Demonstration project on epilepsy in Brazil - Outcome assessment

Purpose: To assess the outcome of patients with epilepsy treated at primary care health units under the framework of the demonstration project on epilepsy in Brazil, part of the WHO/ILAE/IBE Global Campaign Against Epilepsy. Method. We assessed the outcome of patients treated at four primary health units. The staff of the health units underwent information training in epilepsy. The outcome assessment was based on: 1) reduction of seizure frequency, 2) subjective perception from the patient's and the physician's point of view, 3) reduction of absenteeism, 4) social integration (school and work), and 5) sense of independence. Results: A total of 181 patients (93 women - 51%) with a mean age of 38 (range from 2 to 86) years were studied. The mean follow-up was 26 months (range from 1 to 38 months, 11 patients had follow-up of less than 12 months). Seizure frequency was assessed based on a score system, ranging from 0 (no seizure in the previous 24 months) to 7 (> 10 seizure/day). The baseline median seizure-frequency score was 3 (one to three seizures per month). At the end of the study the median seizure-frequency score was 1 (one to three seizures per year). The patients' and relatives' opinions were that in the majority (59%) the health status had improved a lot, some (19%) had improved a little, 20% experienced no change and in 2% the health status was worse. With regard to absenteeism, social integration and sense of independence, there were some modest improvements only. Discussion: The development of a model of epilepsy treatment at primary health level based on the existing health system, with strategic measures centred on the health care providers and the community, has proved to be effective providing important reductions in seizure frequency, as well as in general well being. This model can be applied nationwide, as the key elements already exist provided that strategic measures are put forward in accordance with local health providers and managers

Mesial Temporal Lobe Epilepsy Syndrome: An Updated Overview

Mesial temporal lobe epilepsy (MTLE) is the most common form of partial epilepsy in young adults and also the most frequent type of epilepsy reported in surgical series worldwide. Mesial temporal lobe sclerosis (MTS) is the major underlying cause of MTLE, and it is present in 60-70% of patients with MTLE who undergo surgery for treatment of medically refractory seizures. Pathogenetic mechanisms underlying this distinct hippocampal pathology remains undetermined. Recent findings suggest a developmental malformation of hippocampus (inherited or acquired) that in association with subsequent injury (e.g. trauma, infection, complex febrile seizures) could develop ongoing seizures, resulting in the full-blown neuropathological features of MTS. Genetic background, age and type of initial precipitating injury, and vulnerability related to programmed cell death pathways are probable mechanisms involved in the development of MTS. Definitions for medical intractability may vary among centers, but usually include failure to achieve seizure control with two or more AEDs with adequate dosage and posology. The decision as to when one should perform surgery in patients with MTLE is a relevant issue that needs more investigation. Recent evidence discussed in this review indicates that longer duration of uncontrolled seizures is associated with an increased risk of unsuccessful surgery.113141144Cendes F, Kahane P, Brodie MJ, Andermann F. The mesio-temporal lobe epilepsy syndrome. In: Roger J, Bureau M, Dravet C, Genton P, Tassinari CA, Wolf P, editors. Epileptic syndromes in infancy, childhood and adolescence. 3 rd ed. Eastleigh UK: John Libbey &ampCo Ltd2002. p.513-30Kobayashi, E., Lopes-Cendes, I., Guerreiro, C.A., Sousa, S.C., Guerreiro, M.M., Cendes, F., Seizure outcome and hippocampal atrophy in familial mesial temporal lobe epilepsy (2001) Neurology, 56, pp. 166-172Gloor, P., Mesial temporal sclerosis: Historical background and an overview from a modern perspective (1991) Epilepsy surgery, pp. 689-703. , Luders H, editor, New York: Raven Press;Meencke, H.J., Veith, G., Hippocampal sclerosis in epilepsy (1991) Epilepsy surgery, pp. 705-715. , Luders H, ed, New York: Raven Press;Bruton, C.J., (1988) The neuropathology of temporal lobe epilepsy, , New York: Oxford University Press;Camfield, C.S., Camfield, P.R., Gordon, K., Wirrell, E., Dooley, J.M., Incidence of epilepsy in childhood and adolecesce - A population-based study in Nova Scotia from 1977 to 1985 (1996) Epilepsia, 37, pp. 19-23Camfield, P., Camfield, C., Gordon, K., Dooley, J., What types of epilepsy are preceded by febrile seizures? A population-based study of children (1994) Dev Med Child Neurol, 36, pp. 887-892Cendes, F., Febrile seizures and mesial temporal sclerosis (2004) Curr Opin Neurol, 17, pp. 161-164VanLandingham, K.E., Heinz, E.R., Cavazos, J.E., Lewis, D.V., Magnetic resonance imaging evidence of hippocampal injury after prolonged focal febrile convulsions (1998) Ann Neurol, 43, pp. 413-426Kobayashi, E., D'Agostino, M.D., Lopes-Cendes, I., Berkovic, S.F., Li, M.L., Andermann, E., Hippocampal atrophy and T2-weighted signal changes in familial mesial temporal lobe epilepsy (2003) Neurology, 60, pp. 405-409Cendes, F., Progressive hippocampal and extrahippocampal atrophy in drug resistant epilepsy (2005) Curr Opin Neurol, 18, pp. 173-177Blumcke, I., Thom, M., Wiestler, O.D., Ammon's horn sclerosis: A maldevelopmental disorder associated with temporal lobe epilepsy (2002) Brain Pathol, 12, pp. 199-211Mathern, G.W., Pretorius, J.K., Babb, T.L., Influence of the type of initial precipitating injury and at what age it occurs on course and outcome in patients with temporal lobe seizures (1995) J Neurosurg, 82, pp. 220-227Mathern, G.W., Babb, T.L., Leite, J.P., Pretorius, K., Yeoman, K.M., Kuhlman, P.A., The pathogenic and progressive features of chronic human hippocampal epilepsy (1996) Epilepsy Res, 26, pp. 151-161Shinoda, S., Schindler, C.K., Meller, R., So, N.K., Araki, T., Yamamoto, A., Bim regulation may determine hippocampal vulnerability after injurious seizures and in temporal lobe epilepsy (2004) J Clin Invest, 113, pp. 1059-1068Kobayashi, E., Li, L.M., Lopes-Cendes, I., Cendes, F., Magnetic resonance imaging evidence of hippocampal sclerosis in asymptomatic, first-degree relatives of patients with familial mesial temporal lobe epilepsy (2002) Arch Neurol, 59, pp. 1891-1894Kim, W.J., Park, S.C., Lee, S.J., Lee, J.H., Kim, J.Y., Lee, B.I., The prognosis for control of seizures with medications in patients with MRI evidence for mesial temporal sclerosis (1999) Epilepsia, 40, pp. 290-293Montenegro, M.A., Ferreira, C.M., Cendes, F., Li, L.M., Guerreiro, C.A., Clobazam as add-on therapy for temporal lobe epilepsy and hippocampal sclerosis (2005) Can J Neurol Sci, 32, pp. 93-96Rodin, E.A., (1968) The prognosis of patients with epilepsy, , Springfield: Charles C. Thomas;Sillanpaa, M., Remission of seizures and predictors of intractability in long-term follow-up (1993) Epilepsia, 34, pp. 930-936Camfield, C., Camfield, P., Smith, B., Gordon, K., Dooley, J., Biologic factors as predictors of social outcome of epilepsy in intellectually normal children: A population-based study (1993) J Pediatr, 122, pp. 869-873Semah, F., Picot, M.C., Adam, C., Broglin, D., Arzimanoglou, A., Bazin, B., Is the underlying cause of epilepsy a major prognostic factor for recurrence? (1998) Neurology, 51, pp. 1256-1262Wiebe, S., Blume, W.T., Girvin, J.P., Eliasziw, M., A randomized, controlled trial of surgery for temporal-lobe epilepsy (2001) N Engl J Med, 345, pp. 311-318Yoon, H.H., Kwon, H.L., Mattson, R.H., Spencer, D.D., Spencer, S.S., Long-term seizure outcome in patients initially seizure-free after resective epilepsy surgery (2003) Neurology, 61, pp. 445-450Trevathan, E., Gilliam, F., Lost years: Delayed referral for surgically treatable epilepsy (2003) Neurology, 61, pp. 432-433Gilliam, F., Kuzniecky, R., Meador, K., Martin, R., Sawrie, S., Viikinsalo, M., Patient-oriented outcome assessment after temporal lobectomy for refractory epilepsy (1999) Neurology, 53, pp. 687-694Berg, A.T., Langfitt, J., Shinnar, S., Vickrey, B.G., Sperling, M.R., Walczak, T., How long does it take for partial epilepsy to become intractable? (2003) Neurology, 60, pp. 186-19

Demonstration project on epilepsy in Brazil - Situation assessment

Purpose: To provide a situation assessment of services for people with epilepsy in the context of primary health care, as part of the Demonstration Project on Epilepsy in Brazil, part of the WHO/ILAE/IBE Global Campaign 'Epilepsy out of the shadows'. Methods: We performed a door-to-door epidemiological survey in three areas to assess the prevalence of epilepsy and its treatment gap. We surveyed a sample of 598 primary health care workers from different regions of Brazil to assess their perceptions of the management of people with epilepsy in the primary care setting. Results: The lifetime prevalence of epilepsy was 9.2/1,000 people [95% Cl 8.4-10.0] and the estimated prevalence of active epilepsy was 5.4/1,000 people. Thirty-eight percent of patients with active epilepsy were on inadequate treatment, including 19% who were taking no medication. The survey of health workers showed that they estimated that 60% of patients under their care were seizure-free. They estimated that 55% of patients were on monotherapy and that 59% had been referred to neurologists. The estimated mean percentage of patients who were working or studying was 56%. Most of the physicians (73%) did not feel confident in managing people with epilepsy. Discussion: The epidemiological survey in the areas of the Demonstration Project showed that the prevalence of epilepsy is similar to that in other resource-poor countries, and that the treatment gap is high. One factor contributing to the treatment gap is inadequacy of health care delivery. The situation could readily be improved in Brazil, as the primary health care system has the key elements required for epilepsy management. To make this effective and efficient requires: i) an established referral network, ii) continuous provision of AEDs, iii) close monitoring of epilepsy management via the notification system (Sistema de lnformacao da Atencao Basica - SIAB) and iv) continuous education of health professionals. The educational program should be broad spectrum and include not only medical management, but also psycho-social aspects of epilepsy

Espectroscopia De Fósforo Por Ressonância Magnética Na Investigação De Epilepsia Do Lobo Temporal: lendo Nas Entrelinhas Das Alterações Metabólicas

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Striatal and extrastriatal atrophy in Huntington's disease and its relationship with length of the CAG repeat

Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that affects the striatum most severely. However, except for juvenile forms, relative preservation of the cerebellum has been reported. The objective of the present study was to perform MRI measurements of caudate, putamen, cerebral, and cerebellar volumes and correlate these findings with the length of the CAG repeat and clinical parameters. We evaluated 50 consecutive patients with HD using MRI volumetric measurements and compared them to normal controls. Age at onset of the disease ranged from 4 to 73 years (mean: 43.1 years). The length of the CAG repeat ranged from 40 to 69 (mean: 47.2 CAG). HD patients presented marked atrophy of the caudate and putamen, as well as reduced cerebellar and cerebral volumes. There was a significant correlation between age at onset of HD and length of the CAG repeat, as well as clinical disability and age at onset. The degree of basal ganglia atrophy correlated with the length of the CAG repeat. There was no correlation between cerebellar or cerebral volume and length of the CAG repeat. However, there was a tendency to a positive correlation between duration of disease and cerebellar atrophy. While there was a negative correlation of length of the CAG repeat with age at disease onset and with striatal degeneration, its influence on extrastriatal atrophy, including the cerebellum, was not clear. Extrastriatal atrophy occurs later in HD and may be related to disease duration.1129113

Thalamic metabolic abnormalities in patients with Huntington's disease measured by magnetic resonance spectroscopy

Huntington's disease (HD) is a neurologic disorder that is not completely understood; its fundamental physiological mechanisms and chemical effects remain somewhat unclear. Among these uncertainties, we can highlight information about the concentrations of brain metabolites, which have been widely discussed. Concentration differences in affected, compared to healthy, individuals could lead to the development of useful tools for evaluating the progression of disease, or to the advance of investigations of different/alternative treatments. The aim of this study was to compare the thalamic concentration of metabolites in HD patients and healthy individuals using magnetic resonance spectroscopy. We used a 2.0-Tesla magnetic field, repetition time of 1500 ms, and echo time of 135 ms. Spectra from 40 adult HD patients and 26 control subjects were compared. Quantitative analysis was performed using the LCModel method. There were statistically significant differences between HD patients and controls in the concentrations of N-acetylaspartate+N-acetylaspartylglutamate (NAA+NAAG; t-test, P<0.001), and glycerophosphocholine+phosphocholine (GPC+PCh; t-test, P=0.001) relative to creatine+phosphocreatine (Cr+PCr). The NAA+NAAG/Cr+PCr ratio was decreased by 9% and GPC+PCh/Cr+PCr increased by 17% in patients compared with controls. There were no correlations between the concentration ratios and clinical features. Although these results could be caused by T1 and T2 changes, rather than variations in metabolite concentrations given the short repetition time and long echo time values used, our findings point to thalamic dysfunction, corroborating prior evidence.72272

Late Onset Temporal Lobe Epilepsy With Mri Evidence Of Mesial Temporal Sclerosis Following Acute Neurocysticercosis: Case Report.

The objective of this case report is to describe magnetic resonance imaging (MRI) evidence of mesial temporal sclerosis (MTS) in a patient with new onset temporal lobe epilepsy (TLE) and acute neurocysticercosis with multiple cysts. A 56 years old man with new onset headache, Simple Partial Seizures and Complex Partial Seizures underwent CT scan and lumbar puncture as diagnose proceeding. Multiple cysts and meningitis were identified, with a positive immunology for cysticercosis. Seizures were recorded over the left temporal region in a routine EEG. Treatment with albendazole was performed for 21 days, with clinical improvement and seizure remission after 4 months. An MRI scan 11 months after treatment, showed complete resolution of those cystic lesions and a left hippocampal atrophy (HA) with hyperintense T2 signal. The presence of HA and hyperintense T2 signal in this patient has not, to date, been associated with a poor seizure control. This patient presented with MRI evidence of left MTS after new onset partial seizures of left temporal lobe origin. Although we did not have a previous MRI scan, it is likely that this hippocampal abnormality was due to the acute inflammatory response to cysticercosis associated to repeated partial seizures. This suggests that acute neurocysticercosis associated with repeated seizures may cause MTS and late onset TLE.59255-

Phosphorus magnetic resonance spectroscopy in the investigation of temporal lobe epilepsy: ‘reading between the lines’ of metabolic abnormalities

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International Veterinary Epilepsy Task Force recommendations for a veterinary epilepsy-specific MRI protocol

Epilepsy is one of the most common chronic neurological diseases in veterinary practice. Magnetic resonance imaging (MRI) is regarded as an important diagnostic test to reach the diagnosis of idiopathic epilepsy. However, given that the diagnosis requires the exclusion of other differentials for seizures, the parameters for MRI examination should allow the detection of subtle lesions which may not be obvious with existing techniques. In addition, there are several differentials for idiopathic epilepsy in humans, for example some focal cortical dysplasias, which may only apparent with special sequences, imaging planes and/or particular techniques used in performing the MRI scan. As a result, there is a need to standardize MRI examination in veterinary patients with techniques that reliably diagnose subtle lesions, identify post-seizure changes, and which will allow for future identification of underlying causes of seizures not yet apparent in the veterinary literature. There is a need for a standardized veterinary epilepsy-specific MRI protocol which will facilitate more detailed examination of areas susceptible to generating and perpetuating seizures, is cost efficient, simple to perform and can be adapted for both low and high field scanners. Standardisation of imaging will improve clinical communication and uniformity of case definition between research studies. A 6–7 sequence epilepsy-specific MRI protocol for veterinary patients is proposed and further advanced MR and functional imaging is reviewed

The Importance Of Accurate Anatomic Assessment For The Volumetric Analysis Of The Amygdala.

There is a wide range of values reported in volumetric studies of the amygdala. The use of single plane thick magnetic resonance imaging (MRI) may prevent the correct visualization of anatomic landmarks and yield imprecise results. To assess whether there is a difference between volumetric analysis of the amygdala performed with single plane MRI 3-mm slices and with multiplanar analysis of MRI 1-mm slices, we studied healthy subjects and patients with temporal lobe epilepsy. We performed manual delineation of the amygdala on T1-weighted inversion recovery, 3-mm coronal slices and manual delineation of the amygdala on three-dimensional volumetric T1-weighted images with 1-mm slice thickness. The data were compared using a dependent t-test. There was a significant difference between the volumes obtained by the coronal plane-based measurements and the volumes obtained by three-dimensional analysis (P < 0.001). An incorrect estimate of the amygdala volume may preclude a correct analysis of the biological effects of alterations in amygdala volume. Three-dimensional analysis is preferred because it is based on more extensive anatomical assessment and the results are similar to those obtained in post-mortem studies.38409-1