The comorbidity of headaches in pediatric epilepsy patients: How common and what types?

Objectives:To estimate the prevalence and characteristics of headache in pediatric epileptic patients.Methods:This cross-sectional study was performed over 6 months period from January 2018 to June 2018 at King Abdullah Specialist Children Hospital, King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia using a structured questionnaire in pediatric patients with epilepsy.Results:There were 142 patients enrolled (males, 57.7%; average age, 10.7±3.1 years) with idiopathic epilepsy (n=115, 81%) or symptomatic epilepsy (n=27, 19%). Additionally, patients had focal epilepsy (n=102, 72%) or generalized epilepsy (n=40, 28%), and among them, 11 had absence epilepsy. Overall, 65 (45.7%) patients had headaches compared with 3/153 (2%) in the control group (p < 0.0001). Among the 65 patients with headaches, 29 (44.6%) had migraine-type, 12 (18.4%) had tension-type, and 24 (36.9%) had unclassified headache. There was no significant difference in age, gender, type of epilepsy syndrome, and antiepileptic used except in patients with or without headache. For migraine patients, there was a lower headache prevalence in the subgroup treated with valproic acid compared with other treatments.Conclusion:Headache, predominantly migraine, is a common problem in pediatric epileptic patients and choosing valproic acid when possible can be important in preventing migraine in these patients.

Epilepsy and headache are chronic paroxysmal disorders that affect adult and pediatric patients1 with episodic manifestations. Headache or (cephalalgia) is defined as a feeling of pain in the region of the head or neck. Primary headaches include migraines, tension-type headache, and cluster headache. Epileptic seizure is a brief episode of signs or symptoms caused by abnormal excessive synchronized neuronal activity.2 Epilepsy is defined as a condition where the patient has an enduring tendency to have recurrent unprovoked seizures.2 These two disorders coexist in some patients.3 There are few studies on the comorbidity of headaches in children with epilepsy.4-6 Other studies reported a significant association between migraine and epilepsy.1,7-9 Additionally, the genetic predisposition for both entities was reported in some forms of channelopathy,10 and others found more prevalence of migraine headache in specific diseases in pediatric like benign epilepsy with centrotemporal spikes and juvenile myoclonic epilepsy.11 Seizure-associated headache is common, with an incidence of 42–51% in adult epileptic patients.12 However, for pediatric patients, it is often neglected by parents and physicians because of other neurological manifestations of the seizure such as loss of consciousness and motor components,13 and approximately 36% of the parents were reported to be unaware that their children experienced headache.14 It is our experience that headache is a common problem in up to 50% of epilepsy patients but we do not know exactly the prevalence, in addition to what type of headache is most commonly found in epileptic pediatric patients. Because of few reports on this topic have conflicting results, the objective of this study was to evaluate the prevalence and characteristics of headache in children with epilepsy who were seen at one center in Saudi Arabia.     

The role of ketogenic diet in controlling epileptic seizures

Objectives:To study the role of the ketogenic diet (KD) in controlling seizures in children with medically resistant epilepsy in Saudi Arabia.Methods:This retrospective study was conducted in the Pediatric Neurology Clinic at a tertiary care epilepsy center. Thirty-one patients with medically resistant epilepsy were enrolled from 2013 to 2018. The seizure reduction variables were evaluated at 6, 12, 18 and 24 months after enrollment.Results:Of the 31 patients, 14 (45.2%) were males and 17 (54.8%) were females. The most common types of seizures were myoclonic seizures and mixed seizures, both of which occurred in 9 (29%) of the participants. Of the participants, 15 (48.4%) had seizures one to 5 times per day. Six months after starting a KD, 2 (6.45%) of participants were seizure-free; 6 (19.35%) were seizure-free after 12 months of treatment.Conclusion:The present study highlighted the effectiveness of KD in medically resistant epilepsy children to local population. A larger cohort is warrant to confirm these findings.

Epilepsy is a common neurological disorder. It is characterized by seizures and affects approximately 65–70 million people worldwide.1 Children and the elderly are most commonly affected by seizures; the condition is rarer in adults.2 Epilepsy remains a challenging neurological disorder despite effective pharmacological therapies.3 More than 30% of epileptic patients do not achieve complete control of seizures with available anti-epileptic drugs (AEDs).1,4 Around 20-40% of patients with epilepsy have refractory epilepsy “failure of, adequate trials of 2 tolerated, appropriately chosen and used AED schedules (whether as monotherapies or in combination) to achieve the sustained seizure freedom” and who are not candidates for surgery, non-pharmacological interventions should be considered for ketogenic diet (KD) treatment.5,6The KD has been proposed for first time in 1920s as a non-pharmacologic treatment to control refractory childhood epilepsy.1 The KD is a high-fat, low-protein, low-carbohydrate diet, with ketogenic ratio of 4:1 or 3:1 in grams which is the most commonly administered ratio.7,8 The KD increases the production of ketone bodies, which brain uses these ketone bodies as an energy source instead of glucose.2 Some hypotheses have been proposed regarding the KD’s anti-seizure effects, suggesting that changes in the nature and degree of energy metabolism in the brain, changes in neurotransmitter function, changes in synaptic transmission, and changes in neuronal cellular properties may explain the diet’s effectiveness.9 Higher ketone levels correlate with better seizure control.9 Ten to 15% of children with epilepsy become completely seizure free on a KD.3 Furthermore, the KD has a prolonged beneficial effect even after it is discontinued.9The International Ketogenic Diet Study Group strongly recommends that the KD be considered as a treatment for children with epilepsy who fail to respond to two or three anticonvulsant medications, regardless of age or gender.9 Our objective was to study the efficacy of the KD in children with medically resistant epilepsy in Saudi Arabia.     

Prevalence of epilepsy in Saudi pregnant women and possible effects of anti-epileptic drugs on pregnancy outcomes

Objectives:To determine the prevalence of epilepsy in Saudi pregnant women and estimate the frequency of seizure types in suffering individuals using different anti-epileptic drug modalities. It also aimed to report the teratogenic effects of anti-epileptic drugs as observed in neonates.Methods:This prospective study was conducted at King Fahd University Hospital from June 2018 to July 2019. Sixty-eight pregnant women diagnosed with epilepsy were included in this study. Seizure types and their frequencies were recorded along with anti-epileptic drug therapies and their association with fetal/neonatal malformations.Results:Out of 68 epileptic pregnant females, 30 (44.1%) experienced focal seizures and 38 (55.9%) experienced generalized seizures. Thirty-nine (57.3%) received monotherapy, 21 (30.9%) received polytherapy and 8 (11.8%) did not take antiepileptic drugs during pregnancy. Thirty-six (52.9%) patients experienced no change in seizure frequency during pregnancy, 19 (27.9%) experienced increase in seizure frequency and 13 (19.1%) showed decreased seizure frequency. The pregnancy outcomes analysis showed 2 (2.9%) intrauterine fetal deaths, whereas 4 (4.9%) neonates showed facial and/or organ malformations.Conclusion:The frequency of seizures was found to increase in only 27.9% of the pregnant women in the sample. Malformation and mortality rates were higher in fetuses/neonates of patients with generalized seizures. It was observed that for the patient group using monotherapy, the rate of healthy babies was higher than that of the group using polytherapy.

Epilepsy is defined as a brain disorder characterized by an enduring predisposition to generate epileptic seizures, with cognitive, neurobiological, psychological, and social consequences.1 An epileptic seizure is “a transient occurrence of signs and symptoms due to abnormal excessive or synchronous neuronal activity in the brain”.1 According to the World Health Organization (WHO), it is the second most burdensome neurological disorder that can cause cognitive, neurobiological and psychosocial abnormalities in the suffering individuals.2,3 In women with epilepsy (WWE), disruption of menstruation cycle, fertility, pregnancy, fetus development, menopause and sexual dysfunction has been reported.4 The condition becomes more critical when WWE become pregnant. It has been estimated that the maternal death rate for this group is 10 times higher as compared to normal pregnancy cases.5 Moreover, the frequent seizures during pregnancy have been known to jeopardize the cognitive and physical development of the fetus.6 Thus, epilepsy is not only harmful for the mother but may also pose potentially worse consequences for the fetus.The management of seizures through anti-epileptic drugs (AEDs) in pregnant WWE is a challenging task because during the risk-benefit assessment, the needs of the suffering WWE against the potential adverse effects of AEDs on the fetus need to be taken into account as well.7 Several studies suggest the association of AEDs with congenital malformations which put physicians in a difficult spot. In this challenging situation, the attending physician is expected to choose between the available treatment options (mono- or polytherapy) during pregnancy. Therefore, the most critical issue is the regulation of the minimum effective dose of AEDs during pregnancy to prevent any damage to the fetus from seizures as well as AEDs.8A plethora of evidence has reported the prevalence of epilepsy and its possible outcomes in the general population.2,9,10 However, less is known about the prevalence of epilepsy in Saudi pregnant women, the association of AEDs with the frequency of seizures and their impact on fetal development. The quantification of epileptic pregnant patients and their pregnancy outcomes is important in order to provide optimal counseling and patient care. Therefore, the aims of this study were multifold: (1) to determine the prevalence of epilepsy in pregnant females, (2) to estimate the frequency of seizure types in suffering individuals using different anti-epileptic drug modalities, (3) to assess pregnancy outcomes and possible birth defects in WWE taking monotherapy, polytherapy or no therapy during pregnancy.     

Consanguinity in patients with mesial temporal lobe epilepsy due to hippocampal sclerosis in a Saudi population

Objectives:To investigate if there is an association between consanguinity and hippocampal sclerosis (HS) in the Saudi population.Methods:A retrospective case-control study was conducted by assessing the prevalence of consanguinity in patients with pathologically proven HS, who underwent epilepsy surgery at King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia, between January 2004 and December 2015. We reviewed the medical records to extract data, which included; age, gender, duration of epilepsy, history of febrile seizure, family history of epilepsy in a first or second-degree relative, and pathology reports.Results:A total of 120 patients, out of which 40 patients (65% male) having mesial temporal lobe epilepsy due to HS, and 80 controls (56% male) with cryptogenic epilepsy, were identified. Twenty-two patients (53.5%) in the HS group had a history of consanguinity. In the control group, 30 patients (37.5%) had a history of consanguinity. The odds ratio was 2.04 (95% confidence interval = 0.94 - 4.4, p = 0.052). A family history of epilepsy was found in 28% of the patients with HS and 32.5% cryptogenic epilepsy. Only 8 patients (19.5%) with HS reported a history of febrile seizure.Conclusion:Our retrospective case-control study suggests that consanguinity might increase the likelihood of developing HS.

Consanguineous marriage, in clinical genetics, is defined as a union between couples related as second cousins or closer.1 Saudi Arabian culture has a higher consanguinity rate than other Arab, Asian, and Western communities. Consanguinity is not known to increase the risk of idiopathic or cryptogenic epilepsy, based on recent studies from Saudi Arabia and UAE.2,3 However, there is a strong evidence that the marriage of first cousins is one of the main reasons for the increased prevalence of autosomal recessive diseases.4The association between consanguinity and epilepsy due to hippocampal sclerosis (HS), the most common pathology found in patients with intractable focal epilepsy, has not been adequately studied.5 A familial type of mesial temporal lobe epilepsy (FMTLE) has been described with homogeneous and heterogeneous clinical manifestations.6-8 Moreover, magnetic resonance imaging (MRI) of the brain of asymptomatic first-degree relatives of patients with confirmed FMTLE showed evidence of HS, which supports the genetic predisposition to FMTLE.9 Although, FMTLE is widely recognized as an autosomal dominant disease with incomplete penetrance, autosomal recessive or X-linked forms have also been reported. Still, they have not been confirmed by extensive studies.10In this study, we aimed to investigate the association between consanguinity and HS in a Saudi population. This study may help expand our knowledge of the underlying mechanisms of HS and may shed some light on a possible genetic substrate that contributes to the development of HS in this population.     

Knowledge of and attitudes toward epilepsy surgery among neurologists in Saudi Arabia

Objectives:To investigate the levels of knowledge and attitudes toward epilepsy surgery among neurologists in Saudi Arabia and evaluate the factors that affect the physicians’ knowledge and attitudes.Methods:A quantitative observational cross-sectional study conducted at King Saud University Medical City, Riyadh. The data were collected using a newly developed, self-administered online questionnaire. The questionnaire contained 3 sections: demographic information, knowledge, and attitudes which then sent to neurologist in Saudi Arabia from December 2016 to March 2017.Results:A total of 106 neurologists met our inclusion criteria. Eighty percent of the participants had at least one epilepsy center in their city, and 78% indicated that they had access to adequate expertise and resources to enable the appropriate selection of epilepsy surgical candidates. Only 57.5% of the neurologists had a sufficient level of knowledge regarding epilepsy surgery. Neurologists with higher level of knowledge referred more patients to EMU and discussed epilepsy surgery more often with their patients. Overall, more than half of the neurologists (52.8%) had a positive attitude toward epilepsy surgery. There was a significantly positive correlation between the scores of knowledge and attitude (p<0.001).Conclusion:Neurologists in Saudi Arabia appear to have moderate knowledge of and positive attitudes toward epilepsy surgery. The place of the last neurology certificate, type of practicing hospital, and access to expertise and resources, affected their knowledge. Adequate knowledge was positively correlated with attitude.

Epilepsy is one of the most common neurological disorders in Saudi Arabia, with an estimated prevalence of 6.54 per 1000 individuals.1 It is estimated that 20-40% of newly diagnosed patients are expected to fail seizure control with medical treatment.2 Refractory epilepsy is commonly defined as the failure to achieve freedom from seizures despite two or more antiepileptic drugs (AEDs).3 Patients with drug-resistant epilepsy (DRE) have a lower quality of life and face more social difficulties than patients with controlled epilepsy.4-6 More than half of the economic burden of epilepsy is accounted for by patients with refractory epilepsy.7 Epilepsy surgery is the best evidence-based treatment option for these patients with drug resistant focal epilepsy.8,9 The current guidelines for patient referral to epilepsy surgery were published in 2003 by the American Academy of Neurology.9 The benefits of epilepsy surgery may not only reduce the frequency of seizures but might also lower mortality and improve the quality of life.10 Despite its proven efficacy and favorable outcome, the average referral delay of an epilepsy surgery candidate is more than 20 years.11 This delay is predominately attributed to non-adherence to referral guidelines, which results from a lack of awareness and familiarity with them.12 Another factor that contributes to the delay is patients’ misbeliefs regarding the surgery’s risks and benefits, which are highly influenced by physicians’ lack of knowledge.13 Epilepsy surgery was introduced to Saudi Arabia in 1998.14 Several epilepsy surgery centers have subsequently been established across the country. A survey in 2013 showed that 56% of health-care professionals in Saudi Arabia were not aware that surgery was a treatment option for patients with epilepsy.15 Our aims in this study were to investigate the levels of knowledge and attitudes toward epilepsy surgery among neurologists in Saudi Arabia and evaluate the factors that affect the physicians’ knowledge and attitudes.     

The etiology and risk factors of convulsive status epilepticus in pediatric patients of tertiary center in Saudi Arabia

Objectives:To assess the etiology of status epilepticus (SE) among the pediatric patients of a tertiary center in Jeddah, Kingdom of Saudi Arabia (KSA).Methods:Data from 88 cases was obtained retrospectively from 2006 to 2017 from King Abdulaziz University Hospital (KAUH). Patients aged between 28 days and 14 years with the symptoms of SE were selected for this study.Results:The data show that the proportions of SE etiologies were 30.5% for febrile seizure, 11.9% for electrolytes imbalance, 8.5% for hydrocephalus, 6.8% for CNS infections, and 6.8% for neoplasm. The other etiologies of SE were trauma, fever, intractable epilepsy, cerebrovascular accident, hemorrhagic stroke, etc. There were no clear etiologies in 8 cases. Most of the patients were not of Saudi origin (64.3%), boys (67.8%), and had generalized epilepsy types (91.8%), and 58.6% of the patients complied with epilepsy medications.Conclusion:The most prevalent etiology of convulsive status epilepticus was a febrile seizure, followed by electrolyte imbalance and hydrocephalus. However, a nationwide study in KSA must be conducted to determine the major etiologies of SE for its effective management and prevention. Educating families and patients regarding antiepileptic drugs is necessary.

Status epilepticus (SE) is a life-threatening seizure and common pediatric crisis. It is the most critical case of seizure. For every 100,000 children, 73 had SE. The younger the child, the higher the incidence of SE will be. The incidence of SE in children aged less than 2 is high at 156 per 100,000 and is at the highest for infants.1-3 The SE is the commonest pediatric neurological emergency and a major cause of admission to the pediatric intensive care unit (PICU). Historically, many attempts have been made to define and explain seizure and SE. The International League Against Epilepsy (ILAE) recently defined SE as generalized convulsive SE for any tonic-clonic seizures that continue more than 5 minutes, as focal SE for any focal seizure that continues more than 10 minutes.4 Clinicians describe it in a very operational and timed manner, stating that early action can prevent neuronal injuries from happening. They define SE as a continuous attack that exceeds 5 minutes or 2 or more separate attacks with incomplete recovery of consciousness between attacks.5 The SE is of 2 types: the convulsive type (CSE), which is the commonest, and the non-convulsive type (NCSE), which accounts for only 6% of the cases.6The main risk factors for SE are listed in many studies, with acute febrile illness being listed as a major cause. One study conducted in Japan found that prolonged febrile seizure is the cause of SE for 81.6% of the cases, while the causes for 18.4% of the cases were epilepsy, encephalopathy/encephalitis, and meningitis.7 Another study was carried out at Ayub Medical College with 125 patients, where most of the cases were caused due to acute febrile illnesses.8 In a cohort study conducted by the Specialized Children Hospital’s Pediatric Emergency Department at Cairo University, the acute symptomatic (AS) causes were the main etiologies. In that study, most AS cases (61%) were caused due to central nervous system (CNS) infection; the other causes were metabolic, hemorrhagic stroke, and neoplasim; most of them were infants aged less than one year at the SE onset (67%) and suffered from refractory seizure (86%) in which the seizure lasts for more than 60 minutes.9 Another main cause of SE was epilepsy; as mentioned in previous study in 1994, most SE patients have a history of epilepsy. Febrile seizures, meningitis, encephalitis, and metabolic disease were the other etiologies of SE in the study.10 However, only few studies include pediatric cases from Riyadh city of Saudi Arabia11 and a guideline management,12 and only a few and old (2002 and less) studies on SE pediatric patients in Jeddah,13,14 and thus making our study a significant contribution in this field. The study objectives are to identify the most common SE risk factors to prevent them in the future.     

Neurodevelopmental and epilepsy outcomes of patients with infantile spasms treated in a tertiary care center

Objectives:To assess the neurodevelopmental and epilepsy outcomes in children with infantile spasms (IS).Methods:A retrospective chart review of all patients with infantile spasms admitted to King Khalid University Hospital (KKUH), Riyadh, Saudi Arabia between January 2000 and December 2017. Infants who were diagnosed to have IS as per the International League Against Epilepsy (ILAE) definition were included in this study. Patients who lost follow-up and those who did not receive treatment at KKUH were excluded.Results:Total of 53 patients were included and categorized into unknown, cryptogenic and symptomatic type of IS. The majority had symptomatic etiology (71.7%). Type of etiology and delay in initiation of treatment were significant predictors of motor and cognitive outcomes, but not seizure control. Patients with unknown IS, who were diagnosed earlier (0.72-month), had better neurodevelopmental outcomes. Vigabatrin in combination with either Adrenocorticotropic hormone (ACTH) or Prednisolone showed better seizure control in comparison to monotherapy and other combination modalities.Conclusion:Neurodevelopmental outcomes of IS are strongly associated with the underlying etiology. Early initiation of treatments had a favorable cognitive and motor outcome. Early response to combination therapy with resolution of spasms and hypsarrhythmia had better seizure outcomes. However, motor and cognitive outcomes were not affected by the response to the combination therapy.

Infantile spasms (IS) is a rare form of epileptic encephalopathy characterized by spasms and hypsarrhythmia on the electroencephalogram (EEG).1 IS is diagnosed at 6 months of age on average.2 There are no reports on the incidence of IS in Saudi Arabia. International estimates put the figure at 1:6,000 – 1:4,000 live births. Studies suggest that IS constitutes 2% of childhood epilepsies, and a quarter of epilepsy cases are seen during the first year of life.3 The etiology of IS is divided into prenatal, perinatal, or postnatal disorders.3 Genetic defects associated with IS are increasingly being understood. Tuberous sclerosis is the most common disorder associated with IS and constitutes 10-30% of symptomatic IS. It is an autosomal dominant disorder caused by mutations in TSC1 and TSC2 genes, with variable manifestations, including cardiac and renal tumors, cutaneous malformations, and seizures.4 Mutations in the ARX gene and cyclin-dependent kinase-like protein 5 (CDKL5), 14q12 duplications harboring FOXG1, STXBP1, and PLCB1 have been also implicated in IS.5-8 Other prenatal causes include hydrocephalus, hydranencephaly, Trisomy 21, Hypoxic-ischemic encephalopathy, congenital infections, and trauma. IS can also occur secondary to a perinatal or postnatal disorders, including meningitis, trauma, encephalitis, intracranial hemorrhage, and inborn errors of metabolism.9When there is no clear cause of IS identified, IS can be labeled as unknown or cryptogenic. Uunknown IS is characterized by normal development prior to the onset of IS. Cryptogenic IS, on the other hand, is diagnosed when no known etiology is determined, but prior developmental delay has occurred.10Neurodevelopmental regression is characteristic of IS, and children with history of IS often have poor neurodevelopmental outcomes.11 In a cohort of 180 patient, 53% of infants with symptomatic IS have normal seizure status and cognitive development compared to 21.9% of infants with unknown IS.12The long-term survival of patients with IS thought to be poor and was related to the underlying condition, where unknown IS generally has a better prognosis than symptomatic IS.11 The aim of this study was to identify those patients and treatment factors that correlate with favorable neurodevelopmental and epilepsy outcomes in a tertiary hospital in Saudi Arabia.     

Frequency of antiepileptic drugs and response change in pediatric patients receiving 2 or more antiepileptic drugs

Objectives:To investigate the frequency of changes in antiepileptic drugs (AEDs) use, as well as concomitant changes in the degree of seizure control in pediatric patients, who are receiving 2 or more AEDs.Methods:A prospective follow-up study at Jordan University Hospital’s pediatric neurology clinics was conducted on epileptic pediatric patients receiving at least 2 AEDs between December 2013 and April 2014. Patients were followed for 12 months.Results:A total of 82 patients were included, with a mean age of 7.2±4.7 years. The mean number of AEDs received by patients at enrollment was 2.4±0.6, and 2.5±0.7 after follow-up. Most patients (63.4%) experienced no change in seizure control, and the majority reported at least one adverse drug reaction. Most patients received lower doses than recommended, both at the beginning and end of the study. During the year, only 3 patients (4%) were eligible for dose tapering, which would then be converted to monotherapy. Follow-up appointments average was 4.2±2.9 visits/patients in one year. The frequency of medication changes and dose adjustment was very low, about one-third (29.3%) of patients requiring no change in AEDs during any follow-up visits.Conclusion:During the one year follow-up study, most patients on polytherapy maintained their level of response to the AEDs, with minimal changes in their regimen despite frequent follow-up visits. Only a small percent could be converted to AEDs monotherapy.

Epilepsy is the most common neurological disorder in children.1 Despite the increase in the number of antiepileptic drugs (AEDs), about one third of patients with childhood epilepsy continue to have seizures.2 Successful management of epilepsy depends on many factors, including the cause of epilepsy, type of epilepsy, and AED-related issues, such as: dosing, compliance, side effects, monitoring, and pharmacokinetics.3,4 Most AEDs have a suboptimal patient tolerability profile (with a wide range of side effects); the second-generation AEDs may be better tolerated than the traditional AEDs, but are not actually more effective.5Due to the complexity of classifying seizures, as well as the difficulty in choosing the most appropriate AED, many patients must deal with drug therapy changes over the course of their treatment/management.5-7 It has been estimated that approximately 50% of patients who are newly diagnosed with epilepsy become seizure-free for at least 12 months on their first AED.7 However, about one-third receive 2 or more AEDs, while continuing to experience seizures.5 In general, patients requiring multiple AEDs to manage their epilepsy represent the more resistant group of patients. This group must be closely followed to optimize their AED regimen;8 as the change in drug therapy might be more likely, but improvement is expected to be limited. Cited reasons for lack of improvement include incorrect diagnosis, inappropriate choice of AED and/or dose, in addition to lack of adherence. Data regarding follow-up studies of pediatric patients on AED polytherapy is scarce.9,10 The aim of the current study is to conduct a short-term (12 months) follow-up study of the change in AEDs, as well as the response in a subgroup of pediatric epileptic patients receiving AED polytherapy.     

Epilepsy in patients with insulin-dependent diabetes and relation to glutamic acid decarboxylase 65

Objectives:To report if the association of epilepsy in pediatric patients (below the age of 15 years) with Insulin-dependent Diabetes (IDDM) at King Fahad Medical City (KFMC) is higher than the prevalence of epilepsy in the same age group (who have no IDDM) in our community. Consequently, we would determine if there is a relationship between the presence of epilepsy in diabetic children and the presence of positive antiGAD65 antibodies.Methods:This cohort study included 305 pediatric patients below the age of 15 years with Insulin-dependent Diabetes Mellitus (IDDM). They were randomly recruited at the Pediatric Endocrinology Clinic in KFMC. The patients’ caregivers were given a questionnaire between December 2015 till March 2019 to determine the seizure disorder history. There was also a retrospective review of 214 patients’ files for anti-GAD 65 positivity.Results:Our study found a significant relation between the presence of epilepsy in children with IDDM. Therefore, we could confirm the relationship between the existence of epilepsy in children with IDDM and having positive GAD65 antibodies.Conclusion:Our study supports the presence of consistent relation between having IDDM and having epilepsy in children and between the latter and the presence of positive GAD65 antibodies.

Insulin dependent diabetes Mellitus (IDDM) is a common condition in children and adolescents worldwide and so is epilepsy.1,2 Recently, there were increasing reports suggesting a potential association between having IDDM and the occurrence of epilepsy.3 Their association might represent simply a chance to relate their underlying mechanisms. However, the cause-effect relationship is not fully well defined. Literature from other countries have shown the increased prevalence of seizure disorders in this group of patients.4,5 There are scarce studies in the literature investigating IDDM characteristics contributing to having epilepsy, including positive GAD 65 antibodies. In this study that ran in King Fahad Medical City, Riyadh, Kingdom of Saudi Arabia (KSA) we are aiming to determine if the prevalence of epilepsy among 1DDM children under the age of 15 years (in our center) is higher than controls (same age without IDDM), and to check the positivity of anti-GAD 65 amongst those patients in order to find if there is a relationship between epilepsy in children with diabetes and the presence of positive GAD65 Antibodies.     

Awareness of Saudi community toward multiple sclerosis in Qassim Region,Saudi Arabia

Objectives:To evaluate the awareness of Saudis in Qassim region, Saudi Arabia on multiple sclerosis (MS).Methods:A cross-sectional study was conducted on 350 Saudis in Qassim, Saudi Arabia between January 2019 and June 2019 using a pre-designed questionnaire including socio-demographic data and questions evaluating knowledge about MS.Results:The majority of studied participants were adult females (74%), between 20-30 year-old (45.1%) and with high education level or above (80.6%). Nearly one third of the studied group had good knowledge regarding MS (31.7%). Half of the studied group knew that central nervous system (CNS) is the system affected by MS, 52% reported that vitamin D deficiency, family history of MS, personal history of autoimmune disease, viruses, and obesity are the factors which increase the risk of developing MS. Approximately, 62.9% knew that blurred and double vision, numbness, paralysis or weakness and difficulty in concentration and memorizing are symptoms of MS. Female participants and those knowing someone having MS had a significant good knowledge level regarding MS than others. Getting knowledge from combined sources from internet or social media; family, friends or neighbors and health workers was significantly more prevalent among those with good knowledge.Conclusion:Most of the study participants had limited knowledge regarding MS. Planning health education programs for the public is essential.

Multiple sclerosis (MS) is an inflammatory neurodegenerative disease affecting the central nervous system (CNS).1,2 Among middle-aged adults (between 20 and 40 years), MS is considered as one of the main life-long disability chronic neurological causes which causes expressive health care and economic loads. Women are at 3 to 4 folds increased risk to be affected by MS than men.3,4 Neurologically, the common manifestations are loss of coordination and balance, visual impairment, weakness, fatigue, numbness, pain, bladder dysfunction, mood changes, and cognitive dysfunction.5,6 Multiple sclerosis has a complex pathophysiology. It is widely recommended as an autoimmune disease, and is mediated by autoreactive lymphocytes that cross the blood-brain barrier (BBB) and enter the CNS, causing local inflammation; producing demyelination, gliotic scarring, and axonal loss.7 Pathophysiological concepts revealed that exposure to some infectious agents during childhood as well as genetics involvement played a role.8,9 Data reported that females were more affected by MS than males due to genetic predisposition.3 Increased risk of MS was related to some environmental factors like obesity, smoking, and low level of vitamin D.10 It was reported that breastfeeding for more than 4 months is associated with lower risk for multiple sclerosis.11 The etiology of the disease might be related to consanguinity and some local factors.12Recent epidemiological data indicated higher prevalence of MS in Arabian Gulf region, with increasing incidence in Kingdom of Saudi Arabia (KSA).12 In the Gulf area, environmental conditions on one side and modulated lifestyle practices in the other side were palmed as associated factors for increasing MS prevalence.12,13 The objectives of the first MS multicenter registration in KSA which was carried out between 2015 and 2018 were studying the epidemiology of MS, and its pattern besides studying its clinical findings in the whole country. The result of this registration was alarming and warranted prompt community health actions as it indicated an increasing prevalence of the disease in the whole Kingdom.15 Among Saudi nationality the prevalence was 61.95/100,000; whereas between the whole population it was 40.40/100,00016.According to the available literature, community awareness of MS is generally unacceptable. This affects a person’s perception of the disease, delaying the opportunity for early diagnosis and management. Furthermore, it is essential to increase community awareness of MS, as better public understanding of the facts of the disease will engage patients more in the community, and create social activity, education, and employment opportunities. The aim of this study is to evaluate the awareness and level of knowledge regarding MS among the residents of Qassim Region.     

Stroke mimics: Clinical characteristics and outcome

Objectives:To study the prevalence and nature of stroke mimics (SM) among Saudi patients who came to the emergency department with a sudden neurological deficit and suspected stroke.Methods:The electronic health records from February 2016 to July 2018 of patients who were admitted to the Stroke Unit at King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Riyadh, Kingdom of Saudi Arabia (KAMC-RD) with a suspected stroke were all reviewed. A comparison between SM and stroke was established. Our study identified the predictors of SM by using logistic regression analysis. This study was approved by the local institutional review board.Results:Out of 1, 063 patients, 131 (12.3%) had SM. The most common causes were a peripheral vestibular disorder (27.4%) followed by psychogenic causes (24.4%). Stroke mimics were more common among younger individuals and women. Arterial hypertension, diabetes, and smoking were less likely to be found in SMs. At discharge, individuals with SM were more likely to be independent, had milder deficits, and shorter hospital stays. Predictors of SM were young age, female gender, mild deficit at presentation, and good functional status before the stroke.Conclusion:The incidence of stroke mimics is common among suspected stroke patients. Practicing physicians should consider potential diagnostic errors, particularly in the hyperacute phase of the stroke.

Stroke is one of the most common leading causes of mortality worldwide.1 Annually, the diagnosis of stroke in about 25 million people. Moreover, 6.5 million people die from stroke each year.2 Stroke is a life-threatening condition and requires immediate assessment so that patients can receive time-critical interventions such as tissue plasminogen activator (tPA). Therefore, accurate diagnosis is crucial. Sudden onset of neurological symptoms, or signs localized to brain arterial territories, are cause for suspicion of stroke. In most of these cases, a clinical assessment supported by brain computed tomography (CT) and basic laboratory tests are typically enough to make an accurate diagnosis in an emergency department. However, the misdiagnosis of stroke is relatively common. Stroke mimics (SM) are defined as acute onset of focal neurological symptoms, which later diagnosed with a non-vascular origin.3 In some patients with SM may erroneously receive intravenous thrombolysis therapy.4,5Stroke mimics are common in patients with suspected stroke.3-7 Conditions that mimic stroke include metabolic disorders such as hypoglycemia, hyperglycemia, hypernatremia, hyponatremia, uremia, metabolic encephalopathy, and hyperthyroidism.4,6,7,8 Moreover, migraine, seizure, psychological disorders, demyelinating diseases, and brain tumors may also mimic stroke.4,6-8 The characteristics and statistics about SMs among suspected cases of stroke in Saudi Arabia have not been reported. Yahia et al. reported that 15.9% of patients with suspected stroke were SMs, psychiatric etiology was the commonest.9 In this study, we estimated the prevalence, types, and predictors of SM among Saudi patients.     

Complementary and alternative medicine use among ischemic stroke survivors in Jeddah,Saudi Arabia

Objectives:To determine the prevalence and pattern of complementary and alternative medicine (CAM) use and the CAM types used by stroke patients in Jeddah, Kingdom of Saudi Arabia.Methods:This cross-sectional study included 152 ischemic stroke survivors who visited King Fahad Hospital, Jeddah, Kingdom of Saudi Arabia, between January 2018 and December 2019. Phone-based and face-to-face surveys were conducted. Data on the patients’ demographic characteristics, their use of CAM, and their pattern of CAM use were gatheredResults:Ninety (59.2%) stroke patients used CAM, mainly cauterization (29.61%) and Quran recitation by a religious sheik (28.95%). Most of the patients (72.22%) used CAM post-hospitalization and within less than one month from stroke onset (62.22%). A minority (6.67%) of the patients stopped their medication while on CAM. Some patients (25.56% to 31.11%) discussed with their physician their use of CAM. The CAM users reported a subjective improvement in their physical weakness (45.6%) and psychological wellbeing (62.2%). The patients learned about CAM mainly from their relatives and friends (96.7%), and the most common reason for their CAM use was their belief in this intervention (42.22%).Conclusion:The CAM use was prevalent among our cohort of Saudi ischemic stroke patients, and cauterization and Quran recitation were the most commonly used CAM interventions. An effective communication was lacking between the medical professionals and the stroke patients as regards CAM use despite the interest of the patients in this intervention.

Over the past 2 decades, the burden of stroke has increased globally.1 Stroke is the second leading cause of morbidity and mortality; the annual incidence of stroke is approximately 15 million, and over 5.5 million stroke-related deaths occur annually.2–4 Also, the prevalence of the modifiable risk factors of stroke is increasing, and the burden of stroke increases with the aging of the population.5 Adults aged >25 years have an estimated lifetime stroke risk of 24.9%.6 Despite the advancements in acute stroke care, highly effective treatments for the later phases of stroke are lacking. This shortcoming in the stroke care leads patients and their relatives to seek other treatment options, such as complementary and alternative medicine (CAM).7 The CAM is defined by the United State of America National Institutes of Health as “a group of diverse medical and health care systems, practices, and products that are not generally considered conventional medicine.”8 The CAM has been used for a long time now,9 and people widely practice CAM for various health conditions, such as cancer, epilepsy, multiple sclerosis, infertility, surgical procedure, dermatological conditions, hypertension, pain, and other types of illnesses.1,9–11 The prevalence of CAM use varies by country. For example, the prevalence of CAM use is 82% in the United States,12 61% in Australia,13 51% in Malaysia,14 and 61% in Turkey.15 Religion, cultures, and values influence the types and patterns of its use.16 People in Western countries practice herbal medicine, reflexology, aromatherapy, massage, osteopathy, meditation, and spiritual healing;10,17–19 by contrast, those in Asian countries such as China, India, and Taiwan commonly use therapies such as traditional Chinese medicine, Ayurvedic medicine, physical exercises, acupuncture, Tai Chi, yoga, and homeopathy.20–23 In Saudi Arabia, different types of CAM are used including the Quran recitation therapy, honey consumption, plant-based traditional medicine (e.g., Murrah and black seed consumption), cupping (“Hojamah”), and cauterization (“skin ironing”)9,24–27Research highlighted that aside from their potential benefits, some types of CAM have many side effects and potential toxicities, and patients are commonly unaware of these possible dangers.28 One study reported 2 cases wherein the cauterization led to severe complications, such as skin burn.29 Another study that investigated the components of herbal medicines in Saudi Arabia indicated that 15.7% of the tested samples contain toxic amounts of heavy metals, including arsenic and mercury.30 Despite the potential risk of many CAM interventions, it has been noted that many patients do not inform their physicians about their practice of CAM.31Stroke patients in many countries, including the United States (30.6%–46%)32,33 and South Korea (54%), use CAM.34 In Riyadh, Saudi Arabia, 67% of patients with neurological problems use CAM,9 and most of them use cupping (Hojamah) (45.4%), herbal medicine (42.3%), cauterization (33.7%), and Quran recitation (20.4%).9 However, no available study has analyzed the use of CAM particularly by stroke patients in Saudi Arabia. Thus, this study aimed to determine the prevalence and pattern of CAM use and the types of CAM being practiced by ischemic stroke patients relation to their demographic data.     

Electrophysiologic severity of carpal tunnel syndrome in diabetic patients of the Saudi population

Objectives:To study the frequency of multiple vascular risk factors and electrophysiological severity of carpal tunnel syndrome (CTS) in Saudi diabetic patients.Methods:This retrospective cross-sectional study was conducted in Neurology Department, King Fahd Hospital of University, Al-Khobar, Kingdom of Saudi Arabia from April 2017 to March 2018 and included 200 patients with CTS. Body parameters, such as blood pressure (BP), weight, height, and body mass index (BMI), along with laboratory and median nerve electrophysiological parameters, of diabetic and non-diabetic patients were compared, and a p-value<0.05 was considered significant.Results:Frequency of hypertension (HTN) and obesity was significantly higher in diabetic patients (p<0.05). Mean median nerve sensory amplitude (MNSA) was lower in diabetic patients (p<0.05).Non-recordable nerves, as well as bilateral and extremely severe CTS (p<0.05), were more frequently seen in diabetic patients. Age, BMI, systolic BP, low serum high density lipoprotein (HDL), high triglycerides, high fasting blood sugar, and high glycated hemoglobin (Hba1c) levels, known to affect the electrophysiological severity of CTS, had a statistically significant association with diabetes.Conclusion:Diabetes mellitus (DM) and obesity are the most commonly identified risk factors of CTS. Dyslipidemia, HTN and obesity are more frequently seen in diabetic patients with CTS. These concurrent risk factors are confounding the electrophysiological severity of CTS in these patients. Further larger-scale studies with the control of confounding factors are recommended.

Carpal tunnel syndrome (CTS) is known to have a frequent nerve entrapment syndrome and encompasses 45% of non-traumatic nerve lesions.1,2 Carpal tunnel syndrome can result in various problems, including pain and paresthesia in the median nerve distribution, swelling, and in severe cases weakness of the thumb and lateral 3 fingers.3 It affects the daily life activities, such as holding and gripping things by hand, brushing teeth, and driving.4 Carpal tunnel syndrome can be associated with any risk factor that causes pressure on the median nerve inthe wrist, including coexisting comorbidities and working conditions of the individuals.5 Some common conditions that can lead to CTS includes obesity, DM, oral contraceptives, smoking, corticosteroid use, pregnancy, hypothyroidism, rheumatoid arthritis, osteoarthritis, and wrist fracture.6The prevalence of CTS in diabetic patients is 14% without diabetic neuropathy and 30% with diabetic neuropathy.7 Literature has shown a high incidence of CTS in pre-diabetic states.8 Some researchers have also found a relationship between duration of diabetes, Hba1c, and micro vascular complications.9 Although type 2 diabetes is more frequently diagnosed among CTS patients, some studies had reported that the association between diabetes and CTS represents a confusion bias, most likely due to the strong relationship between obesity and type 2 diabetes.10 It has been shown that age, BMI, and other vascular risk factors, including metabolic syndrome, could affect the electrophysiological severity of CTS. Elevated low density lipoprotein (LDL) cholesterol and hyperglycemia were reported as independent risk factors for CTS in some studies.8,11,12 Similarly, obesity, elevated triglycerides, elevated LDL cholesterol and hypertension were shown to be strongly associated with CTS.13 In the study conducted by Balci et al,14 75% of the CTS patients were found to have metabolic syndrome, and the electrophysiological parameters (median nerve sensory onset latency, sensory conduction velocity, sensory amplitude, distal motor latency, motor conduction velocity, and motor amplitude) were worse in patients with metabolic syndrome. Gül et al,15 similarly showed that severity of CTS was even more severe in patients with metabolic syndrome than in those with diabetes.The aim of the present study was to study the frequency of multiple vascular risk factors, such as HTN, dyslipidemia and obesity in CTS patients, and to compare the electrophysiological severity of CTS in Saudi diabetic and non diabetic patients. This population is facing a high burden of multiple vascular risk factors, which are also affecting the severity of CTS.     

Demographic characteristics and types of stroke in Southwestern Saudi Arabia,and the potential demand of neuro endovascular specialists

Objectives:To identify demographic characteristics, and types of stroke in Southwestern (Aseer) Region, Saudi Arabia.Methods:This study is a retrospective and hospital-based. The data of all stroke patients (n=562) admitted to the tertiary care hospital in the province, Aseer Central Hospital (ACH) within the period from January 2016 until December 2017 are included.Results:The mean ± SD of stoke patients’ age was 62.6 ± 17.0 years. Male patients were more than female patients (62.6% and 37.4%, respectively). The majority (93.2%) were Saudi, while 91.5% had below university education and 6.4% were smokers. Interestingly, only 2% of patients had a family history of stroke, while 68.1% were diabetic, 80.4% were hypertensive, 91.6% had high serum cholesterol level, and 10.7% had history of transient ischemic attacks (TIA). Despite the lack of awareness, and leading to delay in reaching health care facility, 58.1% of stroke patients, were arriving to emergency department (ED) in less than 24 hours (potential endovascular therapeutic window).Conclusions:In Aseer Region, Saudi Arabia, stroke affects mainly those who are older, less educated, diabetic, hypertensive, with hypercholesterolemia and previous history of TIA. Associated factors for stroke differ significantly according to their nationality.

Across the globe, stroke is now one of the most common leading causes for morbidity and mortality in adult patients after myocardial infarctions. Together with neoplasm, and myocardial infarctions, the 3 are top causes for death in the developed countries. Even when survive, a major portion of affected patients may suffer lifelong consequences in the form of physical or mental disabilities, which may result in social and economic challenges at the personal level and the society level.1 Two types of brain stroke exist, hemorrhagic and ischemic. There has been more than a 100% rise in stroke incidence in limited-resources countries.2 Stroke is one of the top 3 leading causes of death in the United States of America (USA). Approximately, 140,000 fatalities per year are caused by stroke in the USA. In year 2000, stroke resulted in 15,409 deaths (7%) of all deaths in Canada.3 As per statistics of the World Health Organization,4 almost 15 million annual cases of stroke are recorded worldwide. One third of stroke patients dies and another third remains disabled for the rest of their lives. Indonesian Basic Health Research for instance, estimated that the prevalence of stroke in Indonesian population is somewhere around 12.1 per million whereas in China, the prevalence of stroke somewhere around 2.21%.In order to prevent and minimize the catastrophic sequelae of stroke, early detection is the main key as well as, determining the type of stroke, which is an essential element in clinical care of stroke patients. History and physical examination can provide a great help in distinguishing the type of stroke;5,6 however, evolving diagnostic images are the main keys in sorting stroke types.7 The incidence of stroke is known to increase as age increases, more specifically, after the age of 45 years, the incidence gets doubled every decade of life. More than 70% of the strokes are occurring in patients who are 65 years or older.8 Men (1 in 5) tend to have higher lifetime risk of stroke than women (1 in 6). Men tend to have more risk of stroke at younger age than women, where women have higher rates of stroke at older age as they tend to have longer life expectancy than men.9 Stroke is increasing rapidly in the area of the Middle East.10 Some studies done in this part of the world, to look for the stroke associated factors and found that age, hypertension, previous stroke or history of transient ischemic attack (TIA), diabetes mellitus, hypercholesterolemia, smoking, and atrial fibrillation are the major ones.1 Some national studies have found that patients with diabetes mellitus, hypertension, or high cholesterol are more vulnerable for stroke11 and the prevalence of 178 in every 100,000, was reported in the Eastern region of Saudi Arabia.12 Similar to other parts of the world, stroke burden is rapidly evolving and becoming a major cause of illness and deaths in Saudi Arabia. Akbar and Mushtaq13 found that stroke is more frequent in patients at their sixth decade of life with clear male predominance in Saudi Arabia. According to Almekhlafi’s hospital based study,14 stroke burden is increasing in Saudi Arabia. Out of 548 patients in the study, stroke and its consequences contributed to 32% of the mortality.In 2014, Robert and Zamzami1reported that only 8 centers of 350 hospitals all around Saudi Arabia, are providing stroke thrombolytic therapy, and 2 out of these 8 centers have stroke code teams. However, by 2019, 8 more centers developed stoke code teams, and more hospitals-initiated stroke thrombolysis therapy. The present study aimed to identify demographic characteristics, clinical findings, and types of stroke in Aseer Region, Saudi Arabia.     

Spectrum of medulloblastoma subtypes and frequency of MYC amplification; Experience from a tertiary care center in Saudi Arabia

Objectives:To clarify the spectrum of morphological and molecular subtypes of medulloblastoma (MBL), in addition to MYC and MYCN amplification statuses in a cohort of Saudi patients. The latter was correlated with patient outcome.Methods:We conducted a retrospective cohort study of 57 patients with MBL, diagnosed at the central laboratory of King Abdulaziz Medical City in Riyadh, Saudi Arabia, between 2006 and 2019. Molecular analysis for MYC and MYCN amplification was performed for the 19 most recently diagnosed patients.Results:Classic MBL was the most prevalent histologic subtype and MBL with extensive nodularity was the rarest. The non-WNT/non-SHH molecular subgroup was the most common while the WNT-activated was the least common. Among 19 patients analyzed, MYC and MYCN amplifications were discovered in 2 (10.5%) and 1 (5.3%) cases, respectively, using interphase fluorescence in-situ hybridization. The 2 MYC amplified cases belonged to the large cell/anaplastic subtype and had the worst outcomes.Conclusion:The MYC amplification corresponded with poor prognosis, the large cell/anaplastic variant of MBL, and the non-WNT/non-SHH molecular subtype.

Medulloblastoma (MBL) is the most prevalent pediatric embryonal brain neoplasm originating from the cerebellum and dorsal brain stem.1 There are 4 histomorphological variants of MBL: classic, desmoplastic/nodular, large cell/anaplastic, and MBL with extensive nodularity.1–3 The MBLs are now classified into 4 molecular subtypes: Wingless (WNT)-activated, sonic hedgehog (SHH)-activated, group 3, and group 4. Groups 3 and 4 are less well defined from a molecular standpoint, and are difficult to distinguish from each other using commercially available surrogate biomarkers.4 For practical purposes, MBLs are therefore subdivided into 3 molecular subgroups: WNT-activated, SHH-activated, and non-WNT/non-SHH.Traditionally, patients with MBL were stratified according to their risk of recurrence (average risk: >3 years of age, no/minimal residual disease post-surgery, and no CNS metastasis; and high risk: <3 years of age exclusive to the extensively nodular subtype, significant residual disease post-surgery, and evidence of metastasis within the CNS).4The MYC amplifications have long been associated with highly aggressive MBLs, and occur in 5% to 10% of MBLs.5 The purpose of this study was to recognize the spectrum of MBL histologic and molecular subtypes seen at our institute and to estimate the frequency of MYC and MYCN amplification. Additionally, the prognosis of the MYC amplified cases was compared to that of their non-amplified counterparts.     

An assessment of cerebral venous thrombosis risk factors and associated clinical outcomes in Jazan region,Saudi Arabia

Objectives:To assess cerebral venous thrombosis risk factors, and associated clinical outcomes in Jazan region, Kingdom of Saudi Arabia.Methods:This study is a retrospective review of the medical records of patients diagnosed with cerebral venous thrombosis and admitted to King Fahad Central Hospital in Jazan between 2010 and 2019. Data concerning socio-demographics, clinical features, risk factors, laboratory, and imaging investigations were retrieved. Furthermore, data about cases management, and outcomes, including death, were collected and analysed.Results:A total of 51 medical records were identified. The majority of the patients were females (68.6%), and the mean age of the patients was 33.3 years, of which three patients were under 18 years old. The most frequently recorded symptom was headache (76.5%), followed by seizure (45.1%). The most commonly recorded risk factor was protein S deficiency (57%), followed by anaemia (51%). Venous infarction and haemorrhage were the most common acute complications (13.7%). The majority of the patients had a favourable prognosis where only 27.5% recovered with disability and only one patient died due to the disease.Conclusion:Clinical presentation of cerebral venous thrombosis in Jazan region is similar to other local and international studies. However, anaemia was recorded as a main risk factor for the disease, which might require further investigation to assess the possible association between prevalence of anaemia in Jazan region and the incidence of cerebral venous thrombosis.

Cerebral venous thrombosis (CVT) is a rare form of cerebrovascular disease in comparison with arterial stroke. CVT cases represent approximately 0.5-1% of all types of stroke which mainly occur in young and middle-aged adults.1 The data concerning the global epidemiology of CVT is currently limited.2 However, the incidence of CVT has been reported to vary between countries where the incidence might be higher as in Asian and the Middle Eastern countries in comparison to Australia and European countries.3According to a recent study conducted in Australia, the incidence of CVT was reported to reach 15.7 per 1,000,000 persons on a yearly basis. The incidence was higher among women and among those between 31-50 years old.4 In the Middle East, an Iranian study looked at the frequency of CVT between 2001 and 2004, and the annual frequency of CVT was 12.3 per one million.5 An older study, conducted in the city of Riyadh in Saudi Arabia between 1985 and 1994, identified 40 cases of CVT. Those identified were aged between 16 and 40.6 In addition, in a more recent study conducted in Jeddah and Al-Baha between 1990 and 2010, the number of detected cases of CVT was 111 where 19 of these were detected among children.7The CVT occurs when a thrombus develops as a result of a disturbance of the balance between the process of prothrombosis and thrombolysis.8,9 Risk factors for CVT can be categorised into transient and permanent risk factors. Permanent risk factors are hereditary thrombophilia, systemic diseases or miscellaneous factors, such as obesity. Transient risk factors can be subcategorised into sex-specific, iatrogenic, or miscellaneous risk factors, such as infection, head trauma or anaemia.8 The prevalence of CVT risk factors differs between countries. Infection, pregnancy, post-partum period, and dehydration have been reported to be more common in Asia and the Middle Eastern countries in comparison to European countries.8Patients with CVT exhibit variable clinical manifestations and complications, some of which can be life threatening. The most common clinical presentation is a headache, while some patients exhibit other signs and symptoms, such as seizure, decreased level of consciousness, vomiting, focal neurological deficit, or visual symptoms.8-10 Venous infarction and haemorrhage are frequently reported complications of CVT.11 Late presentation of CVT patients can increase the risk of disability and death. The mortality rate among CVT patients has been reported to vary between 4.3% and 6.8%.12Since CVT risk factors and vulnerable groups can vary between different populations, assessment of the distribution of risk factors among local populations can be clinically valuable. Studies assessing CVT prevalence and associated risk factors and clinical outcomes in Saudi populations are currently limited. Furthermore, data about CVT in Jazan region is currently lacking. This investigation aims to identify cases diagnosed with CVT in Jazan region and to evaluate the risk factors and associated clinical outcomes.     

Visual evoked potentials follow-up in idiopathic intracranial hypertension

Objectives:To demonstrate the importance of visual evoked potential (VEP) in determining the severity and prognosis of the disease and in monitoring the clinical course in patients with idiopathic intracranial hypertension (IIH).Methods:This is a prospective cross-sectional study conducted covering the period between March 2014 and January 2015. The study included 32 patients recently diagnosed with IIH and 30 healthy volunteers. The initial VEP values of the IIH patients were compared to the VEP values of the healthy control group. Furthermore, the initial VEP values of the IIH patients were compared with their VEP values after one month of treatment.Results:The mean age of the IIH patients was 37.8±12.02 years. Of the IIH patients, 27 (84%) were females and 5 (16%) were males. There was a statistically significant association of the initial VEP values with the visual field findings (p=0.011) and visual acuity (p=0.040). Moreover, a statistically significant difference was found between the control group and IIH patients in terms of right (p<0.001) and left P100 values (p<0.001). While 18 (56%) of the initial VEPs of IIH patients were pathological, 14 (44%) of the second VEPs were pathological, and this difference was not statistically significant (p=0.125).Conclusion:A relationship between the VEP P100 values and the severity of the disease was detected, while the importance of monitoring VEP values in the follow-up of IIH patients was not demonstrated.

Idiopathic intracranial hypertension can be defined as raised intracranial pressure with a normal cerebrospinal fluid (CSF).1,2 The overall incidence of IIH is 2.4/100,000; however, the incidence is 22/100,000 in obese women.3 Indeed, the female gender and a high body mass index (BMI) are important risk factors for the disease and female patients account for 90% of the cases.4,5The visual field is commonly affected in IIH patients.6 Although the visual field is currently the most sensitive method to monitor visual disturbances during the course of the disease, its specificity and sensitivity is not 100%.6,7 However, an early diagnosis, determination of the disease severity, and follow-up of the patients with IIH are critical as IIH can cause serious and irreversible visual field defects and even vision loss during the course of the disease.7-9The number of patients with IIH is on the rise globally as the number of patients with obesity increases day by day.10 There is a paucity of precisely sensitive and objective indicators of the disease for monitoring relapses and remission.11,12 Moreover, no sensitive and objective methods have been established yet to determine the disease severity in order to decide whether surgery should be performed in these patients.12-14Despite the potential of IIH to cause visual morbidity, only a limited number of studies are available in the literature to predict the potential impact of IIH on the vision.13 The findings obtained by VEP reflect the functions of the visual pathways extending from the retina to the occipital cortex. The test is particularly sensitive to demonstrate conduction disturbances in the anterior (pre-chiasmatic) visual pathway.15,16 A few studies have shown abnormal findings in VEP tests in patients with IIH, indicating the importance of VEP in the evaluation of the visual functions.17 In the literature, we found only one study evaluating the value of VEP values in the follow-up of patients with IIH.17This study aimed to demonstrate the importance of VEP testing in determining the severity and prognosis of the disease and in monitoring the clinical course of the patients with IIH as the number of studies evaluating this subject is limited in the literature.     

Predicting future handedness and hemispheric dominance during infancy by analyzing sleep spindles

Objectives:To investigate whether sleep spindles asynchrony (SSA) using scalp sleep electroencephalogram (EEG) among children below 2 years of age can predict future handedness.Methods:This is a retrospective study conducted from October 2016 until June 2017 at the King Fahad Medical City (KFMC), Riyadh, Kingdom of Saudi Arabia. We retrospectively reviewed 300 EEGs recorded at our neurophysiology laboratory.We included EEGs performed during sleep for infants aged 2 months to 2 years who have already attained their handedness or those aged above 2 years. We excluded records of children younger than 2 months or above 2 years of age (at the time of the EEG) or those aged below 2 years (at the time of the interview), and severely abnormal tracings and those without sleep or enough SSA .Results:The lateralization of Sleep Spindles (SS) was mostly right-hemispheric (52%) compared to left-hemispheric (36.4%). The overall SS laterality did not correlate with handedness (p=0.81). In the majority of right-handed (64%) and left-handed (60%) children, the SSA was contralateral to the side of hand preference; however, it did not correlate statistically (p=0.377).Conclusion:We were unable to prove a statistically significant correlation between SSA and future hand preference. Further research involving larger cohorts is still needed.

Sleep architecture is an essential part of evaluating any electroencephalogram (EEG). Despite being recognized and described as early as the discovery of EEG, sleep features remain understudied. In most children, Sleep Spindles (SS) do not synchronize over the 2 hemispheres until the child develops Handedness, usually at 2 years of age. This coincidence of the establishment of Handedness and synchrony of SS makes the connection between the 2 maturational developments important. We planned to investigate the possibility of anticipating future hemispheric dominance and future Handedness using the concept of Sleep Spindles Asynchrony (SSA). The SS consist of bilaterally symmetric low to medium voltage bicentral synchronous rhythmic spindle-like waves, with frequency ranging from 12 to 16 Hz, which often appear along K-complexes lasting approximately 2-3 seconds.1 The identification of SS is easy in any sleep EEG due to their abundance in infants and children. Their typical location in adults is a synchronous burst of waves around the vertex; in children, it varies according to age.2–7 It is thought that the origin of sleep spindles is the thalamocortical network. However, there is no functional meaning definitively established for this mechanism so far, which makes our study relevant to this area.3 The SS are not seen in the first 2 months of life and start to appear afterward.8 The absence of sleep spindles during mid-infancy is considered a significant abnormality. However, their independent occurrence (asynchrony) below 2 years of age is common and a normal finding.9–12 These sleep milestones are consistent between subjects, and therefore they are an essential indication of functional maturation.13 Chatburn et al14 found that sleep spindle activity was associated with various aspects of maturation and is an integral part of brain development. It is suggested that sleep EEG may be a marker for intellectual ability.15 Specifically, SS are associated with the intelligence quotient (IQ).16 The SS are also found to be involved in procedural memory.17 There are a few speculations about how Handedness is created in individuals. Although antenatal mechanisms might be essential, analysis considering in utero development verified that Handedness was an extremely accurate indicator of hemispheric dominance after birth.18 Early hand preference does not develop before one-year of age, and the usual age at which Handedness appears is 2 years, coinciding electrographically with SS synchrony, making a causal relationship or mechanism between the 2 markers highly likely.19–20 There is no previously published research to date to have studied this area.21