[Normal variants with an epileptiform appearance in electroencephalograms. A literature review and clinical implications]. / Variantes normales de aspecto epileptiforme en el electroencefalograma. Revisión de la bibliografía e implicaciones clínicas.

Normal epileptiform-like variants or benign epileptiform variants are a diagnostic challenge in the interpretation of electroencephalograms, which require the knowledge and extensive experience of those responsible for the electroencephalographic report. They include a heterogeneous group of findings, some quite uncommon, initially related to epilepsy and various neurological conditions. Most of them are currently considered variants with no pathological significance, and their over-interpretation usually leads to misdiagnosis and the establishment of unnecessary treatments. Prevalence data are variable and usually come from selected populations, so they are difficult to extrapolate to a healthy population. Studies with invasive electrodes and recent series link some of these variants with epilepsy. We aim to review the characteristics and prevalence of the main benign epileptiform variants and to update their clinical significance.

TITLE: Variantes normales de aspecto epileptiforme en el electroencefalograma. Revisión de la bibliografía e implicaciones clínicas.Las variantes normales de aspecto epileptiforme, o variantes epileptiformes benignas, son un reto diagnóstico en la interpretación de los electroencefalogramas que requiere su conocimiento y una amplia experiencia por parte de los responsables del informe electroencefalográfico. Incluyen un grupo heterogéneo de hallazgos, algunos muy infrecuentes, que inicialmente se relacionaron con epilepsia y patologías neurológicas diversas. En la actualidad, la mayoría se consideran variantes sin significado patológico, y su sobreinterpretación habitualmente acarrea diagnósticos erróneos y tratamientos innecesarios. Los datos de prevalencia de estas variantes son muy diversos y proceden habitualmente de poblaciones seleccionadas, por lo que son difícilmente extrapolables a población sana. No obstante, estudios con electrodos invasivos y series más recientes vuelven a asociar algunas de estas variantes con epilepsia. Nuestro objetivo es revisar las características y la prevalencia de las principales variantes epileptiformes benignas y actualizar su significado clínico.

Variantes normales de aspecto epileptiforme en el electroencefalograma. Revisión de la bibliografía e implicaciones clínicas / Normal variants with an epileptiform appearance in electroencephalograms. A literature review and clinical implications

Las variantes normales de aspecto epileptiforme, o variantes epileptiformes benignas, son un reto diagnóstico en la interpretación de los electroencefalogramas que requiere su conocimiento y una amplia experiencia por parte de los responsables del informe electroencefalográfico. Incluyen un grupo heterogéneo de hallazgos, algunos muy infrecuentes, que inicialmente se relacionaron con epilepsia y patologías neurológicas diversas. En la actualidad, la mayoría se consideran variantes sin significado patológico, y su sobreinterpretación habitualmente acarrea diagnósticos erróneos y tratamientos innecesarios. Los datos de prevalencia de estas variantes son muy diversos y proceden habitualmente de poblaciones seleccionadas, por lo que son difícilmente extrapolables a población sana. No obstante, estudios con electrodos invasivos y series más recientes vuelven a asociar algunas de estas variantes con epilepsia. Nuestro objetivo es revisar las características y la prevalencia de las principales variantes epileptiformes benignas y actualizar su significado clínico. (AU)

[Temporal lobe epilepsy. Aetiological classification in 61 paediatric patients]. / Epilepsia del lóbulo temporal. Clasificación etiológica en 61 pacientes en edad pediátrica.

INTRODUCTION: There are very few studies on the aetiology of temporal lobe epilepsy (TLE) in childhood. The purpose of the present study is to analyse the data of 61 children diagnosed with TLE, in order to describe the aetiology of TLE in children seen in a neuropaedriatic clinic. We also discuss the currently proposed classification. PATIENTS AND METHODS: A retrospective analysis was carried out on patients diagnosed with TLE. Patients consisted of 61 children less than 15 years old. RESULTS: Patients were classified into three groups: Group 1 (symptomatic temporal lobe epilepsy) consisted of 25 patients (40.98 %) with any temporal lesion on neuroimaging (tumours, malformations or infections) or significant history; Group 2 (Mesial temporal sclerosis) consisted of 17 patients (27.86 %), a history of simple and complex febrile seizure were common in this group; and Group 3 (Cryptogenic epilepsy) consisted of 19 patients (31.15 %) with no abnormalities on neuroimaging or significant history. CONCLUSION: To our knowledge, this is the largest paediatric series of childhood new-onset TLE assessed only by MRI in the literature. We have modified the previous aetiological classification in order to make the groups more realistic.

[Post-traumatic supraorbital neuralgia: a benign condition]. / Neuralgia supraorbitaria postraumática: una entidad benigna.

INTRODUCTION: Supraorbital neuralgia has only recently been described. Most of the cases reported involve patients suffering from chronic idiopathic neuralgias that are difficult to treat and sometimes require surgery to release the nerve. We present our experience in patients with a variant of this neuralgia which has a known causation, is commonly seen and has a benign prognosis. CASE REPORTS: We studied five patients, four females and one male, with a mean age of 55 years (range: 29-69 years). They had all suffered direct banal traumatic injury to the frontal region due to different causes. Four of them developed continuous, piercing or burning-type pain; three of them had paroxysmal pain and one had itching. There were no autonomic manifestations. All of them were found to be abnormally sensitive in the affected area, with tactile hypaesthesia, hyperalgesia or allodynia and a positive Tinel's sign. Neuroimaging tests were normal. Two patients were treated with gabapentin and amitriptyline. One was treated with an anaesthetic blockade, which afforded temporary relief. Three of them received no treatment at all. After one year of follow-up, all of them had improved and three were no longer in pain, although sensory alterations persisted in all cases. CONCLUSIONS: Post-traumatic supraorbital neuralgia is a frequent condition, although it is probably underdiagnosed. It has its own characteristic clinical and developmental features that distinguish it from idiopathic supraorbital neuralgia. Progress is usually good and it responds favourably to symptomatic treatment, if needed.

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.

[Reversible dementia in neurology outpatient clinics]. / Demencias reversibles en la consulta neurológica extrahospitalaria.

INTRODUCTION: The dementias are one of the commonest conditions seen in Neurology Clinics. Potentially reversible causes are described amongst the various aetiologies although there are doubts as to whether the use of indiscriminate testing to detect them is worthwhile. OBJECTIVE: In a group of demented persons to determine how many had a potentially reversible condition and how many improved, in a prospective, descriptive study in a Neurology Outpatient Clinic. PATIENTS AND METHODS: Using the Mini-Mental State Examination of Folstein and the DSM-III-R criteria for dementia, 121 demented patients were selected. An ordinary biochemical study was made (vitamin B12, thyroid hormones), serology (lues) and neuroimaging (cerebral CT), and when a potentially reversible condition was found, each case was treated. The patients treated were followed-up periodically for an average of 9.6 months (range 2 to 24 months). RESULTS: A potentially reversible condition was seen in 19.8% of the patients. On prospective evaluation only 3.3% had reversible symptoms and in no case were these completely reversed. Improvement was seen in conditions of depressive pseudo-dementia. CONCLUSIONS: Since some causes of dementia are reversible, the neurologist is obliged to investigate and seek these possible aetiologies. This study should be individualized according to clinical criteria to improve profitability of the complementary tests. Our results suggest that there are doubts as to the usefulness of indiscriminate investigation of possible reversible causes of the cases of dementia referred to Outpatient neurologists.

[The importance of neurological examinations in the age of the technological revolution]. / Importancia de la exploración neurológica en la era de la revolución tecnológica.

INTRODUCTION: Neurologic practice and care have been modified in many important ways during the past ten years, to adapt to the explosion of new information and new technology. Students, residents and practicing physicians have been continuing programs to a model that focuses almost exclusively on the applications to neurologic disorders of the new knowledge obtained from biomedical research. On the other hand high demand for outpatient neurologic care prevents adequate patient's evaluation. CASE REPORTS: Case 1: 65 years old female. Occipital headache diagnosed of tensional origin (normal computerized tomography). Two months later is re-evaluated due to intractable pain and hypoglossal lesion. An amplified computerized tomography revealed a occipital condyle metastasis. Case 2: 21 years old female. Clinical suspicion of demyelinating disease due to repeated facial paresis and sensitive disorder. General exploration and computerized tomography revealed temporo-mandibular joint. Case 3: 60 years old female. Valuation of anticoagulant therapy due to repeated transient ischemic attacks. She suffered from peripheral facial palsy related to auditory cholesteatoma. CONCLUSIONS: Neurologic education is nowadays orientated to new technologies. On the other hand, excessive demand prevents adequate valuation and a minute exploration is substituted by complementary evaluations. These situations generate diagnostic mistakes or iatrogenic. It would be important a consideration of the neurologic education profiles and fulfillment of consultations time recommendations for outpatients care.

[Transient cerebral oedema associated to hypoglycaemia]. / Edema cerebral transitorio asociado a hipoglucemia.

INTRODUCTION: In the adult, hypoglycaemia is documented as a consequence of overdose of insulin or oral hypoglycaemic agents. Neonatal hypoglycaemia is common but rarely symptomatic due to protective mechanisms. Very few reports on hypoglycaemic injury are available in adults and most of them in patients with poor outcome. CASE REPORT: Woman, 45 years old. She is referred to emergency room due to insulin overdose and coma of unknown duration. Glucose level was 15 mg% without other metabolic anomalies. Computerized tomography revealed brain oedema most obvious in the parieto-occipital lobes. Therapy with manitol and glucose was started with total functional recovery. CONCLUSIONS: Brain imaging in neonatal hypoglycaemia shows similar findings. In the acute phase oedema in the parieto-occipital cortex and underlying white matter and atrophy or malacic cysts in the chronic phase have been described. Chronic changes are related to long-term sequelae that vary from development delay and epilepsy to persistent vegetative state. In adults lesions involve hippocampus and basal ganglia. The reasons of different sensitivity of these regions are not fully clear. Differences in regional blood flow autoregulation or in excitotoxins receptors of aspartate have been postulated. Hypoglycaemia may induce blood-brain-barrier permeability and subsequently brain oedema.

[Cervical radiculopathy as a form of presentation of non-Hodgkin's lymphoma]. / Radiculopatía cervical como forma de presentación de un linfoma no Hodgkin.

INTRODUCTION: Neurological involvement in no-Hodgkin lymphoma is usually a serious problem leading to subacute compression of the spinal cord secondary to invasion of the spinal extradural space. This condition may be the presenting form of the illness, although relatively infrequently. Onset with involvement of the cauda equina or as an isolated radiculopathy is even rarer and has mainly been described at lumbar level. CLINICAL CASE: We present the case of a 27 year old man with no significant previous clinical history who had progressive, insidious onset of moderate-serious senso-motor radiculopathy at C8. On MR there was an infiltrating mass affecting the nerve root and reaching the spinal extradural space. Following decompression laminectomy the patient's leg recovered and on histology there was a large cell B lymphoma. The other complementary tests showed disseminated disease, although analysis of the cerebro-spinal fluid (including cytology) was normal. HIV seriology was also normal. CONCLUSION: Although isolated nerve root involvement is unusual in the presentation of lymphoma it should be considered to be part of the differential diagnosis of nerve root syndromes in young patients with no history of trauma or degenerative lesions.

[Hypnic headache: a new case]. / Cefalea hípnica: un nuevo caso.

INTRODUCTION: Hypnic headache is a condition characterized by nocturnal episodes of headache which periodically waken the sleeping patient. They usually occur in persons over 55 years of age and are thought to be due to some type of disturbance of biological rhythm. CLINICAL CASE: A 70 year old woman, with no relevant past history, complained that during the previous 12-14 months she had been woken from sleep by episodes of headache. The headache was diffuse, non-pulsatile and very intense. The duration was variable, from 15 to 45 minutes and usually disappeared without requiring analgesics. The pain was not associated with autonomic or ocular disorders, nausea, vomitting or focal neurological signs. Both general and neurological examinations were completely normal. No alterations were seen on cranial CT. Treatment with lithium was started and there was marked improvement in the frequency of headaches. CONCLUSIONS: Hypnic headache is an unusual disorder characterized by episodes of holocranial, or rarely hemicranial, headache of moderate-severe intensity and periodic occurrence. Typically the headache wakes the patient whilst he is asleep ('alarm clock') and there are no autonomic or neurological alterations of any type associated with the episode. The condition usually affects persons over 55 years of age, and it has been related to changes in biological rhythms. This presumed alteration of biological pacemakers has been based on the periodicity of the episodes and the response to lithium.

[MELAS syndrome masquerading as herpes encephalitis: genetic diagnosis]. / Síndrome MELAS que asemeja una encefalitis por virus herpes: diagnóstico genético.

INTRODUCTION: MELAS syndrome (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke like episodes) is a mitochondrial disease related to the mitochondrial DNA mutation. The tRNALeu(UUR) mutation at the base pair 3234 is associated to 80% of cases of MELAS syndrome. The onset with the stroke like episodes is often before the age 40. Herpes simplex encephalitis (HSE) presents as acute episodes of focal neurologic deficit that are often related to the temporal lobe. Occasionally MELAS syndrome and HSE may have a similar clinical presentation. CLINICAL CASE: A 33-year-old woman presented with seizures and a right parietotemporal syndrome of acute onset. The differential diagnosis between HSE and MELAS syndrome was established. MELAS syndrome was suspected based on her phenotype. The diagnosis of MELAS syndrome was confirmed by the presence of the mitochondrial tRNALeu(UUR) mutation at the base pair 3243. CONCLUSION: The clinical presentation of the MELAS syndrome may mimic that of the HSE, and antiviral treatment should be given until the diagnosis of MELAS syndrome is definitive. The study of the mitochondrial DNA helps diagnosing in a non invasive way many patients with MELAS syndrome.

[Idiopathic spinal cord herniation. Presentation of a new case and review of the literature]. / Herniación medular idiopática. Presentación de un nuevo caso y revisión de la literatura.

INTRODUCTION: Idiopathic herniation of the spinal cord is a rarely diagnosed condition. It is important since this cause of paraparesis is potentially curable if diagnosis is made early. Our aim is to report a new case, review the relevant literature, describe the radiological findings and consider the etiopathogenic findings. CLINICAL CASE: We report the case of a 56 year old man with increasing difficulty in walking attributed to stenosis of the lumbar spinal canal, which did not improve after laminectomy. On examination he had sensory and motor deficits compatible with the Brown-Séquard syndrome. Dorsal MR showed ventral displacement of the thoracic spinal cord with disappearance of the anterior subarachnoid space together with a posterior arachnoid cyst at the same site. A further operation was done at the same place and the cyst removed. However, it was impossible to free the spinal cord since there was severe spinal arachnoiditis. The patient made little improvement. CONCLUSIONS: Herniation of the spinal cord is a rarely diagnosed condition. After careful study of the literature it seems that all patients present in a similar manner. Clinically there is a disorder of gait compatible with the Brown-Séquard syndrome. On MR there is sudden ventral displacement of the thoracic spinal cord, associated in some cases with an arachnoid cyst. When surgery is effective there is great improvement, so this diagnosis should be remembered in all cases of progressive paraparesis.

[Usefulness of electroencephalograms in evaluating transient global amnesia]. / Utilidad de la electroencefalografia en la valoracion de la amnesia global transitoria.

INTRODUCTION: Transient global amnesia (TGA) is a perfectly well defined clinical picture, but nevertheless even today its aetiology remains unknown. The three most widely accepted theories suggest it has a vascular origin, it is related with the pathophysiology of migraine or it is of an epileptiform nature. AIM: To analyse whether there is an electroencephalographic pattern that is consistently repeated in a series of electro-encephalograms (EEG) carried out on patients with TGA. PATIENTS AND METHODS: The study consists in a retrospective analysis of a sample of 345 patients referred to have an EEG after an episode of TGA. RESULTS: In almost 20% of the EEGs something that could be considered abnormal was found, although most of these findings (64%) were of little pathological significance. Of the remaining 26%, attention should be drawn to the cases of two patients with subclinical rhythmic electroencephalogram discharges of adults (a pattern with a meaning that is not altogether clear and which has previously been associated with TGA). CONCLUSIONS: A considerable percentage of patients have TGA and EEG alterations, although most of them are of scarce pathological significance or can be attributed to some other underlying condition. We have not succeeded in identifying any pattern that is consistently repeated. Our results suggest that the EEG is a test with low diagnostic effectiveness in this pathology and it is necessary to reconsider the need to systematically perform such tests in suspected cases of TGA.

[Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis]. / Respuesta clinica rapida y espectacular a plasmaferesis en un caso pediatrico de encefalitis anti-NMDA.

INTRODUCTION: Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is being diagnosed more and more frequently in the paediatric age. It should be suspected in children with psychiatric symptoms, encephalopathy, abnormal movements or epileptic seizures. Paraneoplastic cases are less frequent than in adults. CASE REPORT: We report the case of a boy, 2.5 years of age, with subacute encephalopathic signs and symptoms and epileptic seizures followed by behaviour disorders, neurological regression, dyskinesias and insomnia. Results of a cerebrospinal fluid study were normal, the magnetic resonance scan of the head revealed a focal periventricular lesion and diffuse leptomeningeal uptake; moreover, the serial electroencephalograms showed high-amplitude delta activity interspersed with generalised intercritical epileptiform activity. The patient was given empirical treatment with high doses of corticoids and intravenous immunoglobulins with no response. After showing up positive for antibodies against the NMDA receptor, plasmapheresis was begun, which led to his swift and spectacular recovery. After more than 18 months' follow-up, his sequelae are limited to mild behavioural and language alterations. He has had no relapses and has not needed any kind of maintenance treatment. CONCLUSIONS: Anti-NMDA encephalitis is a treatable disorder and, sometimes, the first evidence of an underlying neoplasia, which makes its early recognition and treatment essential. Treatment of the non-paraneoplastic forms are based on immunotherapy: glucocorticoids, intravenous immunoglobulins, plasmapheresis and immunosuppressants. Plasmapheresis can bring about a fast, spectacular improvement.

[The role of ambulatory electroencephalogram monitoring: experience and results in 264 records]. / El papel diagnóstico del Holterelectroencefalograma ambulatorio: experiencia y resultados en 264 registros.

INTRODUCTION: Ambulatory electroencephalogram (EEG) monitoring allows for long-term, mobile electroencephalographic recordings of patients. This study aims to describe and analyze the results obtained with ambulatory EEG in our clinical practice. METHODS: We have analyzed the results of 264 ambulatory EEG records, grouped according to the reason for the request: a) group 1: diagnostic evaluation of episodes of epileptic nature; b) group 2: diagnostic evaluation of paroxysmal episodes, and c) group 3: evaluation of the risk of relapse during anti-seizure treatment withdrawal in certain epileptic patients. RESULTS: a) Group 1 (n=137): normal results were found in 54 records (39.4%). There was generalized epileptic activity in 20 (14.6%) of them (5 with ictal activity) and focal epileptic activity was detected in 57 cases (42%) (8 with ictal activity). No EEG diagnosis could be reached in 6 (4%) recordings due to the presence of artefacts; b) group 2 (n=99): in 47 records (47.5 %), there were no episodes and the Holter-EEG was normal. There was a clinically documented episode without anomalies during Holter-EEG registration in 14 cases (14.2%). In 29 records (29.3%), focal epileptic activity was recorded (ictal 4) and generalized epileptic activity (ictal in 1) was recorded in 4 patients (4%). No EEG diagnosis could be reached in 5 cases (5%), and c) group 3 (n=28): the study was normal in 15 cases (53.6%) and showed focal interictal epileptic activity in 8 (28.6 %) and generalized interictal epileptic activity in 5 of them (17.8%). CONCLUSION: We believe that the ambulatory EEG recordings in correctly selected cases can provide important additional information regarding global assessment of patients with epilepsy.

[Quality of the health care to the epileptic patients in Spain]. / Calidad asistencial en epilepsia. Situación de la asistencia a los pacientes epilépticos en España.

INTRODUCTION: The objective of the study was to evaluate the quality of the health care to the epileptic patient in Spain including recently diagnosed patients, controlled patients and medically refractory patients. METHODS: Throughout years 2001-2002 a questionnaire of consensus was agreed by neurologists from surgical epilepsy units, epilepsy units, community hospitals and outpatient clinics. RESULTS: A total of 139 questionnaires were analysed. Only one third of the hospitals had a specialized epilepsy clinic. The longest waiting lists for diagnostic procedures were video EEG and Holter EEG, with 175 and 97.6 days, respectively. Clear differences between autonomous communities as far as availability of neurologists on duty, availability of diagnostic tests and number of epilepsy units are stated, existing, in general, more resources in the autonomous communities with health transferred before 2002 and Madrid. CONCLUSIONS: As much the lack of resources as the inequalities indicate that we are still far from the quality standards recommended by the International League Against Epilepsy (ILAE), lacking a suitable lanning that eliminates the inequalities and it so approaches us a system of assistance integrated in different levels as it is set out by the international scientific community.

[Guidelines for headaches and dizziness for general physicians: analysis of their impact on visit frequency to outpatient neurological ambulatory services]. / Guías de actuación en cefaleas y mareos para médicos de familia: análisis de su repercusión en la frecuentación de una consulta extrahospitalaria de neurología.

BACKGROUND: The work of neurologists in the Public Health Service is absolutely determined by the demand of the general physicians, who have occasionally been considered to be not too selective. For this reason we have tried to change this demand. METHODS: In our outpatient clinic we have developed some guidelines for the general physicians to orientate them in the selection of patients referred with neurological disease. In this study the impact of the implementation of headache and dizziness guidelines on the annual frequentation from 1993 to 1997 is analysed. We also reviewed 200 cases of patients referred in 1996-1997 and assessed their adequacy to the guidelines, and inquired the general physicians after their demand and percentage of patients with neurologic disease that they referred to the neurologist. RESULTS: There were no significant changes on the demand of consultations on headache or dizziness. We found that 39% of patients referred with headache and 23% of those with dizziness didn't adequate to the guidelines. General physicians referred only 3.3% of the patients seen by them with headache, and 3.7% of those with dizziness. CONCLUSIONS: Sending guidelines to the general physicians has not demonstrated to be effective on improving their selection of patients, and a better implementation of guidelines should be studied, admitting that the care demand of headache and dizziness is high, and that general physicians make an important selective effort.