Global cognitive performance is associated with sleep efficiency measured by polysomnography in patients with Parkinson's disease.

AIM: Sleep disorders can be associated with an increased risk for cognitive decline in patients with Parkinson's disease (PD). The aim of this study was to examine the association between cognitive status and presence of sleep symptoms and sleep disorders in PD patients. METHODS: We evaluated excessive sleepiness, other sleep symptoms, and performed polysomnography and neuropsychological evaluation in 79 patients. They were classified as having normal cognition (PDNC), mild cognitive impairment (PDMCI), or dementia (PDD). RESULTS: There were 29 PDNC, 39 PDMCI, and 11 PDD patients. PDD patients were older, had higher scores on the Unified Parkinson's Disease Rating Scale, and lower Schwab and England Activities of Daily Living scores than PDNC patients. After analysis of the polysomnographic variables, it was also found that PDD patients had a lower sleep efficiency, lower total sleep time, and lower number of sleep state changes than PDNC patients. In a stepwise analysis, defining Mattis Dementia Rating Scale scores as the dependent variable, the results were a model that selected three variables that accounted for 59% of the variation in the Mattis Dementia Rating Scale score: wake time after sleep onset, number of state changes, and schooling. CONCLUSION: We found a significant association between global cognitive performance and wake time after sleep onset and the number of state changes during sleep measured in the polysomnography of PD patients. However, we did not find any other association between sleep disorders or symptoms and cognitive status or cognitive performance of PD patients.

Sleep-disordered breathing among acute ischemic stroke patients in Brazil.

OBJECTIVES: Sleep-disordered breathing (SDB) is very common in acute stroke patients and has been related to poor outcome. However, there is a lack of data about the association between SDB and stroke in developing countries. The study aims to characterize the frequency and severity of SDB in Brazilian patients during the acute phase of ischemic stroke; to identify clinical and laboratorial data related to SDB in those patients; and to assess the relationship between sleep apnea and functional outcome after six months of stroke. METHODS: Clinical data and laboratorial tests were collected at hospital admission. The polysomnography was performed on the first night after stroke symptoms onset. Functional outcome was assessed by the modified Rankin Scale (mRS). RESULTS: We prospectively evaluated 69 patients with their first-ever acute ischemic stroke. The mean apnea-hypopnea index (AHI) was 37.7 ± 30.2. Fifty-three patients (76.8%) exhibited an AHI ≥ 10 with predominantly obstructive respiratory events (90.6%), and thirty-three (47.8%) had severe sleep apnea. Age (OR: 1.09; 95% CI: 1.03-1.15; p= 0.004) and hematocrit (OR: 1.18; 95% CI: 1.03-1.34; p= 0.01) were independent predictors of sleep apnea. Age (OR: 1.13; 95% CI: 1.03-1.24; p= 0.01), body mass index (OR: 1.54; 95% CI: 1.54-2.18; p= 0.01), and hematocrit (OR: 1.19; 95% CI: 1.01-1.40; p= 0.04) were independent predictors of severe sleep apnea. The National Institutes of Health Stroke Scale (NIHSS; OR: 1.30; 95% CI: 1.1-1.5; p= 0.001) and severe sleep apnea (OR: 9.7; 95% CI: 1.3-73.8; p= 0.03) were independently associated to mRS >2 at six months, after adjusting for confounders. CONCLUSION: Patients with acute ischemic stroke in Brazil have a high frequency of SDB. Severe sleep apnea is associated with a poor long-term functional outcome following stroke in that population.

SOS score: an optimized score to screen acute stroke patients for obstructive sleep apnea.

BACKGROUND: Obstructive sleep apnea (OSA) is frequent in acute stroke patients, and has been associated with higher mortality and worse prognosis. Polysomnography (PSG) is the gold standard diagnostic method for OSA, but it is impracticable as a routine for all acute stroke patients. We evaluated the accuracy of two OSA screening tools, the Berlin Questionnaire (BQ), and the Epworth Sleepiness Scale (ESS) when administered to relatives of acute stroke patients; we also compared these tools against a combined screening score (SOS score). METHODS: Ischemic stroke patients were submitted to a full PSG at the first night after onset of symptoms. OSA severity was measured by apnea-hypopnea index (AHI). BQ and ESS were administered to relatives of stroke patients before the PSG and compared to SOS score for accuracy and C-statistics. RESULTS: We prospectively studied 39 patients. OSA (AHI ≥10/h) was present in 76.9%. The SOS score [area under the curve (AUC): 0.812; P = 0.005] and ESS (AUC: 0.789; P = 0.009) had good predictive value for OSA. The SOS score was the only tool with significant predictive value (AUC: 0.686; P = 0.048) for severe OSA (AHI ≥30/h), when compared to ESS (P = 0.119) and BQ (P = 0.191). The threshold of SOS ≤10 showed high sensitivity (90%) and negative predictive value (96.2%) for OSA; SOS ≥20 showed high specificity (100%) and positive predictive value (92.5%) for severe OSA. CONCLUSIONS: The SOS score administered to relatives of stroke patients is a useful tool to screen for OSA and may decrease the need for PSG in acute stroke setting.

Adherence of obstructive sleep apnea syndrome patients to continuous positive airway pressure in a public service.

INTRODUCTION: The standard therapy for obstructive sleep apnea syndrome (OSAS) is continuous positive airway pressure (CPAP), but its correct and frequent use is essential to control the disease. PURPOSE: To analyze adherence to CPAP among patients with OSAS treated in a multidisciplinary outpatient clinic of a public tertiary hospital. METHODS: This was a retrospective study evaluating 156 patients with OSAS who underwent polysomnography for CPAP titration from 2008 to 2011. The patients were divided into two groups, those with good adherence to CPAP (a mean use of four or more hours per night) and those with poor adherence. The groups were compared regarding the following data: gender, age, body mass index, associated diseases, AHI at diagnostic polysomnography, and pressure (cmH2O) suggested by titration polysomnography. RESULTS: 125 patients were analyzed, and it was observed that 82 of the patients (65%) had good adherence, whereas 43 (35%) showed poor adherence. Comparison between groups revealed that patients with a higher apnea-hypopnea index (AHI) were those who better adhered to treatment with CPAP. CONCLUSIONS: the rate of adherence to CPAP among OSAS patients undergoing clinical monitoring at a public service was 65%. Patients with a higher AHI were those who adhered better to treatment with CPAP.

Adesão de pacientes com síndrome da apneia obstrutiva do sono à pressão positiva contínua em via aérea em um serviço público / Adherence of obstructive sleep apnea syndrome patients to continuous positive airway pressure in a public service

Introdução: A terapia padrão para tratamento da síndrome da apneia obstrutiva do sono (SAOS) é o aparelho de pressão positiva contínua em via aérea (CPAP); todavia, seu uso correto e frequente é determinante para o controle da doença. Objetivo: Analisar a adesão ao CPAP entre pacientes com SAOS tratados em um ambulatório multidisciplinar em um hospital público. Métodos: Estudo retrospectivo avaliando 156 pacientes com SAOS que foram submetidos à polissonografia para titulação de CPAP de 2008 a 2011. Os pacientes foram divididos em dois grupos: os com boa e os com má adesão. Os grupos foram comparados entre si em relação aos seus dados: sexo, idade, IMC, doenças associadas, IAH à polissonografia diagnóstica e pressão em (cmH20) sugerida à polissonografia de titulação. Resultados: Foram analisados 125 pacientes, demonstrando que 82 dels (65%) tinham boa adesão, enquanto 43 (35%) eram maus usuários. A comparação entre os grupos mostrou que os pacientes com maior índice de apneia e hipopneias (IAH) eram os com melhor adesão ao tratamento com CPAP. Conclusões: A taxa de adesão ao CPAP entre os pacientes com SAOS monitorizados regularmente em um serviço público foi de 65%. Os pacientes com pior IAH foram os mais aderentes ao tratamento com CPAP. .

Introduction: The standard therapy for obstructive sleep apnea syndrome (OSAS) is continuous positive airway pressure (CPAP), but its correct and frequent use is essential to control the disease. Purpose: To analyze adherence to CPAP among patients with OSAS treated in a multidisciplinary outpatient clinic of a public tertiary hospital. Methods: This was a retrospective study evaluating 156 patients with OSAS who underwent polysomnography for CPAP titration from 2008 to 2011. The patients were divided into two groups, those with good adherence to CPAP (a mean use of four or more hours per night) and those with poor adherence. The groups were compared regarding the following data: gender, age, body mass index, associated diseases, AHI at diagnostic polysomnography, and pressure (cmH2O) suggested by titration polysomnography. Results: 125 patients were analyzed, and it was observed that 82 of the patients (65%) had good adherence, whereas 43 (35%) showed poor adherence. Comparison between groups revealed that patients with a higher apnea-hypopnea index (AHI) were those who better adhered to treatment with CPAP. Conclusions: the rate of adherence to CPAP among OSAS patients undergoing clinical monitoring at a public service was 65%. Patients with a higher AHI were those who adhered better to treatment with CPAP. .

The use of a mandibular repositioning device for obstructive sleep apnea.

The objective of this study was to verify the effect of a mandibular repositioning device (MRD) on polysomnographic parameters and on the mean electromyographic activity of the masseter and temporal muscles in individuals with obstructive sleep apnea syndrome (OSAS). This is a prospective cohort study conducted at multidisciplinary OSAS center in a tertiary referral center. Nineteen individuals with mild or moderate OSAS associated with Mallampati 3-4 were treated with an MRD during sleep. The subjects underwent diurnal electromyography (EM) and nocturnal polysomnography (PSG) examinations both prior and after initial treatment (3 months with MRD for PSG and 6 and 12 months of treatment for EM). The examinations performed at different times were compared. Comparison of the initial and final polysomnography examination revealed a significant mean reduction of apnea-hypopnea index (AHI) from 13.8 to 7.8. The successful treatment rate with the MRD was 52.6%, and the improved treatment rate was 68.4%. Patients with lower pre-treatment AHI presented higher rates of cure. There was no statistically significant change in electromyography examination among different times. The MRD reduced the apnea-hypopnea index in individuals with enlarged base of tongue and mild and moderate OSAS without damaging the function of the masseter and temporal muscles as determined by electromyography.

Predictors of uvulopalatopharyngoplasty success in the treatment of obstructive sleep apnea syndrome.

OBJECTIVE: Uvulopalatopharyngoplasty (UPPP) has been described as an option for treating obstructive sleep apnea syndrome (OSAS), with variable success rates. The main purpose of our study was to correlate UPPP success to craniofacial bony structure and orofacial muscles function. METHODS: Clinical variables, including body mass index (BMI), age, and preoperative apnea-hypopnea index (AHI); cephalometric measurements of the craniofacial region and hyoid bone position; and muscle function variables including clinical protocol and tongue strength measures were evaluated in 54 patients who underwent UPPP in the last 7years. The measurements were related to the success or failure of UPPP based on the results of preoperative and postoperative polysomnography (PSG). RESULTS: The variables BMI, preoperative AHI, and cephalometric measurements showed no influence on surgical success. The clinical muscle protocol also was similar between groups. However, the muscle strength of the anterior portion of the tongue was significantly greater in the group that showed surgical success compared to those with surgical failure. CONCLUSION: OSAS is a multifactorial disease and diagnostic symptom assessments should be individualized. In addition, special attention should be given to functional muscle alterations of the airways, as they might influence the evolution of the disease.

Polysomnographic evaluation of propofol-induced sleep in patients with respiratory sleep disorders and controls.

OBJECTIVES/HYPOTHESIS: The treatment for obstructive sleep apnea syndrome (OSAS) depends on correct localization of upper airway obstruction, exception made for continuous positive airway pressure (CPAP). Drug-induced sleep endoscopy (DISE) with propofol allows this evaluation, but the drug effects on sleep parameters are not yet well established. Our objective was to study by polysomnography (PSG) whether propofol would change sleep parameters by means of a prospective cross-sectional clinical study in a tertiary hospital. STUDY DESIGN: Thirty non-obese subjects (6 controls and 24 OSAS patients) underwent two daytime PSGs, one with DISE and the other without DISE. METHODS: During DISE exam, propofol was administered intravenously in continuous infusion using a target-controlled infusion pump. The parameters evaluated were: presence of snoring, apnea-hypopnea index (AHI), oxyhemoglobin saturation (SaO2), and sleep macroarchitecture. RESULTS: Snoring was absent in all healthy subjects during DISE sleep with propofol, and present in all OSAS patients (100%). AHI and mean SaO2 showed no statistical difference between the two tests, with and without propofol. However, minimum SaO2 was significantly lower during propofol infusion (88.64 for without vs. 85.04 for with propofol; P < 0.01). Regarding sleep macroarchitecture, the tests with propofol significantly increased N3 sleep and totally extinguished REM sleep (P < 0.005). CONCLUSIONS: The results demonstrate that propofol significantly changes sleep macroarchitecture. However, the main respiratory parameters, AHI and mean SaO2 , remained unaffected. Thus, in order to determine the sites of obstruction, propofol DISE used with target-controlled infusion proved to be an effective drug for endoscopic evaluation of patients with OSAS.

Cognitive and surgical outcome in mesial temporal lobe epilepsy associated with hippocampal sclerosis plus neurocysticercosis: a cohort study.

BACKGROUND: Where neurocysticercosis (NCC) is endemic, chronic calcified neurocysticercosis (cNCC) can be observed in patients with mesial temporal lobe epilepsy associated with hippocampal sclerosis (MTLE-HS). Considering that both disorders cause recurrent seizures or cognitive impairment, we evaluated if temporal lobectomy is cognitively safe and effective for seizure control in MTLE-HS plus cNCC. METHODS: Retrospective cohort study of neuropsychological profile and surgical outcome of 324 MTLE-HS patients submitted to temporal lobectomy, comparing the results according to the presence or absence of cNCC. FINDINGS: cNCC occurred in 126 (38.9%) of our MTLE-HS patients, a frequency higher than expected, more frequently in women than in men (O.R. = 1.66; 95% C.I. = 1.05-2.61; p = 0.03). Left-side (but not right side) surgery caused impairment in selected neuropsychological tests, but this impairment was not accentuated by the presence of cNCC. Ninety-four (74.6%) patients with MTLE-HS plus cNCC and 153 patients (77.3%) with MTLE-HS alone were Engel class I after surgery (O.R. = 1.16; 95% C.I. = 0.69-1.95; p = 0.58). However, the chances of Engel class IA were significantly lower in MTLE-HS plus cNCC than in patients with MTLE-HS alone (31.7% versus 48.5%; O.R. = 2.02; 95% C.I. = 1.27-3.23; p = 0.003). Patients with MTLE-HS plus cNCC showed higher rates of Engel class ID (15.1% versus 6.6%; O.R. = 2.50; 95% C.I. = 1.20-5.32; p = 0.012). INTERPRETATION: cNCC can be highly prevalent among MTLE-HS patients living in areas where neurocysticercosis is endemic, suggesting a cause-effect relationship between the two diseases. cNCC does not add further risk for cognitive decline after surgery in MTLE-HS patients. The rates of Engel class I outcome were very similar for the two groups; however, MTLE-HS plus cNCC patients achieved Engel IA status less frequently, and Engel ID status more frequently. Temporal lobectomy can be safely performed in most patients with MTLE-HS plus cNCC without affecting cognitive outcome. Long-term surgical seizure control in MTLE-HS plus cNCC is still satisfactory, as long as selected patients remain under medication.

Supine sleep and positional sleep apnea after acute ischemic stroke and intracerebral hemorrhage

OBJECTIVE: Obstructive sleep apnea is frequent during the acute phase of stroke, and it is associated with poorer outcomes. A well-established relationship between supine sleep and obstructive sleep apnea severity exists in non-stroke patients. This study investigated the frequency of supine sleep and positional obstructive sleep apnea in patients with ischemic or hemorrhagic stroke. METHODS: Patients who suffered their first acute stroke, either ischemic or hemorrhagic, were subjected to a full polysomnography, including the continuous monitoring of sleep positions, during the first night after symptom onset. Obstructive sleep apnea severity was measured using the apnea-hypopnea index, and the NIHSS measured stroke severity. RESULTS: We prospectively studied 66 stroke patients. The mean age was 57.6±11.5 years, and the mean body mass index was 26.5±4.9. Obstructive sleep apnea (apnea-hypopnea index >5) was present in 78.8% of patients, and the mean apnea-hypopnea index was 29.7±26.6. The majority of subjects (66.7%) spent the entire sleep time in a supine position, and positional obstructive sleep apnea was clearly present in the other 23.1% of cases. A positive correlation was observed between the NIHSS and sleep time in the supine position (r s = 0.5; p<0.001). CONCLUSIONS: Prolonged supine positioning during sleep was highly frequent after stroke, and it was related to stroke severity. Positional sleep apnea was observed in one quarter of stroke patients, which was likely underestimated during the acute phase of stroke. The adequate positioning of patients during sleep during the acute phase of stroke may decrease obstructive respiratory events, regardless of the stroke subtype.

Supine sleep and positional sleep apnea after acute ischemic stroke and intracerebral hemorrhage.

OBJECTIVE: Obstructive sleep apnea is frequent during the acute phase of stroke, and it is associated with poorer outcomes. A well-established relationship between supine sleep and obstructive sleep apnea severity exists in non-stroke patients. This study investigated the frequency of supine sleep and positional obstructive sleep apnea in patients with ischemic or hemorrhagic stroke. METHODS: Patients who suffered their first acute stroke, either ischemic or hemorrhagic, were subjected to a full polysomnography, including the continuous monitoring of sleep positions, during the first night after symptom onset. Obstructive sleep apnea severity was measured using the apnea-hypopnea index, and the NIHSS measured stroke severity. RESULTS: We prospectively studied 66 stroke patients. The mean age was 57.6 ± 11.5 years, and the mean body mass index was 26.5 ± 4.9. Obstructive sleep apnea (apnea-hypopnea index ≥5) was present in 78.8% of patients, and the mean apnea-hypopnea index was 29.7 ± 26.6. The majority of subjects (66.7%) spent the entire sleep time in a supine position, and positional obstructive sleep apnea was clearly present in the other 23.1% of cases. A positive correlation was observed between the NIHSS and sleep time in the supine position (r(s) = 0.5; p<0.001). CONCLUSIONS: Prolonged supine positioning during sleep was highly frequent after stroke, and it was related to stroke severity. Positional sleep apnea was observed in one quarter of stroke patients, which was likely underestimated during the acute phase of stroke. The adequate positioning of patients during sleep during the acute phase of stroke may decrease obstructive respiratory events, regardless of the stroke subtype.

Mortality in children with severe epilepsy: 10 years of follow-up.

Epilepsy is the main neurological condition in children and adolescents. Unfortunately patients with medical refractory epilepsy are more susceptible for clinical complications and death. We report a prospectively evaluated cohort of children followed for approximately 10 years. Fifty-three of 1012 patients died. Forty-two patients died due to epilepsy or its clinical complications and the main causes of death were pneumonia (in 16 cases), sepsis (in 9 patients), status epilepticus (in 8 patients). In 11 patients cause of death was sudden unexpected death in epilepsy (SUDEP). Mental retardation was significantly more frequent in patients who did not die from SUDEP. SUDEP may be a significant condition associated with mortality in children and adolescents with epilepsy.

Mortality in children with severe epilepsy: 10 years of follow-up / Mortalidade em crianças com epilepsia severa: 10 anos de seguimento

Epilepsy is the main neurological condition in children and adolescents. Unfortunately patients with medical refractory epilepsy are more susceptible for clinical complications and death. We report a prospectively evaluated cohort of children followed for approximately 10 years. Fifty-three of 1012 patients died. Forty-two patients died due to epilepsy or its clinical complications and the main causes of death were pneumonia (in 16 cases), sepses (in 9 patients), status epilepticus (in 8 patients). In 11 patients cause of death was sudden unexpected death in epilepsy (SUDEP). Mental retardation was significantly more frequent in patients who did not die from SUDEP. SUDEP may be a significant condition associated with mortality in children and adolescents with epilepsy.

Epilepsia é uma das condições neurológicas mais comuns em crianças e adolescentes. Infelizmente, pacientes com epilepsias refratárias ao tratamento medicamentoso estão mais susceptíveis a complicações clínicas e óbito. Neste trabalho reportamos, em uma análise prospectiva, um cohort de crianças acompanhadas por aproximadamente dez anos. Cinquenta e três de 1012 pacientes foram a óbito. Quarenta e dois pacientes morreram em decorrência da epilepsia ou de suas complicações clínicas; as principais causas foram pneumonia (16 casos), sepse (9 casos) e estado de mal epiléptico (8 casos). Em 11 pacientes a causa da morte foi SUDEP. A presença de retardo mental foi significantemente associada a mortalidade em crianças e adolescentes com epilepsia.

Cephalometric evaluation of facial pattern and hyoid bone position in children with obstructive sleep apnea syndrome.

OBJECTIVES: To assess the development of face and hyoid bone in children with obstructive sleep apnea syndrome (OSAS) through lateral cephalometries. MATERIALS AND METHODS: Children aged 7-10 years with mixed dentition and with no previous otorhinolaryngologic, orthodontic or speech therapy treatments were studied. Twenty nasal breathers were compared to 20 mouth breathing children diagnosed as OSAS patients. All children underwent otorhinolaryngologic evaluation and cephalometries; children with OSAS also underwent nocturnal polysomnography in a sleep laboratory. RESULTS: Children with OSAS presented increase in total and lower anterior heights of the face when compared to nasal breathers. In addition, children with OSAS presented a significantly more anterior and inferior position of the hyoid bone than nasal breathers. No significant differences in upper, anterior or posterior heights of the face were observed between groups. CONCLUSION: The results suggest that there are evident and early changes in facial growth and development among children with OSAS, characterized by increased total and inferior anterior heights of the face, as well as more anterior and inferior position of the hyoid bone.

Propofol-induced sleep: polysomnographic evaluation of patients with obstructive sleep apnea and controls.

OBJECTIVE: The localization of upper airway obstruction in patients with obstructive sleep apnea (OSA) may optimize treatment. Nasoendoscopy during propofol sedation allows such an evaluation, but the effect of this drug on respiratory patterns and muscle relaxation is unknown. The objective of the present study was to determine through polysomnography whether propofol would change sleep parameters. STUDY DESIGN: Prospective study of subjects submitted to polysomnography under sedation with propofol. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Fifteen non-obese subjects (4 controls/11 OSA patients) were submitted to two diurnal polysomnograms (90-120 minutes of sleep), with and without the use of propofol. The parameters presence of snoring, apnea-hypopnea index (AHI), oxygen desaturation, and sleep architecture were compared. RESULTS: The use of propofol did not induce snoring in the control subjects, whereas 100 percent of the OSA patients snored. AHI and mean oxygen saturation (SaO(2)) did not differ significantly between examinations with and without sedation. However, minimum SaO(2) differed significantly (P < 0.05) with sedation, being lower during propofol sedation. Propofol also significantly changed the sleep architecture, with a significant increase in N3 sleep (P < 0.005) and total abolishment of rapid eye movement sleep (P < 0.0005) during propofol sedation. CONCLUSIONS: These preliminary results allow us to infer that sedation with propofol changes sleep architecture but permits respiratory evaluation, because the main respiratory parameters evaluated in OSA are maintained. These preliminary results support the view that nasoendoscopy under propofol sedation is a promising examination for management of this disease.

Obstructive sleep apnea is frequent in patients with hypertensive intracerebral hemorrhage and is related to perihematoma edema.

BACKGROUND: Obstructive sleep apnea (OSA) is related to increased systemic inflammation and arterial hypertension. We hypothesize that OSA is frequent in patients with acute hypertensive intracerebral hemorrhage (ICH) and is related to the perihematoma edema. METHODS: Thirty-two non-comatose patients with a hypertensive ICH underwent polysomnography in the acute phase. Perihematoma edema volume was measured on CT scans at admission, after 24 h (early control) and after 4-5 days (late control). The Spearman coefficient (r(s)) was used for correlations. RESULTS: OSA occurred in 19 (59.4%) patients. The apnea-hypopnea index was correlated with relative edema at admission CT (r(s) = 0.40; p = 0.031), early CT (r(s) = 0.46; p = 0.011) and at late CT (r(s) = 0.59; p = 0.006). CONCLUSIONS: OSA is highly frequent during the acute phase of hypertensive ICH and is related to perihematoma edema.

Does sudden unexpected death in children with epilepsy occur more frequently in those with high seizure frequency? / Morte súbita em crianças com epilepsia ocorre naquelas que apresentam maior frequência de crises?

People with epilepsy are more likely to die prematurely and the most common epilepsy-related category of death is sudden unexpected death in epilepsy (SUDEP). Several studies have reported a moderate or high seizure frequency among SUDEP cases and SUDEP is considered rare in patients in remission. METHOD: We reviewed the occurrence of SUDEP in our epilepsy unit over an 8-year period to identify a potential association between seizure frequency and SUDEP occurrence in children with epilepsy. RESULTS: From 835 patients evaluated, 12 had suffered SUDEP and nearly all of the SUDEP cases in our children are related to chronic uncontrolled epilepsy (daily - 50.0 percent, two to four/week - 41.7 percent, monthly - 8.3 percent). CONCLUSION: SUDEP is not a rare event in children and increased mortality was recorded in those individuals who had not responded to pharmacologic treatment. Improved seizure control seems to be one of the most important measures to prevent SUDEP.

Pessoas com epilepsia têm maior chance de morrer prematuramente e a principal causa de morte relacionada à epilepsia é a morte súbita em epilepsia (SUDEP). Vários estudos têm relatado uma freqüência de crises moderada ou elevada em pacientes com SUDEP e SUDEP é considerada rara em pacientes em remissão. MÉTODO: Revisamos a ocorrência de SUDEP em nossa unidade de epilepsia por um período de oito anos com o objetivo de identificar uma possível associação entre freqüência de crises e a ocorrência de SUDEP em crianças com epilepsia. RESULTADOS: De 835 pacientes avaliados, 12 evoluíram com SUDEP e a maioria das crianças apresentava epilepsia crônica não controlada (50 por cento com crises diárias; 41,7 por cento com duas a quatro crises/semana; 8,3 por cento com crises mensais). CONCLUSÃO: SUDEP não é um evento raro em crianças e maior mortalidade foi observada em indivíduos que não responderam ao tratamento medicamentoso. Melhor controle de crises parece ser uma das principais medidas na prevenção de SUDEP.

Does sudden unexpected death in children with epilepsy occur more frequently in those with high seizure frequency?

UNLABELLED: People with epilepsy are more likely to die prematurely and the most common epilepsy-related category of death is sudden unexpected death in epilepsy (SUDEP). Several studies have reported a moderate or high seizure frequency among SUDEP cases and SUDEP is considered rare in patients in remission. METHOD: We reviewed the occurrence of SUDEP in our epilepsy unit over an 8-year period to identify a potential association between seizure frequency and SUDEP occurrence in children with epilepsy. RESULTS: From 835 patients evaluated, 12 had suffered SUDEP and nearly all of the SUDEP cases in our children are related to chronic uncontrolled epilepsy (daily--50.0%, two to four/week--41.7%, monthly--8.3%). CONCLUSION: SUDEP is not a rare event in children and increased mortality was recorded in those individuals who had not responded to pharmacologic treatment. Improved seizure control seems to be one of the most important measures to prevent SUDEP.

Pontine activation during focal status epilepticus secondary to hamartoma of the floor of the fourth ventricle.

Epileptic seizures associated with hamartoma of the floor of the fourth ventricle (HFFV) are generally resistant to antiepileptic medication, may evolve into status epilepticus, and can respond favorably to surgical therapy. HFFV are rare, and during the neonatal or infantile period may be associated with repetitive and stereotyped attacks of hemifacial spasm, eye blinking, facial movements, head deviation and dysautonomic manifestations. Similarly, to gelastic seizures provoked by hypothalamic hamartomas, it has been suggested that these spells arise from within the HFFV, thus constituting a type of non-cortical seizure. We report an infant female patient that developed continuous left hemifacial attacks since she was 2-month-old, and that underwent presurgical investigation when she was 18-month-old. MRI disclosed a left sided HFFV, Video-EEG showed non-localizing and non-lateralizing findings, and SPECT aligned with MRI showed marked hyperperfusion within the hamartoma, spreading to ipsilateral cerebellar parenchyma and brainstem nuclei. Patient underwent lesionectomy and became seizure-free. We found two evidences on literature supporting the hypothesis of non-cortical seizures related to HFFV. The first, intra-cerebellar recordings surrounding hamartoma showed electrical activity related to seizures. The second, subtracted SPECT co-registered MRI showed hyperemia within hamartoma. The present report provides the third additional evidence. We found the involvement not only of the hamartoma, and pars of cerebellar hemisphere, but also an intense hyperemia involving brainstem nuclei during seizures. We believe that all these findings suggest a short subcortical network responsible for generating seizures in HFFV patients.

Temporal lobe epilepsy surgery in children and adolescents: clinical characteristics and post-surgical outcome.

BACKGROUND AND PURPOSE: Temporal lobe epilepsy (TLE) encompasses 10-20% of the cases of intractable epilepsy in pediatric patients. Mesial temporal sclerosis (MTS) can still be encountered in adolescent patients, but is rare in children under 5 years of age. In this paper we report on the surgical outcome of a series of TLE patients ranging in age from 1 to 18 years at the time of operation. PATIENTS AND METHODS: Thirty-five patients (37 surgeries) with medically intractable TLE were operated upon between January 1996 and December 2002. The following variables were analyzed: age at surgery, age at epilepsy onset, history of an initial precipitating injury, etiology, seizure semiology, interictal and ictal EEG findings, surgical complications, and post-surgical seizure outcome. RESULTS: There were 68.6% females and 31.3% males, and complex partial seizures (CPS) occurred in 86.5%. The most common etiology was MTS (40%) followed by isolated cortical developmental abnormalities (22.9%). In the age group up to 5 years, cortical development abnormalities predominated, and 71% of these children had multifocal interictal EEG. Patients older than 10 years had more frequently MTS (78.6%) and focal temporal interictal EEG abnormalities. Post-surgical seizure outcome showed that 88.5% of patients were in Engel classes I and II. CONCLUSIONS: Adolescents with TLE had clinical features, electrographic findings, and seizure outcome similar to those observed in adult patients. However, younger children up to 5 years of age had distinct ictal semiology and different etiological, electrophysiological and outcome profiles, clearly suggesting that they behave as a special subgroup within the TLE.