Objetivo: Presentar nuestra experiencia en el tratamiento de meningiomas del ángulo pontocerebeloso, desde 1972 a 2013. Material y Método: Se realizó un trabajo retrospectivo y descriptivo. Se incluyeron 8 casos de pacientes con meningiomas de ángulo pontocerebeloso, cuyas edades oscilaron entre 41 y 68 años; 7 de ellos fueron mujeres. Resultados: Se realizaron 8 cirugías de exéresis tumoral. En un paciente se realizó, además, radioterapia por presentar recurrencia. En 5 casos la resección fue total (Simpson 1 o 2) y en 3 fue parcial. Los primeros se localizaban todos a nivel posterior del complejo VII-VIII. Hubo un caso de muerte postquirúrgica por sepsis secundaria a un absceso de la cavidad operatoria. Conclusión: En los meningiomas de ángulo pontocerebeloso, principalmente en aquellos ubicados por detrás del conducto auditivo interno, la exéresis completa es posible y constituye un tratamiento capaz de controlar la enfermedad. La radioterapia sería una opción a considerar en un número limitado de enfermos.

Objective: To present our experience in the treatment of cerebellopontine angle (CPA) meningiomas from 1972 to 2013. Materials and Methods: A descriptive, retrospective study was performed involving eight patients (7 females) with a cerebellopontine angle meningioma, whose ages ranged from 41 to 68 years. Results: Tumor resection was performed in all eight patients, with one patient requiring subsequent radiotherapy for tumor recurrence. In five patients, resection was complete (Simpson 1 or 2), while only partial resection was achieved in three. The former lesions were all posterior to the VII-VIII complex. There was one death from post-surgical sepsis secondary to an abscess in the surgical cavity. Conclusions: With meningiomas of the CPA, and especially those located behind the internal auditory canal, complete excision is possible and treatment can control the disease. Radiotherapy is an option to consider in a limited number of patients.

Major and minor arterial malformations in patients with cutaneous vascular abnormalities.

The association of persistent embryonic arteries and the absence of 1 carotid or vertebral arteries with facial or neck hemangioma or vascular malformation have been frequently described. The abnormalities can involve major or minor vessels. Of 22 patients of our series with this neurocutaneous syndrome, 20 had the origin of both anterior cerebral arteries from the same internal carotid artery. Thirteen patients showed absence or hypoplasia of 1 carotid artery and 10 of 1 vertebral artery; 10 showed persistence of the trigeminal artery; 3 had persistent proatlantal artery; 6 showed the absence of the posterior communicating artery; and 4 had hypoplastic posterior cerebral artery. Other less frequent abnormalities were found in 7 patients. Intellectual level of most patients was either borderline or below normal. Abnormalities in the vascularization and perfusion of the frontal lobes may contribute to the borderline or lower mental level of these patients.

[Cerebral hemisphere tumours in neurofibromatosis type 1 during childhood]. / Tumores de los hemisferios cerebrales en la neurofibromatosis tipo 1 durante la infancia.

AIM: To present seven tumors of the cerebral hemispheres in 6 children with neurofibromatosis type 1 (NF1). PATIENTS AND METHODS: Six patients (three males and three females) of 600 cases of a series with NF1 showed features of cerebral hemispheres tumor (seizures, headache and hemiparesis). They were studied neurologically, by EEG and by image (MR and/or spectroscopic-MR) because of these features or simply because having NF1. RESULTS: All the patients had the two diagnostic criteria of the NF1. Six patients had seven tumors (it was because one of them had one tumor in every frontal lobe, both with the same image characteristics), but they did not were removed and they were not studied histologically. The histological study was made to the other five patients and showed that the histological nature corresponded to pilocytic astrocytoma in one patient, neuroepitelial dysembryoplastic tumor in one, polymorphe xanthoastrocytoma in one, neuroectodermic hamartoma in one, and inflammatory chronic non-granulomatose lesion in one. CONCLUSION: The prevalence of the tumors in the cerebral hemispheres is very low (1%) in the patients with NF1. The tumors commonly are histologically benign, and they can be found in peripheral or deep region of the cerebral hemispheres. Identity of the tumors by MR study commonly is easy and the treatment is surgical in most cases. However, urgent treatment very seldom is necessary in these tumors, and most frequently is possible to take the attitude of 'wait and see' before to decide the definite treatment.

Cirugía de epilepsia con electrocorticografía intraoperatoria / Epilepsy surgery with intraoperative electrocorticography

OBJETIVO: presentar nuestra experiencia en cirugía de epilepsia con electrocorticografía intraoperatoria, en 19 pacientes. MATERIAL Y MÉTODO: estudio retrospectivo basado en historias clínicas de pacientes con epilepsia lesional operados en el Sanatorio Allende, de Córdoba, entre el 1 de diciembre de 1997 y el 30 de noviembre de 2013. En esta serie hubo 14 enfermos menores de 20 años y sólo 5 mayores de esa edad. Las lesiones fueron: en 10 (52,6%) displasias corticales, en 6 (31,5%) tumores, en 2 gliosis cicatrizal y en 1 cavernoma frontal. La localización fue temporal en 4 (21%) y extratemporal en 15 (79%). Tenían epilepsia refractaria 13 (67,3%) enfermos. RESULTADOS: el tratamiento fue satisfactorio si analizamos el control de las crisis. Actualmente 14 (73,6%) están libres de crisis, sólo 4 de ellos tienen un EEG anormal, por lo cual continúan medicados. De los 5 (26,4%) enfermos que continúan con crisis, 3 tienen episodios esporádicos y tienen una sola medicación; los otros 2 tienen crisis frecuentes por lo cual reciben 3 fármacos antiepilépticos. CONCLUSIÓN: la electrocorticografía intraoperatoria nos ha permitido identificar con precisión el foco epileptógeno, que en muchos casos esta adyacente o distante de la lesión

INTRODUCTION: to present our experience in epilepsy surgery with intraoperative electrocorticography in 19 patients. MATERIAL AND METHOD: retrospective study based on clinical records of patients with epilepsy operated on between December 1997 and November 2013 in Sanatorio Allende of Córdoba. In this series there were 14 patients younger than 20 years. Included: 10 (52,6) cortical displeases, 6 (31,5%) tumours, 2 cicatricial gliosis, and 1 cavernoma. The localization was temporal in 4 (21%), and extratemporal in 15 (79%). Thirteen (67,3%) patients had medically intractable epilepsies. RESULTS: in terms of epilepsy, surgical treatment with intraoperative electrocorticography was satisfactory. At the present: 14(73%) are free of seizures; only 4 had abnormal EEG and go on with anticonvulsive medication. Five patients to remain with epilepsy, only 2 of them had frequent crisis and required three anticonvulsive drugs. CONCLUSION: the intraoperative electrocorticography permitted to identify the epileptogenic area with accuracy. This area may be situated adjacent or distant to the primary lesion

Cirugía descompresiva occipito-cervical en un adulto con osteopetrosis y malformación de chiari I / Occipito-cervical decompressive surgery in an adult with chiari I osteopetrosis and malformation

Introducción: la osteopetrosis es una rara enfermedad de origen genético, causada por una disminución en la resorción del tejido óseo, pudiendo ser autosómica dominante (benigna) o autosómica recesiva (maligna). Por su afectación craneal puede causar un severo compromiso neurológico (ceguera, parálisis facial, hipoacusia, hipertensión intracraneal, entre otros). El tratamiento incluye el uso de Interferon gamma, el transplante de médula ósea y la cirugía.Descripción del caso: presentamos una paciente de 32 años con osteopetrosis de tipo autonómico dominante asociada a malformación de Chiari I. La misma presentaba cefalea, síndrome cerebeloso, cuadriparesia, disartria, hipoacusia e hipertensión arterial, con edema de papila bilateral. Se realizó una cirugía de descompresión suboccipital, con laminectomía de C1 y duroplastia. Resultados: la paciente tuvo una franca mejoría, la cual mantiene tras 5 años de seguimiento. Continúa tatamiento con Interferon Gamma, Calcitriol y Carbamacepina.

Introduction: osteopetrosis is a rare genetic disease, caused by a decrease in bone resorption. It may be autosomal dominant (benign) or autosomal recessive (malignant). Due to cranial involvement it can cause severe neurological deterioration (blindness, facial paralysis, hearing loss, intracranial hypertension). Treatment includes the use of gamma interferon, bone marrow transplantation and surgery. Case Presentation: we present a 32 year old patient with autosomal dominant osteopetrosis type associated with Chiari I Malformation. She had headaches, cerebellar syndrome, quadriparesis, dysarthria, hearing loss and arterial hypertension, with bilateral papilledema. We conducted a suboccipital decompression surgery with C1 laminectomy and duroplasty. Results: the patient had a marked improvement, which remains after 5 years of follow up. She continues treatment with Interferon Gamma, Calcitriol and Carbamazepine.

Epilepsy surgery in MRI negative patient / Cirurgia da epilepsia em paciente com ressonancia magnética normal

Introdução: Epilepsia não-lesional ou epilepsia sem alteração na ressonancia magnética é caracterizada pela ausência de um foco potencialmente epileptogênico.Nesta situação, procedimentos cirúrgicos são mais complexos e mais desafiadores. Relato do caso: Um paciente de 6 anos e 8 meses apresentava quadro de epilepsia refratária de início aos 8 meses: as crises eram predominantemente do tipo parciais complexas. Não havia sinais neurológicos focais, mas observavam-se alterações cognitivas, de comportamento e do aprendizado. O vídeo EEG mostrava foco temporo-ocipital esquerdo, com irradiação para a direita. A ressonancia magnética e o PET inter-ictal eram normais, embora o SPETC ictal mostrasse focos occipital esquerdo e temporal direito, este de provável propagação secundária. A ressecção do foco à esquerda foi realizada em dois tempos, com uso de eletrodos subdurais. O estudo anátomo-patológico mostrou displasia cortical tipo 1A. Na revisão de dois anos, o paciente apresentou apenas uma crise convulsiva anual, tendo apresentado melhora no comportamento e aprendizado, bem como melhora cognitiva. Conclusão: Neste caso de epilepsia refratária, sem alterações na RM, o SPETC foi essencial para a localização do foco epileptogênico.