Epilepsy and musical perception. A perspective involving 14 patients. / Epilepsia y percepción musical. Una visión a través de 14 pacientes.

INTRODUCTION: The relationship between epilepsy and music is poorly understood. Musicogenic epilepsy, which involves seizures triggered by music, and epilepsy that triggers or involves musical experiences are rare. Anti-seizure medications (ASMs) may affect cognition and possibly the musical sphere. The relationship between epilepsy, ASMs and music perception is insufficiently investigated in the literature. This study describes the clinical characteristics of patients with epilepsy with advanced musical knowledge, and aims to understand the disease's involvement in the musical sphere. PATIENTS AND METHODS: A qualitative study was conducted in epileptic patients with musical knowledge, investigating their musical perception before and after a diagnosis of epilepsy and after a change of ASM when this was possible. Questionnaires and recordings of music were used to assess musical perception. RESULTS: Fourteen patients had musical knowledge, and the majority of these (50%) had temporal lobe epilepsy. A total of 92.8% of the patients stated that epilepsy or its medications had affected them in the musical sphere. There was no clear relationship between the lateralisation of the epilepsy and musical involvement. 42.9% were professional musicians, and had to give up their profession. The patients prescribed with more than one ASM had greater musical involvement. CONCLUSIONS: Temporal lobe epilepsy appears to have the greatest effect on music perception, and more studies with ASM and music perception are needed to determine its effects.

TITLE: Epilepsia y percepción musical. Una visión a través de 14 pacientes.Introducción. La relación entre la epilepsia y la música es poco comprendida. La epilepsia musicógena, que involucra crisis desencadenadas por la música, y la epilepsia que produce o involucra experiencias musicales son poco comunes. Se sabe que los medicamentos anticrisis (MAC) pueden afectar a la cognición y posiblemente a la esfera musical. La relación entre la epilepsia, los MAC y la percepción musical está insuficientemente investigada en la bibliografía. El objetivo de este estudio es describir las características clínicas de pacientes con epilepsia con conocimientos musicales avanzados e intentar comprender la afectación de la enfermedad a la esfera musical. Pacientes y métodos. Se llevó a cabo un estudio cualitativo en pacientes epilépticos con conocimientos musicales, investigando su percepción musical antes y después del diagnóstico de epilepsia y, cuando fue posible, tras el cambio de MAC. Se utilizaron cuestionarios y grabaciones musicales para evaluar la percepción musical. Resultados. Catorce pacientes tenían conocimientos musicales, la mayoría (50%) con epilepsia del lóbulo temporal. Un 92,8% de los pacientes indicó que la epilepsia o sus medicamentos le habían afectado en la esfera musical. No había una clara relación entre lateralización de la epilepsia y afectación musical. Un 42,9% eran músicos profesionales y tuvieron que dejar la profesión. Los pacientes con más de un MAC pautado tenían mayor afectación musical. Conclusiones. La epilepsia del lóbulo temporal parece ser la que más afecta a la percepción musical, y hacen falta más estudios con MAC y percepción musical para dilucidar sus efectos.

[The sign of the cross: a very rare automatism in temporal lobe epilepsy. Two case reports]. / La señal de la cruz: un automatismo muy poco frecuente en la epilepsia del lóbulo temporal. Descripción de dos casos.

INTRODUCTION: In the evaluation of drug-resistant epilepsy (DRF), a detailed analysis of the semiology is essential to establish a diagnostic hypothesis of the location of the epileptogenic zone. Cross-sign (CS) is a very infrequent complex manual automatism described for the first time in 2008 and rarely reported in the literature. CASE REPORT: We present two cases from our series of patients monitored by videoEEG, one of whom also studied with deep electrodes, in which we describe the location of the discharge while performing the CS. A bibliographic review is also carried out to try to establish a localizing and/or lateralizing value of this sign. CONCLUSION: The sign of the cross is a rare ictal automatism that occurs in patients with temporal lobe epilepsy. The hand used to make the sign of the cross is the dominant one, regardless of the origin of the crises.

TITLE: La señal de la cruz: un automatismo muy poco frecuente en la epilepsia del lóbulo temporal. Descripción de dos casos.Introducción. En la evaluación de la epilepsia farmacorresistente, el análisis detallado de la semiología es fundamental para establecer una hipótesis diagnóstica de la localización de la zona epileptógena. La señal de la cruz es un automatismo manual complejo muy infrecuente descrito por primera vez en 2008 y poco referido en la bibliografía. Caso clínico. Presentamos dos casos con presencia de la señal de la cruz de nuestra serie de pacientes monitorizados mediante videoelectroencefalograma, uno de ellos estudiado también con electrodos profundos, en los que describimos la localización de la descarga en el momento de realizar la señal de la cruz. Se realiza también una revisión bibliográfica para intentar establecer un valor localizador y/o lateralizador de este signo. Conclusión. La señal de la cruz es un raro automatismo ictal que ocurre en pacientes con epilepsia del lóbulo temporal. La mano empleada para la señal de la cruz es la dominante, independientemente del origen de las crisis.

[No fear of anything: epilepsy, psychiatric comorbidity and surgery. A clinical case report]. / Sin miedo a nada: epilepsia, comorbilidad psiquiátrica y cirugía. Descripción de un caso clínico.

INTRODUCTION: Psychiatric conditions are common in patients with drug-resistant temporal lobe epilepsy. It can be extremely disabling, as it hinders the patient's integration into society. Emotional symptoms may occur during crises or can be chronic and not directly related to seizures. Some of the most common disorders are depression, followed by anxiety disorders and obsessive symptoms such as phobias. CASE REPORT: In the clinical case reported here, the patient suffers from drug-resistant focal epilepsy and psychiatric comorbidity (anxiety-depressive syndrome and phobias). In the pre-surgical evaluation, the origin of the seizures was established as the right mesial temporal region, and so surgery was performed. In check-ups, an improvement of both conditions was observed after surgery to treat her epilepsy. CONCLUSION: The activation of the amygdala and its interaction with different brain structures seem to play an important role in emotional learning and aversive stimuli, thereby conditioning (depending on its pattern of activation) the individual's ability to control their fear. There seems to be excessive activation in individuals with phobias. Surgical resection by anteromedial temporal lobectomy in patients with temporal lobe epilepsy is an effective treatment to achieve control over refractory seizures, and may also help to ease emotional symptoms, thus adding an extra benefit. A pre-surgical evaluation with a multidisciplinary approach can be used to predict post-operative neuropsychological change.

TITLE: Sin miedo a nada: epilepsia, comorbilidad psiquiátrica y cirugía. Descripción de un caso clínico.Introducción. Es común que la patología psiquiátrica se manifieste en pacientes con epilepsia farmacorresistente del lóbulo temporal. Puede llegar a ser tremendamente incapacitante, puesto que obstaculiza la integración del paciente en la sociedad. Los síntomas emocionales pueden aparecer durante las crisis o presentarse crónicamente y no directamente relacionados con ellas. Entre los trastornos más frecuentes se encuentra la depresión, seguida por los trastornos de ansiedad y los síntomas obsesivos, como pueden ser las fobias. Caso clínico. En el caso clínico presentado, la paciente padece una epilepsia focal farmacorresistente y comorbilidad psiquiátrica (síndrome ansiosodepresivo y fobias). En la evaluación prequirúrgica se establece como origen de las crisis la región temporal mesial derecha, por lo que se realiza cirugía. En revisiones se constata una mejoría de ambas patologías tras la actuación quirúrgica sobre su epilepsia. Conclusión. La activación de la amígdala y su interacción con diferentes estructuras cerebrales parecen desempeñar un papel importante en el aprendizaje emocional y en estímulos aversivos, condicionando, en función de su patrón de activación, la habilidad del individuo para controlar el miedo. Parece existir un exceso de activación en los individuos que presentan fobias. La resección quirúrgica mediante lobectomía temporal anteromedial en pacientes con epilepsia del lóbulo temporal es un tratamiento eficaz para el control de las crisis refractarias, que, además, puede ayudar a remitir los síntomas emocionales, añadiendo un beneficio adicional. Una evaluación prequirúrgica previa con un enfoque multidisciplinar puede utilizarse para predecir el cambio neuropsicológico postoperatorio.

[Temporal pole encephalocele, a surgical treatable cause of drug-resistant epilepsy]. / Encefalocele del polo temporal: una causa tratable quirúrgicamente de epilepsia farmacorresistente.

INTRODUCTION: Encephaloceles are herniation of brain parenchyma through a bony skull defect that can cause drug-resistant epilepsy. In these cases, a surgical approach should be considered. CASE REPORT: 38-year-old man with drug-resistant epilepsy and 1.5 T magnetic resonance imaging performed with no relevant findings. After video-electroencephalogram, 3 T magnetic resonance imaging and F-18-fluoro-deoxy-glucose positron emission tomography, a right temporal encephalocele was confirmed. A right temporal polar resection was performed four years ago and the patient remains seizure-free. DISCUSSION: Anterior temporal encephaloceles are an underdiagnosed cause of epilepsy, and a 3 T magnetic resonance imaging reviewed by an epilepsy expert radiologists is key to diagnosis. CONCLUSION: In drug-resistant cases with presurgical evaluation compatible with the location, surgical treatment must be considered.

TITLE: Encefalocele del polo temporal: una causa tratable quirúrgicamente de epilepsia farmacorresistente.Introducción. Los encefaloceles son herniaciones del parénquima cerebral consecuencia de un defecto óseo. Si producen epilepsia farmacorresistente, hay que plantearse un abordaje quirúrgico. Caso clínico. Varón de 38 años con epilepsia farmacorresistente y estudio estándar de resonancia magnética de 1,5 T sin hallazgos. Tras realizar un videoelectroencefalograma, una resonancia magnética de 3 T y una tomografía por emisión de positrones con fluorodesoxiglucosa, se confirmó la presencia de un encefalocele del polo temporal derecho. Se efectuó una resección de ese polo temporal y cuatro años después el paciente continúa libre de crisis. Discusión. Los encefaloceles temporales anteriores constituyen una causa infradiagnosticada de epilepsia en cuyo estudio es clave la resonancia magnética de 3 T y la evaluación por un radiólogo experimentado en epilepsia. Conclusión. En casos farmacorresistentes y evaluación prequirúrgica de la epilepsia congruente con la localización, hay que considerar su tratamiento quirúrgico.

Perampanel effectiveness and safety as early add-on treatment for focal-onset seizures: PEREAGAL study.

BACKGROUND: Perampanel (PER) is an effective adjunctive therapy for controlling focal-onset seizures (FOS), but few studies have examined its effects as an early add-on for the treatment of FOS in daily clinical practice. METHODS: Our retrospective, multicenter, observational study evaluated the effectiveness and safety of PER as an early add-on in 77 patients with FOS, with and without focal to bilateral tonic-clonic seizures (FBTCS) after 3, 6 and 12 months in a real-world setting. RESULTS: After 12 months of treatment (median dose 6 [4,8] mg/day), the retention rate was 79.2 % and 60 % of patients (39/65) experienced a ≥50 % reduction in seizure frequency relative to baseline. The seizure-free rate was 38.5 % for all seizures (25/65) and 60 % for FBTCS (12/20). The responder rate at 12 months was significantly higher when PER was given with one concomitant AED (72.2 %) compared to when PER was given with two concomitant AEDs (44.8 %). Drug-related adverse events (AEs) were reported in 40.3 % of patients, most of them being mild (64.2 %). Twelve patients (15.6 %) discontinued treatment because of AEs. CONCLUSIONS: PER is an effective and safe early add-on for patients with refractory FOS, especially for those with FBTCS.

[Functional hemispherectomy: long-term follow-up in a series of five patients]. / Hemisferectomia funcional: seguimiento a largo plazo en una serie de cinco casos.

INTRODUCTION: Functional hemispherectomy consists in palliative epilepsy surgical procedure usually performed in patients with pharmaco-resistant epilepsy and hemispheric syndromes. It is based on the neural disconnection of the affected hemisphere with preservation of the vascular supply. AIM: To analyze long-term prognosis and safety of the hemispherectomies performed in our institution. PATIENTS AND METHODS: Retrospective analysis collecting the following variables: age, gender, age of epilepsy onset, type of seizures, etiology, age of epilepsy surgery, prognosis and potential surgical complications. All patients had a minimum of five years of follow up. RESULTS: Five patients (60% females) underwent hemispherotomy between 1999 and 2010. Age of epilepsy onset was 36 months and time of evolution until surgery was 7 years. The most frequent type of seizures were simple motor seizures with secondary generalization (n = 5). Three patients remained seizure free persistently after surgery and another patient had a more than 90% improvement. Time of follow up was 13 years. One patient suffered a bacterial meningitis without sequelae. Six years after surgery a patient suffered hydrocephalous requiring ventriculoperitoneal shunt. CONCLUSIONS: Functional hemispherectomy constitutes an effective method to treat patients with pharmaco-resistant epilepsy, extensive unihemispheric pathology and seizures limited to that hemisphere. Late complications may occur thus long-term follow-up is needed.

TITLE: Hemisferectomia funcional: seguimiento a largo plazo en una serie de cinco casos.Introduccion. La hemisferectomia funcional es una de las tecnicas quirurgicas con intencion paliativa que se pueden realizar en pacientes con epilepsia farmacorresistente y sindromes hemisfericos. Se basa en la desconexion neuronal del hemisferio afectado preservando el arbol vascular. Objetivo. Analizar el pronostico y la seguridad a largo plazo de las hemisferectomias realizadas en nuestro centro. Pacientes y metodos. Revision retrospectiva de los casos intervenidos, recogiendo las siguientes variables clinicas: edad, sexo, edad de inicio de la epilepsia, tipo de crisis, etiologia de la epilepsia, edad de intervencion, pronostico posquirurgico y posibles complicaciones. El seguimiento minimo fue de cinco años. Resultados. Cinco pacientes (60% mujeres) fueron intervenidos entre 1999 y 2010. La edad de inicio de la epilepsia fue de 36 meses, y el tiempo de evolucion hasta la cirugia, de 7 años. El tipo de crisis mas habitual fueron las crisis parciales simples motoras con generalizacion secundaria (n = 5). Tres pacientes permanecieron libres de crisis tras la cirugia, y otro paciente mejoro mas de un 90%. El tiempo medio de seguimiento fue de 13 años. Como complicaciones, una paciente sufrio una meningitis bacteriana sin secuelas posteriores. A los seis años de la cirugia, un paciente presento una hidrocefalia que requirio la implantacion de una valvula de derivacion ventriculoperitoneal. Conclusiones. La hemisferectomia funcional constituye un procedimiento quirurgico eficaz para el tratamiento de pacientes con epilepsia farmacorresistente, patologia hemisferica extensa y crisis limitadas a ese hemisferio. Hay complicaciones que pueden aparecer tardiamente, por lo que se aconseja un seguimiento a largo plazo de estos pacientes.

VNS and pregnancy: A multicentric experience of four cases.

BACKGROUND: Vagus nerve stimulation system (VNS) has been employed worldwide as adjunctive therapy in drug-resistant epileptic patients. Only nine previous pregnancies with six-positive outcomes have been reported in women with epilepsy treated with VNS since 1998. AIMS OF THE STUDY: To communicate the experience of pregnancies in women treated with VNS in our country. METHODS: Clinical data of four female patients treated with VNS during pregnancy and delivery in five gestations is described. RESULTS: Four pregnancy outcomes were positive and one ended in spontaneous abortion, probably more related to the use antiepileptic drugs than VNS itself. Two births were vaginal and the other two with cesarean section. None of the complications during delivery were attributed to VNS. No teratogenicity was documented. CONCLUSIONS: Based on our experience VNS constitutes a safe therapy for the treatment of drug-resistant epilepsy in women of childbearing potential and during pregnancy and delivery. Larger series will be useful to confirm this finding.

EARLY-ESLI study: Long-term experience with eslicarbazepine acetate after first monotherapy failure.

PURPOSE: Evaluate real-life experience with eslicarbazepine acetate (ESL) after first monotherapy failure in a large series of patients with focal epilepsy. METHOD: Multicentre, retrospective, 1-year, observational study in patients older than 18 years, with focal epilepsy, who had failed first antiepileptic drug monotherapy and who received ESL. Data from clinical records were analysed at baseline, 3, 6 and 12 months to assess effectiveness and tolerability. RESULTS: Eslicarbazepine acetate was initiated in 253 patients. The 1-year retention rate was 92.9%, and the final median dose of ESL was 800 mg. At 12 months, 62.3% of patients had been seizure free for 6 months; 37.3% had been seizure free for 1 year. During follow-up, 31.6% of the patients reported ESL-related adverse events (AEs), most commonly somnolence (8.7%) and dizziness (5.1%), and 3.6% discontinued due to AEs. Hyponatraemia was observed in seven patients (2.8%). After starting ESL, 137 patients (54.2%) withdrew the prior monotherapy and converted to ESL monotherapy; 75.9% were seizure free, 87.6% were responders, 4.4% worsened, and 23.4% reported ESL-related AEs. CONCLUSION: Use of ESL after first monotherapy failure was associated with an optimal seizure control and tolerability profile. Over half of patients were converted to ESL monotherapy during follow-up.

Safety, efficacy and outcome-related factors of perampanel over 12 months in a real-world setting: The FYDATA study.

BACKGROUND: Real-world data of current antiepileptic drugs (AEDs) used to treat focal seizures is of importance to understand the efficacy and safety outside of the clinical trial setting. Here we report real-world data from a large series of patients treated with perampanel for 1year. METHODS: FYDATA was a multicentre, retrospective, 1-year observational study assessing the efficacy and safety of adjuvant perampanel in patients ≥12 years of age with focal epilepsy in a real-world setting. At 12 months, the proportion of patients who were seizure free, median percentage seizure reduction, proportion of responders, retention rate and proportion of patients with adverse events (AEs) were assessed. Analyses were also performed to identify any patient-, medication- and disease-related factors associated with a large clinical response or carry a risk for AEs. RESULTS: A total of 464 patients were included in the study with a retention rate of 60.6% at 1year. The mean number of prior AEDs was 7.8. The median percentage reduction in overall seizures was 33.3% (75% for secondary generalised seizures) after 1year, with 7.2% of patients achieving seizure freedom. Furthermore, patients on non-enzyme-inducing AEDs were more likely to achieve seizure freedom, and logistic regression revealed that patients aged ≥65 years, those with epilepsy due to a vascular aetiology and those who had received fewer prior AEDs showed a better clinical response to perampanel. A total of 62.9% of the patients experienced AEs at 12 months; dizziness, somnolence and irritability were the most frequent AEs. Patients with prior psychiatric comorbidities (hyperactivity and personality disorder) were more likely to experience psychiatric AEs with perampanel, and slower titration schedules were associated with less AEs overall. CONCLUSION: Perampanel, for the treatment of focal epilepsy in a real-world setting in a refractory population, over 1year, demonstrates a similar efficacy and safety profile to that observed in clinical trials. Our results have implications for the optimisation of perampanel use in a clinical setting.

Drug-resistant epilepsy: definition and treatment alternatives.

INTRODUCTION: Drug-resistant epilepsy affects 25% of all epileptic patients, and quality of life decreases in these patients due to their seizures. Early detection is crucial in order to establish potential treatment alternatives and determine if the patient is a surgical candidate. DEVELOPMENT: PubMed search for articles, recommendations published by major medical societies, and clinical practice guidelines for drug-resistant epilepsy and its medical and surgical treatment options. Evidence and recommendations are classified according to the criteria of the Oxford Centre for Evidence-Based Medicine (2001) and the European Federation of Neurological Societies (2004) for therapeutic actions. CONCLUSIONS: Identifying patients with drug-resistant epilepsy is important for optimising drug therapy. Experts recommend rational polytherapy with antiepileptic drugs to find more effective combinations with fewer adverse effects. When adequate seizure control is not achieved, a presurgical evaluation in an epilepsy referral centre is recommended. These evaluations explore how to resect the epileptogenic zone without causing functional deficits in cases in which this is feasible. If resective surgery is not achievable, palliative surgery or neurostimulation systems (including vagus nerve, trigeminal nerve, or deep brain stimulation) may be an option. Other treatment alternatives such as ketogenic diet may also be considered in selected patients.

Levetiracetam following liver and kidney failure in late-onset anticonvulsant hypersensitivity syndrome.

Anticonvulsant hypersensitivity syndrome is an adverse drug reaction usually occurring from 1 to 8 weeks after exposure to antiepileptic drugs. It can threaten life by affecting the liver, kidneys, central nervous system or lungs. We present a 47-year-old patient treated with phenytoin, lamotrigine and clobazam for 7 years. He presented with hepatic and renal failure in relation to this syndrome demonstrated by renal biopsy. Prognosis was excellent due to an early diagnosis leading to cessation of the causative agents. Levetiracetam was started with a good response.

Successful outcome of episodes of status epilepticus after vagus nerve stimulation: a multicenter study.

BACKGROUND AND PURPOSE: Vagus nerve stimulation (VNS) has been reported to be a safe and effective treatment for drug-resistant epilepsy. The aim of this study is to describe the effect of VNS in patients with a history of repeated episodes of status epilepticus (SE) before implantation. METHODS: From a total of 83 adult patients with drug-resistant epilepsy who had VNS implanted in four tertiary centers in Spain between 2000 and 2010, eight had a previous history of repeated episodes of SE. We performed a retrospective observational study analyzing the outcome of seizures and episodes of SE after implantation. Stimulation was started at the usual settings, and intensity increased according to clinical response and tolerability. RESULTS: Regarding the eight patients with a history of SE, the mean age at time of VNS implantation was 25.1 [14-40] years. Duration of epilepsy until the implantation was 21.7 [7-39.5] years, and they had been treated with a mean of 12 antiepileptic drugs [10-16]. Mean follow-up since implantation was 4.15 [2-7.5] years. Average seizure frequency decreased from 46 to 8.2 per month. Interestingly, four of the eight patients remained free of new episodes of SE after implantation, and in two additional patients, the frequency decreased by >75%. Adverse effects were mild or moderate in intensity and included mainly coughing and dysphonia. CONCLUSION: In those patients with refractory epilepsy and history of SE who are not surgical candidates, VNS is a safe and effective method to reduce seizure frequency and episodes of SE.

Meningioangiomatosis: clinical-radiological features and surgical outcome.

Meningioangiomatosis (MA) is a rare, benign neoplastic disorder involving the cortex and leptomeninges, the sporadic form, commonly presents as refractory localization-related epilepsy, but could be asymptomatic especially in older patients. The imaging features may be entirely non-specific. Magnetic Resonance Imaging (MRI) erroneously suggests meningioma, lowgrade tumour or vascular malformations. The pathological findings are characterised by proliferation of meningothelial cells and leptomeningeal vessels and calcifications within the mass. Macroscopically there is dense thickening in the underlying cortex, often in a sharply defined area. In this article we report 3 cases of MA, neither of whom had a familiary history or stigmata of Neurofibromatosis (NF). We discuss and place particular emphasis on the clinical presentation and diagnosis imaging, as well as on the outcome. We also review the literature concerning about the aetiology, pathology findings and imaging features of MA.

[A clinico-anatomical correlation study of logorrhoea]. / Estudio de correlación clinicoanatómica de la logorrea.

INTRODUCTION: The degree of verbal production necessary to be considered logorrhoea has still not been defined, and no clear correlation has been established between the topography of the dysfunction and this symptom. AIMS: To provide quantitative data about normal verbal production and to identify the location within the brain of the alterations observed in neuroimage of patients with logorrhoea. SUBJECTS AND METHODS: The oral verbal production of 60 control subjects between 20 and 80 years of age was quantified by analysing five speeches. Ten patients who exceeded the 75th percentile in at least two of the five speeches underwent structural and functional neuroimaging tests. RESULTS. The data on verbal production of normal subjects are reported. Age, sex and habits (smoking, coffee, alcoholic drinks) did not exert an influence, but the degree of schooling was seen to have an effect. All the patients were diagnosed with frontotemporal degeneration, although in one case there were also coexisting vascular risk factors and subcortical vascular lesions, which reduce the degree of certainty of the diagnosis. Cortical atrophy is located in the right anterior temporal lobes (100% anteromedial, 100% anteroinferior, 70% anterolateral), left anterior temporal (90% anteromedial, 90% anteroinferior, 60% anterolateral), right prefrontal (30% basal, 50% dorsolateral, 20% medial) and left prefrontal (20% basal, 30% dorsolateral, 20% medial). CONCLUSIONS: Oral verbal production is influenced by level of education and, in a sample of patients with probable frontotemporal degeneration and logorrhoea, all the patients showed alterations in the anteroinferior and anteromedial regions of the right temporal lobe.

[Biomarkers and PET for assessing the activity of Takayasu's arteritis]. / Biomarcadores y tomografía de emisión de positrones en la valoración de la actividad de la arteritis de Takayasu.

INTRODUCTION: Takayasu's arteritis is a chronic granulomatous vasculitis affecting the medium and large blood vessels that can lead to vascular occlusion. Inflammatory stages occur during the course of the disease. Angiography remains the gold standard examination of choice but less invasive techniques are needed to assess the activity of the disease and to monitor response to treatment. CASE REPORT: We present a case of an asymptomatic 21 year-old female patient with occlusion and stenosis of supra-aortic trunks discovered while performing an ultrasonography for the study of a recent goiter. RESULTS: Angiography confirmed stenosis and vascular occlusions, Takayasu's arteritis being diagnosed. Metalloprotease 9 levels at diagnosis were higher than in control patients and increased when symptoms began. Positron emission tomography performed after starting treatment showed an increase in uptake. CONCLUSIONS: These non-invasive markers could be useful to assess the activity of the disease and even to avoid invasive angiographic examinations.