Cost-effectiveness of HARNESS-MRI protocol in focal drug-resistant epilepsy in a limited-resources country: An Egyptian study.

OBJECTIVES: The international league against epilepsy (ILAE) recommended the harmonized neuroimaging of epilepsy structural sequences (HARNESS-MRI) to improve the detection of epileptogenic lesions in patients with focal drug-resistant epilepsy (DRE). The application of this protocol is still limited in low-resource countries, mainly due to apparent high costs. We aimed to evaluate the cost-effectiveness of the HARNESS-MRI protocol in Egypt and highlighted our experience. METHODS: Patients diagnosed with focal DRE at Cairo University epilepsy clinic underwent both conventional MRI (c-MRI) and HARNESS-MRI. Electro-clinical data were collected and analyzed. After the radiologists' initial diagnosis, a multidisciplinary team re-evaluated the MRI. Lesion detection rate and cost for detecting an extra lesion by HARNESS-MRI protocol were calculated. RESULTS: The study included 230 patients with focal DRE (146, 62% males and 91, 38% females), with a mean age of 20.5 years. Epileptogenic lesions detected by c-MRI and HARNESS-MRI before and after the board meeting were 40, 106, and 131 lesions, respectively (P < 0.001). Sixty-nine percent of the lesions detected by HARNESS-MRI were missed on c-MRI; most commonly were mesial temporal sclerosis (MTS) and Malformations of cortical development (MCDs). Thirty-seven MTS and 32 MCDs were detected with HARNESS-MRI, compared to only 6 and 3, respectively, detected on c-MRI (P < 0.001). HARNESS-MR protocol is more cost-effective than c-MRI in detecting MRI lesions; it can save about 42$ for detecting an extra lesion in MRI. CONCLUSION: The HARNESS-MRI protocol was cost-effective and highly recommended even in limited-resource countries for patients with focal DRE.

Resective epilepsy surgery in a limited-resource settings: A cohort from a multi-disciplinary epilepsy team in a developing country.

Background: Multidisciplinary pre-surgical evaluation is vital for epilepsy surgery decision and outcomes. Resective epilepsy surgery with assisted monitoring is currently a standard treatment for focal drug resistant epilepsy (DRE). In resource-limited countries, lack of epilepsy surgery center is a huge challenge. We presented and illustrated how to create a multidisciplinary protocol with resource-limited settings in a developing country and epilepsy surgery outcome using brain mapping and monitoring techniques for ensuring satisfactory resection. Methods: We created multicentric incomplete but complementary units covering all epilepsy-related sub-specialties and covering a wide geographical area in our country. Then, we conducted a prospective and multicentric study with low resource settings on patients with focal DRE, who underwent resective epilepsy surgery and were followed up for at least 12 months and were evaluated for postoperative seizure outcome and complications if present. Preoperative comprehensive clinical, neurophysiological, neuropsychological, and radiological evaluations were performed by multidisciplinary epilepsy team. Intraoperative brain mapping including awake craniotomy and direct stimulation techniques, neurophysiological monitoring, and electrocorticography was carried out during surgical resection. Results: The study included 47 patients (18 females and 29 males) with mean age 20.4 ± 10.02 years. Twenty-two (46.8%) patients were temporal epilepsy while 25 (53.2%) were extra-temporal epilepsy. The epilepsy surgery outcome at the last follow up was Engel Class I (seizure free) in 35 (74.5%), Class II (almost seizure free) in 8 (17%), Class III (worthwhile improvement) in 3 (6.4%), and Class IV (no worthwhile improvement) in 1 patient (2.1%). Conclusion: With low resource settings and lack of single fully equipped epilepsy center, favorable outcomes after resective surgery in patients with focal DRE could be achieved using careful presurgical multidisciplinary selection, especially with using intraoperative brain mapping and electrocorticography techniques.

Non-convulsive status epilepticus in Sotos syndrome: rare first presentation in a rare syndrome.

INTRODUCTION: Prior to illustration of the causative genetic mutation responsible for Sotos syndrome, diagnosis was based on clinical criteria. They include characteristic facial gestalt, developmental delay, and evidence of overgrowth, in addition to other minor features as cardiac &genitourinary congenital malformation, seizures, scoliosis, among other features. Non-convulsive status epilepticus (NCSE) was not previously reported among Sotos patients. CASE SUMMARY: An eleven-years old boy, with developmental delay, characteristic facial & skeletal features presented to the emergency department with a two-hour episode of lapse of consciousness. Electroencephalogram (EEG) showed fluctuating generalized spike-wave/poly-spike wave discharge <2.5 Hertz (Hz), lasting throughout the duration of recording. Intravenous (IV) levetiracetam was associated with clinical & EEG improvement & accordingly the patient was diagnosed as NCSE. The mother reported history of polyhydramnios, febrile seizure & developmental delay. Through clinical & radiological assessment revealed generalized hypotonia, low intelligence quotient (IQ), congenital ureteric stricture & pulmonary hypertension, prominent retro-cerebellar cistern, in addition to scoliosis & facial features suggestive of Sotos Syndrome. Six months after presentation, the patient remained seizure free on levetiracetam monotherapy. CONCLUSION: NCSE could occur in Sotos syndrome. In our case, the first reported case of NCSE in Sotos syndrome, the characteristic facial & skeletal findings initiated further work up with fulfillment of the criteria required for the clinical diagnosis of Sotos syndrome.

Factors associated with the use of anesthetic drug infusion in patients with status epilepticus and their relation to outcome: a prospective study.

Status epilepticus (SE) is one of the most dreadful neurological emergencies; unfortunately, studies targeting SE are still inadequate. This study aims to identify factors associated with the use of CIVAD in patients presenting with status epilepticus and detect those impact the clinical outcome. A prospective study involving 144 episodes of SE in 144 patients. Patients were categorized according to whether or not they received CIVAD. Subjects underwent clinical assessment, brain imaging, and EEG. The consciousness level was assessed using the Glasgow coma scale (GCS) and the Full outline of responsiveness (FOUR) scale. SE severity score (STESS) and Epidemiology-based mortality score (EMSE) were used as scales for outcome prediction. Continuous IV anesthetic drug infusion was initiated in 36% of patients (+ CIVAD). Such groups showed a significantly worse initial level of consciousness (< 0.001), an unstable course of seizure evolution (0.009), and all of them showed abnormal EEG patterns. A significantly higher number of patients (+ CIVAD) developed complications (< 0.001), had higher outcome prediction scores (< 0.001), and mortality rates (< 0.001) compared to those who did not need CIVAD (- CIVAD). Mortality was associated with acute symptomatic etiology and higher total doses of propofol. Among the study population, mortality among patients who received CIVAD was associated with acute symptomatic SE and prolonged propofol infusion rather than any clinical parameters or predictor scores.

Characteristics and outcome in an Egyptian Cohort with status epilepticus.

OBJECTIVES: Status epilepticus (SE) is an important cause of mortality worldwide. Information regarding associations of mortality outcome in Egypt is limited. The main objective of this study was to describe the clinical characteristics and factors associated with mortality of patients with SE admitted to Cairo University Hospitals. METHODS: A prospective observational hospital-based study was conducted from January 2017 to June 2018. Patients with SE aged ≥12 years old were followed with documentation of outcome on discharge as survival versus death. RESULTS: The study cohort included 144 SE patients. The majority of episodes (96.5%) were with prominent motor features, while nonconvulsive SE occurred in 3.5%. There was a clear semiology evolution in 22.9% of episodes, 56.3% had unchanging semiology (e.g., just convulsive or just nonconvulsive) and the semiology evolution could not be discerned in 20.8%. In relation to treatment, the majority of cases were responsive (46.5%), whereas 43.1% were refractory and only 10.4% were super-refractory SE. Apart from seven patients who were referred to other hospitals, 99 patients survived whereas 38 (26.4%) died. After regression analysis, only absence of a history of epilepsy, semiology evolution and mechanical ventilator use were associated with mortality increasing its odds by 3.7, 5, 111 times, respectively. CONCLUSION: Absence of a history of epilepsy, SE semiology evolution, and mechanical ventilator use was found to be associated with mortality outcome among Egyptian patients with SE.

Psychogenic Nonepileptic Seizures in Patients With Epilepsy: A Comparative Study With Patients With Pure Epilepsy.

ABSTRACT: Psychogenic nonepileptic seizures (PNES) represent management challenges, especially if associated with epilepsy. We aimed to evaluate patients with mixed epilepsy (true and PNES) and compare them with pure epilepsy to identify predictors of psychogenic seizures. This study included 40 patients with pure epilepsy and 40 patients with mixed epilepsy matched in age and sex. Patients underwent neurological assessment, semistructured psychiatric clinical interview, and video electroencephalogram monitoring. We found that unemployment, divorce, and seizure frequency were higher in mixed epilepsy, as well as history of family dysfunction, child adversity, and depressive and dissociative disorders. Both groups were similar regarding family history for seizures and personality dysfunction scores. Family dysfunction, child adversity, and depressive disorders were predictors of PNES. We recommend early evaluation for social instability, family dysfunction, child adversity, and depressive disorders in epileptic patients with higher seizure frequency to avoid misdiagnosis of false drug-resistant epilepsy and enhance proper management.

Effect of Low-Dose Naltrexone on Egyptian Children with Intractable Epilepsy: A Case Series Study.

Epilepsy is a chronic neurological disorder affecting 50 million patients worldwide, in need of continuous treatment, while 30% of them are refractory to treatment. Despite multiple antiepileptic drugs are available for the treatment of epilepsy, still refractory epilepsy especially in children represents a social burden in developing countries. Low-dose naltrexone (LDN) has been proposed as an immune modulator in multiple diseases and proved benefit especially in diseases with immune dysregulation. The purpose of this study is to demonstrate the effect of LDN in the treatment of children with intractable epilepsy and discuss its potential role in epileptogenesis process.

External validation of STESS and EMSE as outcome prediction scores in an Egyptian cohort with status epilepticus.

PURPOSE: There is a lack of data concerning the performance of the outcome prediction scores in patients with status epilepticus (SE) in developing countries. The aim of this study was to compare the predictive performances of the status epilepticus severity score (STESS) and the epidemiology-based mortality score in status epilepticus (EMSE) and adaptation of such scoring system to be compatible with the nature of society. METHOD: This is a prospective study, conducted in Egypt from the period of January 2017 to June 2018. The main outcome measure was survival versus death, on hospital discharge. The cutoff point with the best sensitivity and specificity to predict mortality was determined through a receiver operating characteristic (ROC) curve. RESULTS: Among the 144 patients with SE with a mean age of 39.3 ±â€¯19.5 years recruited into the study, 38 patients (26.3%) died in the hospital with the survival of 99 patients while 7 patients (4.9%) were referred to other centers with an unknown outcome. Although EMSE had a bit larger area under the curve (AUC) (0.846) than STESS-3 (AUC 0.824), STESS-3 had the best performance as in-hospital death prediction score as it has a higher negative predictive value (94.6%) than that of EMSE (90.9%) in order not to miss high-risk patients. CONCLUSION: In the Egyptian population, STESS and EMSE are useful tools in predicting mortality outcome of SE. The STESS performed significantly better than EMSEE combinations as a mortality prediction score.

Assessment of School-Aged Children of Mothers With Idiopathic Generalized Epilepsy.

The aim is to examine the cognitive domains, behavioral domains, and electroencephalogram (EEG) findings in children of mothers with idiopathic generalized epilepsy who had been exposed to antiepileptic drugs (AEDs) in utero. Forty school-aged children born to 23 mothers with idiopathic generalized epilepsy were compared with 40 healthy children born to 34 healthy mothers. Stanford-Binet Intelligence Scale was applied to all children to assess their cognitive functions. Child Behavior Checklist was used to assess their behavioral characteristics. EEG was done for the epileptic mothers and their children. Children exposed to AEDs showed significantly lower scores in the verbal reasoning, visual reasoning, and global intelligence quotient (IQ). There was a significantly positive correlation between children's global IQ and maternal global IQ. Multiple regression analysis showed that in utero exposure to valproate and maternal IQ were the most independent factors affecting children's IQ. EEG findings of participating children were normal. Exposure to valproic acid during fetal life and maternal IQ represent confounding factors affecting the IQ of children with in utero exposure to AEDs.

Insights into sleep-related hyper-motor epilepsy: an Egyptian case series.

Objective: Sleep-related hypermotor epilepsy (SHE) is a sleep-related focal epilepsy which is often misdiagnosed. Despite active pharmacological therapy in the management of this disorder, satisfactory seizure control still cannot be achieved. Therefore, the aim of the present study was to identify this disorder among people who were seeking medical advice at Cairo University Epilepsy Unit (CUEU), characterizing its clinical, electroencephalographic and imaging features besides identifying possible indicators of inadequate seizure control on drug-therapy. Patients and methods: This study was carried out on 26 patients with SHE who were subjected to detailed history taking and examination in addition to home video recording, video electroencephalographic (EEG) monitoring and brain imaging. Ictal semiology and EEGs were reviewed and analyzed by experienced neurologists. Results: SHE is an uncommon sleep-related focal epilepsy. In our series, median age of the patients was 18.5 years. It is characterized by being sporadic, with often frontal lobe seizure onset (14/26, 53.8%) and with occasional occurrence in wakefulness. Adolescence age at disease onset (11 years, 6-15), duration less than 1 min, clustering, lack of auras and often uninformative brain imaging (22/25, 88%) are all documented features. Moreover, it has a relatively poor outcome on pharmacological therapy (16/26, 61.5%). Longer disease duration (>4.5 years) was a significant feature of the patients exhibiting inadequate seizure control. Conclusions: Our data show relatively poor prognosis of SHE on medical therapy. Its outcome is significantly related to disease duration at the time of diagnosis. Abbreviation: SHE = Sleep related hypermotor epilepsy.

Prognosis of Bell Palsy: A Clinical, Neurophysiological, and Ultrasound Study.

PURPOSE: Bell palsy is the most common cause of acute facial nerve paralysis. Ultrasound has proved its ability in detecting structural lesions along the course of the affected nerves.The current work aimed at studying the accuracy of ultrasound to predict the prognosis of Bell palsy in correlation to the clinical scale and nerve conduction studies. METHODS: The study included 20 cases of acute Bell palsy treated with prednisolone and physiotherapy. The participants were examined using the House-Brackmann (HB) scale, electrophysiologically and neurosonologically in the affected side and healthy side that served as a control. RESULTS: There was significant correlation between HB outcomes with onset of HB results. There was significant increase in the distal facial nerve diameter on the affected side compared with the normal side (P < 0.001). Although ultrasound at onset did not predict the outcome, nerve conduction studies did predict the outcome. CONCLUSIONS: Baseline HB clinical assessment of Bell palsy gives information on the clinical outcome of the disease. In addition to that, initial nerve conduction studies proved to be superior to ultrasound in predicting the outcome.

Ictal asystole: a case presentation.

BACKGROUND: Epileptic seizures can lead to cardiac arrhythmias. The arrhythmias may be in the form of tachycardia, bradycardia or asystole. Ictal bradycardia and asystole can lead to sudden unexpected death. CASE PRESENTATION: A case report of a 40-year-old male with complex partial temporal lobe epilepsy. He has coincident attacks of fall and pallor. The patient underwent simultaneous electrocardiogram (ECG) and video electroencephalogram (EEG) monitoring. The slow activity in EEG coincide with the appearance of bradycardia in ECG then cardiac asystole which clinically correspond to the patient syncope. After insertion of a cardiac pacemaker, only complex partial attacks develop with a marked reduction in frequency and no more fall attacks. CONCLUSION: Epileptic seizures can present with cardiac arrhythmias, with ictal asystole leading to sudden unexpected death. Simultaneous EEG and ECG are essential for the diagnosis. A cardiac pacemaker can be lifesaving for patients with ictal arrhythmias.

Role of concentric needle Single Fiber Electromyography in detection of subclinical motor involvement in carpal tunnel syndrome.

BACKGROUND: Conventional motor nerve conduction studies are usually normal in early and mild carpal tunnel syndrome (CTS). Single-fiber electromyography (SFEMG) measures the mean consecutive difference (MCD) as an expression of the variability in impulse transmission over the motor endplates and along the nerve fibers distally to the last branching point and along the muscle fibers.Application of concentric needle SFEMG in a group of CTS patients who showed pure sensory abnormalities in nerve conduction studies to examine for subclinical motor involvement. METHODS: Thirty CTS patients having only sensory involvement proved clinically and by conventional electrophysiological studies were included in addition to 30 control subjects. Concentric needle SFEMG was performed to the abductor pollicis brevis (APB), abductor digiti minimi (ADM), and extensor digitorum communis (EDC) muscles. RESULTS: There was a statistically significant difference in the MCD between the patient and control groups in the APB only (p = 0.038). CONCLUSIONS: The results suggest the presence of a subclinical motor median neuropathy at the wrist in patients with early and mild carpal tunnel syndrome and highlight the validity of the concentric needle SFEMG in early neuropathies. TRIAL REGISTRATION: PACTR201802002971380 registered 12 February 2018, retrospectively registered.

Focal interictal epileptiform discharges in idiopathic generalized epilepsy.

Are idiopathic generalized epilepsies (IGEs) truly generalized? Do IGEs represent a continuum or rather distinct syndromes? Focal changes in the electroencephalography (EEG) have been reported in IGEs. The aim of this work is to investigate focal interictal epileptiform discharges (IEDs) in IGEs, and their relation to clinical variables. Forty-one IGE patients (classified according to ILAE, 2001) were recruited from a tertiary center (age 23 ± 10.938 years). Their files were reviewed and they were subjected to clinical examination and interictal EEG. Patients with focal IEDs were compared to those without focal IEDs. Nine patients had juvenile myoclonic epilepsy (JME) and 32 had idiopathic epilepsy with generalized tonic-clonic seizures only (EGTCSA). Focal IEDs were found in 20 patients, mostly in the frontal (45.5 %) and temporal (31.8 %) distribution. Patients with focal IEDs were treated with a larger number of combined antiepileptic drugs (AEDs) (p value = 0.022). No significant difference was found between the two groups regarding age, sex, age at onset, epilepsy syndrome, seizure frequency, family history, AEDs used (sodium valproate and carbamazepine) and their doses. Seventeen EGTCSA patients had focal IEDs. They were treated with larger number of combined AEDs (p value = 0.0142). No significant difference was found between the EGTCSA patients with and those without focal IEDs regarding age, sex, age at onset, seizure frequency, family history and AEDs doses. Caution must be applied in the interpretation of interictal focal IEDs. These focal changes may be related to prognosis, however this needs further investigation.