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Journal: Seizure : the journal of the British Epilepsy Association

167

PURPOSE: Epilepsy is very common in the Kingdom of Saudi Arabia, with a prevalence of 6.54 per 1000. The present study was conducted to investigate the level of public awareness, and the attitudes and knowledge regarding epilepsy in the Saudi population in Riyadh - capital city of Saudi Arabia. METHODS: A survey consisting of 19 questions pertaining to epilepsy awareness was distributed to Saudi citizens living in Riyadh older than 15 years of age in malls, supermarkets, health clubs, mosques, universities and schools. RESULTS: Of the 7078 respondents who completed the questionnaire, 6756 (95.5%) had heard about epilepsy, 3024 (42.7%) had witnessed what they believed to be a seizure and 5164 (73%) would allow their children to interact with an individual who had epilepsy. However, 5382 (76%) respondents would not want their children to marry an individual with epilepsy, 1004 (14.2%) believed that epilepsy was infectious and 574 (8.1%) believed that epilepsy was a type of mental illness. A total of 1509 (21.3%) respondents were not aware of a single potential cause of epilepsy, 3493 (50.6%) would not seek medical advice if one of their relatives had epilepsy, 2221 (31.4%) did not know how to deal with an individual experiencing an epileptic episode and 6554 (92.6%) did not know that surgery was a treatment option for individuals with epilepsy in Saudi Arabia. Of the 7078 respondents, 3237 (45.7%) would not abide by a physician’s advice not to operate a motor vehicle because of their illness, of whom 1631 (50.4%) cited problems with the public transportation system as a reason for disregarding the doctor’s advice. The effect of age and level of education were statistically significant on most of the study variables. CONCLUSION: The level of epilepsy awareness in the Saudi population needs improvement.

Concepts: Saudi Arabia, Arabian Peninsula, Riyadh, Mecca, Jeddah, Ha'il, Dammam, Medina

28

Hypothalamic hamartoma (HH) is the main structural cause of central precocious puberty (CPP). HH is frequently associated with cognitive impairment and epileptic encephalopathies. Disease severity in case series from neurology services may be biased towards more neurologically impaired patients.

Concepts: Brain, Benign tumor, Cognition, Neurology, Epilepsy, Puberty, Hypothalamic hamartoma, Precocious puberty

27

PURPOSE: The aim of this study is to analyze the electroclinical features, treatment, and evolution of patients with Rasmussen syndrome (RS). MATERIALS AND METHODS: We conducted a retrospective, descriptive study in 32 consecutive patients with RS followed between 1990 and 2012. RESULTS: Twenty boys and 12 girls were included in the study. The mean and median ages at onset of the seizures were 6.5 and 7 years, respectively. Twenty-eight cases had epilepsia partialis continua that had started at a mean age of 9.5 years. Fixed hemiparesis occurred within the first two years after seizure onset in 26 patients. The ictal EEG showed a multifocal origin, but confined to the affected hemisphere in all patients. Mild focal atrophy involved the temporo-insular region associated with enlargement of the ipsilateral horn and Sylvian fissure. An abnormal cortical and/or subcortical hyperintense signal was observed in T2 and Flair images in 25 and 17 patients, respectively. T2 hyperintensity and atrophy in the basal ganglia was documented in five patients. Corticosteroids associated with immunoglobulins were used in 25 patients. Surgical treatment was performed in 25 patients. After a mean follow-up of 13 years (range, 2-20) good surgical outcome - Engel class I - was observed in 23 of 25 patients operated. CONCLUSION: Corticosteroid and intravenous immunoglobulin treatment should be considered in the early stages of the disease. Patients with RS had a good response to surgical excision of the affected hemisphere.

Concepts: Antibody, Surgery, Cerebral cortex, Basal ganglia, Epilepsy, Seizure, Rasmussen's encephalitis, Epilepsia partialis continua

27

PURPOSE: The efficacy and safety of the anti-convulsive drug levetiracetam (LEV) has been well documented but few clinical studies have investigated tolerance to LEV. The aim of this study was to evaluate the loss of the initial efficacy of LEV in adult patients with refractory partial-onset seizures. METHODS: We enrolled patients with refractory partial epilepsy who were started on add-on LEV treatment. The efficacy of LEV was evaluated every three months and the seizure frequency was decided by the average number of monthly seizures. A responder was defined as a patient with a ≥50% reduction in seizure frequency from the baseline. Seizure freedom was defined as a seizure-free status from the beginning of LEV treatment to the evaluation period. Loss of the initial efficacy was defined as a shift from responder status during the first three months of LEV treatment to non-responder status during the follow-up period. RESULTS: A total of 95 epilepsy patients were analyzed. During the first three months of LEV treatment, 50 (52.6%) of the 95 patients were responders with a ≥50% seizure reduction. Nine patients (18.0%) showed a loss of initial efficacy during the second three-month period. In contrast, only two (4.0%) of the non-responders during the first three months became responders during the next three months. However, this difference did not reach statistical significance (P=0.054). Based on Kaplan-Meier survival estimates, 49.2% of the patients who initially responded to LEV treatment during the first three months were predicted to lose this response at 42 months. Loss of the initial efficacy of LEV treatment occurred mostly within 18 months. CONCLUSION: This study suggests that the occurrence of tolerance is more common than late gain of efficacy of treatment although larger prospective studies would have to be carried out to prove this observation.

Concepts: Epilepsy, Anticonvulsant, Seizure, Status epilepticus, Diazepam, Postictal state, Myoclonus, Photosensitive epilepsy

12

OBJECTIVE: To evaluate the efficacy and safety of the ketogenic diet (KD) treatment of refractory childhood epilepsy in China and determine which children are more likely to respond. METHODS: Between 2004 and 2011, we prospectively enrolled 317 children with refractory epilepsy for the KD treatment in Shenzhen Children’s Hospital and followed up for at least a year. Outcome was measured by seizure frequencies before and after the diet, change in anticonvulsant use and adverse effects. We also evaluated influences of different variables (starting age, duration of epilepsy and underlying conditions) on the outcome. RESULTS: Intent-to-treat analysis showed that after 3, 6 and 12 months, 62.8%, 42.0% and 24.3% remained on the diet, 35.0%, 26.2% and 18.6% showed >50% seizure reduction, including 20.8%, 13.6% and 10.7% seizure free, respectively. Starting age may influence efficacy. The ≥10 age group showed worse response than the <10 age group, though the difference was statistically significant (p=0.039) at 3 month only. Other variables such as duration of epilepsy at the start of the diet, seizure types and aetiologies showed no significant influence on efficacy. Frequently reported complications included GI disturbance, food refusal and hypoproteinaemia. CONCLUSIONS: The KD is a safe and efficacious therapy for intractable childhood epilepsy in Chinese children. The influence of age on efficacy is worth further investigation.

Concepts: Randomized controlled trial, Neurology, Epilepsy, Anticonvulsant, Seizure, Status epilepticus, Absence seizure, Ketogenic diet

11

PURPOSE: Although most neuroscientists and physicians would argue against Cartesian dualism, Descartes’s version of the psyche/soma divide, which has been controversial since he proposed it in the seventeenth century, continues to haunt contemporary neurological diagnoses through terms such as functional, organic, and psychogenic. Drawing on my own experiences as a person with medically unexplained seizures, I ask what this language actually means if all human experience has an organic basis. METHODS: Close reading of a textbook chapter on psychogenic seizures. RESULTS: I expose the author’s unreflective embrace of psyche and soma as distinct entities, his inherent bias against illnesses labeled psychogenic, and the implicit sexism of his position. I further argue that even when a patient’s symptoms are not alleviated, heightened self-consciousness and narrative framing can strengthen his or her sense of agency and have therapeutic benefits. CONCLUSION: The ethical treatment of patients requires a respect for their stories.

Concepts: Medicine, Brain, Philosophy of mind, Mind, Philosophy of science, Philosophical terminology, Soul, René Descartes

2

Urinary incontinence may occur both in epileptic seizures (ES) and in non-epileptic events (NEE) such as psychogenic nonepileptic events (PNEEs) and syncope. A comprehensive search of the literature to determine the accuracy of this physical finding and its prevalence in epileptic seizures and syncope is still lacking. To undertake a systematic review to determine sensitivity, specificity and likelihood ratios (LR) of urinary incontinence in the differential diagnosis between ES and NEEs (including syncope and PNEEs).

Concepts: Medical terms, Urinary incontinence, Medical diagnosis, Epilepsy, Seizure, Differential diagnosis

1

Epileptic seizures can lead to changes in autonomic function affecting the sympathetic, parasympathetic, and enteric nervous systems. Changes in cardiac signals are potential biomarkers that may provide an extra-cerebral indicator of ictal onset in some patients. Heart rate can be measured easily when compared to other biomarkers that are commonly associated with seizures (e.g., long-term EEG), and therefore it has become an interesting parameter to explore for detecting seizures. Understanding the prevalence and magnitude of heart rate changes associated with seizures, as well as the timing of such changes relative to seizure onset, is fundamental to the development and use of cardiac based algorithms for seizure detection. We reviewed 34 articles that reported the prevalence of ictal tachycardia in patients with epilepsy. Scientific literature supports the occurrence of significant increases in heart rate associated with ictal events in a large proportion of patients with epilepsy (82%) using concurrent electroencephalogram (EEG) and electrocardiogram (ECG). The average percentage of seizures associated with significant heart rate changes was similar for generalized (64%) and partial onset seizures (71%). Intra-individual variability was noted in several articles, with the majority of studies reporting significant increase in heart rate during seizures originating from the temporal lobe. Accurate detection of seizures is likely to require an adjustable threshold given the variability in the magnitude of heart rate changes associated with seizures within and across patients.

Concepts: Neurology, Electroencephalography, Epilepsy, Autonomic nervous system, Seizure, Sympathetic nervous system, Myoclonus, Parasympathetic nervous system

1

The present study aimed to assess the impact of the ketogenic diet on arterial morphology and endothelial function of the big vessels of the neck and on cardiac diastolic function, in a cohort of epileptic children and young adults treated with the ketogenic diet.

Concepts: Heart, Blood vessel, Endothelium, Epilepsy, Anticonvulsant, Study design, Ketogenic diet

0

To identify risk factors (RF) of pediatric convulsive status epilepticus (SE) and to determine whether defining SE as seizures ≥5min (SE5) or seizures ≥30min (SE30) would modify the risk factors identified.

Concepts: Definition, Neurology, Seizure, Status epilepticus, Complex partial status epilepticus