The impact of the time to last seizure before admission to the epilepsy monitoring unit (EMU) on epilepsy classifications.

INTRODUCTION AND BACKGROUND: The impact of the timing of the last seizure (TTLS) prior to admission to the epilepsy monitoring unit (EMU) on epilepsy classification is unclear for which we conducted this study. METHODS: We reviewed patients with epilepsy admitted to EMU between January 2021 and April 2022 and identified TTLS before EMU admission. We considered EMU yield as whether; it confirmed epilepsy classification, added new knowledge to the classification, or failed to classify epilepsy. RESULTS: We studied 156 patients. There were 72 (46%) men, with a mean age of 30. TTLS was divided according to a one- or three-month cutoff. We confirmed the pre-EMU epilepsy classification in 52 (33%) patients, learned new findings on epilepsy classification in 80 (51%) patients, and failed to classify epilepsy in 24 (15%) patients. Patients with "confirmed epilepsy classifications" reported seizures sooner to EMU admission than other groups (0.7 vs. 2.3 months, p-value = 0.02, 95% CI; -1.8, -1.3). Also, the odds of confirming epilepsy classification were more than two times in patients with TTLS within a month compared to those with TTLS of more than a month (OR = 2.4, p-value = 0.04, 95% CI; 1.1, 5.9). The odds were also higher when the 3-month TTLS cutoff was considered (OR = 6.2, p-value = 0.002, 95% CI; 1.6, 40.2). Confirming epilepsy classification was also associated with earlier seizures recorded at one- or three-month cutoff (OR = 2.1 and OR = 2.3, respectively, p-value = 0.05). We did not observe similar findings when we modified the classification or failed to reach a classification. CONCLUSIONS: The timing of the last seizure before EMU admission appeared to influence the yield of EMU and enhanced the confirmation of epilepsy classifications. Such findings can improve the utilization of EMU in the presurgical evaluation of patients with epilepsy.

The safety and efficacy of modifying the admission protocol to the epilepsy monitoring unit in response to the COVID-19 pandemic.

PURPOSE: The coronavirus disease 2019 (COVID-19) pandemic has impacted admission to epilepsy monitoring units (EMUs) for classification and presurgical evaluation of patients with refractory epilepsy. We modified the EMU admission protocol via anti-seizure medications (ASM) withdrawal implemented one day before admission; thus, we aimed to evaluate the efficacy and safety of this modified protocol. METHODS: In January 2021, we initiated ASM tapering 24 h before-rather than on the first day after-EMU admission, contrasting with the previous protocol. We retrospectively reviewed EMU admissions between January and April of 2018, 2019, and 2021, and identified the time required to record the first seizure, and EMU yield to confirm or change the epilepsy classification. We also evaluated the safety of the modified protocol, by monitoring the seizure frequency for up to 5 months after the discharge from the hospital. RESULTS: One hundred four patients were included (mean age: 30 years, men: 43%); excluding a longer disease duration and abundance of normal routine electro-encephalogram (EEG) in patients admitted before the pandemic, no differences were observed in patients' characteristics. On average, it took 41 h and 21 h to record the first seizure using the standard and modified protocols, respectively (p < 0.001, 95% CI: 10-30). Other characteristics were investigated both before and after the COVID-19 pandemic, and epilepsy classifications were confirmed twice using the modified protocol (OR = 2.4, p = 0.04, 95% CI: 1.1-5.5). Multivariate regression analysis confirmed the shorter time to record the first seizure using the modified admission protocol (23 h less, p < 0.001; 95% CI: 12-34). Finally, 36 (86%) patients admitted during the pandemic exhibited no increase in seizure frequency after the discharge from the hospital. CONCLUSIONS: Initiating ASM withdrawal one day before EMU admission was deemed to be an efficient and safe way to confirm epilepsy classification and significantly decrease the length of hospital stay. Ultimately, this will shorten the long waiting list for EMU admission created by the COVID-19 pandemic.

The role of brain magnetic resonance imaging on the timing of antiepileptic drugs withdrawal following mesial temporal lobe epilepsy surgery.

OBJECTIVES: To assess the influence of magnetic resonance imaging (MRI) brain findings on the timing of antiepileptic drugs (AEDs) withdrawal following anterior temporal lobectomy (ATL) in patients with mesial temporal lobe epilepsy (MTLE). METHODS: We conducted a retrospective chart review at King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi Arabia from Jan, 2004 - Dec, 2017 of patients with MTLE who underwent ATL and included patients who had a minimum of 2 years of follow-up. We evaluated the association between the time required to start tapering and discontinuing AEDs after ATL in patients with Engel class I outcomes and their preoperative brain MRI. RESULTS: We studied 64 patients who underwent ATL. The majority of patients (63%) had hippocampal sclerosis (HS) on histopathology. Following ATL, the mean time to start tapering AEDs was 10 months and AEDs were discontinued at a mean of 48 months. Abnormal brain MRI was observed in 53 (83%) patients, with findings suggestive of mesial temporal sclerosis (MTS) accounting for 75% of these abnormalities. The presence of any MRI abnormality was associated with a 10-month earlier tapering of AEDs (p<0.01), and an 18-month earlier complete withdrawal of AEDs (p<0.01). The odds of being seizure-free within the first year were higher if MTS was present in the brain MRI (adjusted OR=16). Similarly, this was associated with seizure freedom after the first year (adjusted OR=14.8, p<0.01). The presence of unilateral temporal IEDs on preoperative EEG were also associated with earlier tapering and discontinuation of AEDs as well as a seizure-free state after ATL surgery (OR=8.5 and 4.2, for the first and second year respectively, p<0.01). CONCLUSION: Patients with abnormal MRI findings and unilateral IEDs had earlier tapering of AEDs with an overall shorter AED discontinuation plan. Moreover, the presence of MTS on MRI, along with unilateral IEDs, were predictors of seizure freedom following ATL.

Effects of fasting during Ramadan on seizure control and quality of life in patients with epilepsy.

BACKGROUND: During Ramadan, the ninth month of the lunar Islamic calendar, adult Muslims are obliged to fast, which involves refraining from taking any food, beverages, or oral medications from dawn to sunset. Fasting's effect on seizure control is not fully understood, and a few observational studies have provided inconclusive results. This study aimed to investigate the effect of fasting during Ramadan on seizure control and quality of life in adult patients with epilepsy. METHODS: This was a prospective observational study over a 3-month period (one month before fasting, the fasting month, and one month after fasting). We recruited adult patients with active epilepsy who were able to fast during the month of Ramadan. The primary outcome measures were as follows: 1) seizure control and 2) quality of life score using the Arabic version of the Ferrans and Powers Quality of Life Index (QLI). Changes in anticonvulsant medications were not allowed during the study period. We used a seizure log provided to participants to record the number of seizures during the 3-month period. Quality of life was scored at the end of each month of the study period. RESULTS: Thirty-seven patients were studied (59% males). The mean age was 30 years (range, 14-51 years), and mean age at epilepsy onset was 13 years (range, 0.5-35 years). On average, patients were on three antiepileptic medications at baseline (range: 2-5). A total of 1576 seizures were reported during the 3-month follow-up, where seizures prior to fasting represented 35.5% of all seizures. Multilinear regression analysis revealed a significant decline of seizures by 21% during the fasting month compared with baseline (adjusted coefficient = 0.79, p < 0.01, 95% confidence interval (CI); 0.61-0.98, R2 = 0.81) and by 29% during post fasting compared with baseline (adjusted coefficient = 0.71, p < 0.01, 95% CI; 0.53-0.90, R2 = 0.79). No significant change was found in the QLI scores calculated during the three months of the study period. CONCLUSION: Fasting during Ramadan might have a positive impact on seizure control in patients with epilepsy, which continued during the month following fasting, whereas the quality of life scores were not affected by fasting.

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

OBJECTIVE: 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.

Atypical Neurologic Phenotype and Novel SLC30A10 Mutation in Two Brothers with Hereditary Hypermanganesemia.

Manganese (Mn) is an essential element in trace quantity but large amounts are toxic. A novel hereditary disorder encompassing high blood Mn levels, dystonia, polycythemia, distinctive T1 hyperintense signals in the basal ganglia on magnetic resonance imaging (MRI) brain, and chronic liver disease was recently described. The disorder is caused by mutations in a Mn transporter encoding gene SLC30A10. We are reporting the clinical features of this rare disorder in two Saudi brothers. The older brother presented with progressive gait difficulties, hypotonia, intermittent dystonia, polycythemia, and characteristic T1-hyperintense lesions on MRI brain. SLC30A10 sequencing identified a novel missense mutation. The younger brother was identified in presymptomatic phase on family screening. Chelation therapy with disodium calcium edetate (ethylenediaminetetraacetic acid, EDTA) led to stabilization of gait, reduction in Mn levels, and resolution of polycythemia. We wish to highlight the atypical neurologic presentation, a novel missense mutation, and beneficial effect of EDTA in this rare disease.

Outcome of intracranial arterial stenting of symptomatic atherosclerotic disease: A single center experience from Saudi Arabia.

OBJECTIVE: To present our local experience with intracranial angioplasty and stenting used for the treatment of symptomatic intracranial stenosis to assess its safety, efficacy, and outcome. METHODS: This is a retrospective review of all the patients with symptomatic intracranial atherosclerotic disease who underwent endovascular treatment in King Faisal Specialist Hospital and research center, Jeddah, Kingdom of Saudi Arabia from January 2003 to December 2014. Clinical, procedural, and outcome variables were gathered. RESULTS: We identified 22 patients who were referred for stenting of symptomatic intracranial atherosclerotic stenosis. In all but 3, the stents were deployed successfully (86% procedural success rate). The procedure was carried out under conscious sedation in 32%. Excellent flow was restored immediately in all successfully-stented cases. Post procedural strokes occurred in 4 patients (17.4%). One non-neurological death was identified in a patient who suffered a major post procedural stroke (4.3%). CONCLUSION: Intracranial atherosclerotic disease is not uncommon in our population. Angioplasty and stenting might be a valid option for the treatment of patients with recurrent symptoms despite optimal medical treatment.

Association between serum vitamin D levels and age in patients with epilepsy: a retrospective study from an epilepsy center in Saudi Arabia.

BACKGROUND: Vitamin D deficiency has been linked to various medical conditions such as bone loss, decreased mineralization, endocrine disorders, and central nervous system disorders, including epilepsy. Vitamin D deficiency is prevalent among patients with epilepsy (PWE). However, the specific association between vitamin D levels and age in PWE is unclear. OBJECTIVES: Identify the relation between vitamin D level and age in PWE and evaluate factors that may play a role in seizure control. DESIGN: Retrospective analytical medical record review SETTING: Outpatient epilepsy research clinic in Saudi Arabia PATIENTS AND METHODS: Between November 2016 and April 2020, we selected eligible PWE aged older than 14 years whose vita-min D levels were recorded at least once after reviewing 1550 patient electronic files. We analyzed data on serum vitamin D level by age and other factors, vitamin D supplement use, seizure classification, and conducted a multivariate logistic regression to assess associations with seizure control. MAIN OUTCOME MEASURES: Relationships between vitamin D levels and age and factors that might affect seizure control. SAMPLE SIZE: 524 patients RESULTS: The prevalence of low serum vitamin D levels was high (86.8%). The median vitamin D level in all patients was low (38 nmol/L), and was lower in young PWE than in adult PWE (P<.01). Only 146 patients received vitamin D supplements. High vitamin D levels were associated with a 40% seizure reduction. CONCLUSION: Vitamin D deficiency is underestimated in PWE in Saudi Arabia, and is more prevalent among young adults and patients on polytherapy than in other PWE. Patients with high vitamin D levels had good seizure control compared with those with low levels. The effect of vitamin D supplements on seizure control should be further investigated in randomized control trials. LIMITATIONS: Retrospective study and no categorization by presence of supplementation. CONFLICT OF INTEREST: None.

Pattern reversal visual evoked potentials (prVEPs) in autosomal recessive hereditary spastic paraplegia with thin corpus callosum (ARHSPTCC) patients with SPG 11 mutations in Saudi Arabia, cross section hospital base study.

OBJECTIVE: To retrospectively report prVEPs in SPG11 ARHSP-TCC. BACKGROUND: ARHSPTCC is characterized by a thin corpus callosum, progressive spastic paraparesis, cognitive decline,and axonal neuropathy by SPG11 mutations. Additionally, seizures, cerebellar ataxia, speech and swallowing problems, extrapyramidal signs, and skeletal deformities may occur. Neuroradiological findings include thinning of the anterior corpus callosum (TCC), periventricular white matter changes, and cortical atrophy. Electromyography and nerve conduction studies may reveal axonal neuropathy or anterior horn involvement. However, optic nerve involvement and prVEPs have not been well described. DESIGN/METHODS: Routine prVEPs were performed in 11 subjects with genetically confirmed (Athena Diagnostic USA) SPG11 ARHSPTCC. Independent stimulation of each eye with a full-field checkerboard pattern reverse stimulation technique was performed. Repetitive waveforms were averaged and the P-100 was recorded. RESULTS: Eleven subjects aged 20 to 37 years were studied, 5 were female. Nine were from consanguineous parents. Nine had a family history and 3 pairs were siblings. Nine had TCC, 8 had diffuse brain atrophy and 1 had cerebellum and brainstem atrophy. Additionally, 9 had bilaterally abnormal prVEPs. The mean P100 latency of the left eye was 129.45 ms±19.47, and a mean amplitude of 7 µV±2.33, while the right had a mean P100 of 127.72 ms±12.69, and mean amplitude of 6.74 µV±2.84. CONCLUSIONS: Abnormal prVEPs occurred in 81.82% of our subjects with significantly prolonged P100 bilateral responses. This indicates that the visual pathway is affected in patients with SPG11 ARHSPTCC. However, no specific mutation was predominant. prVEPs should be considered in the routine evaluation for spastic paraparesis.

The Optimal Dose of Amobarbital in the Wada Test for the Presurgical Evaluation of Patients With Temporal Lobe Epilepsy.

OBJECTIVE: The use of amobarbital in the Wada test varied between epilepsy centers, with no unified dosing or protocols available in the literature to standardize its use. We aimed to determine the dose of amobarbital in the presurgical evaluations of patients with temporal lobe epilepsy. METHODS: A retrospective study of patients with temporal lobe epilepsy seen between January 2004 and December 2018 in King Faisal Specialist Hospital and Research Centre in Jeddah, Saudi Arabia, was conducted, and those who successfully underwent a Wada test were studied. A neuropsychologist or a neurologist will assess the memory and language, using standardized testing. RESULTS: A total of 90 patients were studied. The mean age was 30 years (range, 16-52 years), where 49 (57%) of them were men. All patients had a routine neurological examination, including language and memory. The average dose of amobarbital given was 10.1.1 mg (range, 65.7-150 mg). There was no statistical difference between the dosing given to patients who passed or failed the memory testing (101.4 mg vs 94.7 mg, P = 0.1). Multivariate regression analysis showed that amobarbital dose needed an adjustment to patient's weight only for those older than 30 years, (P < 0.05; 95% confidence interval, 0.1-0.5), where an increase in the dose by 0.3 mg·kg·y was required to execute Wada test successfully. CONCLUSION: It was only the patient's age that could influence the modification of Amobarbital dose in the Wada test, yet establishing a universal protocol is challenging because of the lack of well-defined dose determinants.

Endolymphatic sac tumor at the cerebellopontine angle: A case report and review of literature.

INTRODUCTION: Endolymphatic sac tumors may present as sporadic or may be associated with Von Hippel-Lindau disease. Patients generally present with hearing loss, tinnitus and vertigo. The tumor is highly vascular which may lead to erosion of the adjacent bony and vascular structures, resulting in heavy bleeding during surgery. PRESENTATION: A twenty-five year-old female presented with a five year history of chronic ear discharge, left sided facial weakness, and recent onset of ataxia. DISCUSSION: The unusual clinical presentation made management challenging, in large part due to profuse bleeding. Pre-operative embolization of the vessels supplying the tumor may reduce blood loss during surgical excision. Radiotherapy could be considered for any residual tumor. CONCLUSION: The patient was diagnosed with an endolymphatic sac tumor of sporadic origin which presented at the cerebellopontine angle and was managed with a multidisciplinary approach.

Prolonged hemiplegic migraine.

Hemiplegic migraine is a rare form of migraine variant. It is characterized by recurrent attacks of headache associated with temporary neurological deficit, usually unilateral hemiparesis or hemiplegia. It can be difficult to distinguish from migrainous stroke clinically, and a full neurological work-up and careful review of medical history and symptoms are necessary for the diagnosis. Two forms of hemiplegic migraine are known: familial and sporadic, phenotypically similar, differentiated by the absence of family history of similar attacks in the sporadic form. We report a case of sporadic hemiplegic migraine with unusually prolonged deficit and progression to quadriplegia with complete return to baseline 7 days after onset. Diffusion weighted images helped in excluding infarction.

Non-convulsive seizures and electroencephalography findings as predictors of clinical outcomes at a tertiary intensive care unit in Saudi Arabia.

OBJECTIVE: Electroencephalography (EEG) in the intensive care unit (ICU) is often done to detect non-convulsive seizures (NCS). The outcome of ICU patients with NCS strongly depends on the underlying etiology. The implication of NCS and other EEG findings on clinical outcome independent from their etiology is not well understood and our aim to investigate it. PATIENTS AND METHODS: We retrospectively identified all adult patients in the ICU who underwent EEG monitoring between January 2008 and December 2011. The main goals were to define the rate of NCS or non-convulsive status epilepticus (NCSE) occurrence in our center among patients who underwent EEG monitoring and to examine if NCS/NCSE are associated with poor outcome [defined as death or dependence] with and without adjustment for underlying etiology. The rate of poor outcome among different EEG categories were also investigated. RESULTS: During the study period, 177 patients underwent EEG monitoring in our ICU. The overall outcome was poor in 62.7% of those undergoing EEG. The rate of occurrence of NCS/NCSE was 8.5% and was associated with poor outcome in 86.7% with an odds ratio (OR) of 5.1 (95% confidence interval [CI] 1.09-23.8). This association was maintained after adjusting for underlying etiologies with OR 5.6 (95% CI 1.05-29.6). The rate of poor outcome was high in the presence of periodic discharges and sharp and slow waves of 75% and 61.5%, respectively. CONCLUSIONS: Our cohort of ICU patients undergoing EEGs had a poor outcome. Those who developed NCS/NCSE experienced an even worse outcome regardless of the underlying etiology.

Improving Reports Turnaround Time: An Essential Healthcare Quality Dimension.

Turnaround time is one of the most important healthcare performance indicators. King Faisal Specialist Hospital and Research Center in Jeddah, Saudi Arabia worked on reducing the reports turnaround time of the neurophysiology lab from more than two weeks to only five working days for 90% of cases. The main quality improvement methodology used was the FOCUS PDCA. Using root cause analysis, Pareto analysis and qualitative survey methods, the main factors contributing to the delay of turnaround time and the suggested improvement strategies were identified and implemented, through restructuring transcriptionists daily tasks, rescheduling physicians time and alerting for new reports, engaging consultants, consistent coordination and prioritizing critical reports. After implementation; 92% of reports are verified within 5 days compared to only 6% before implementation. 7% of reports were verified in 5 days to 2 weeks and only 1% of reports needed more than 2 weeks compared to 76% before implementation.

Vagus nerve stimulation for refractory epilepsy: experience from Saudi Arabia.

BACKGROUND: Vagus nerve stimulation (VNS) has been approved for the treatment of refractory partial epilepsy in adults and children over 12 years of age. Later on, its application expanded to include younger children and other types of epilepsy. We report our experience with this treatment modality for refractory epilepsy in Saudi Arabia. DESIGN AND SETTINGS: Open-label, uncontrolled, retrospective study of patients with refractory epilepsy, who were treated with VNS in a tertiary care hospital from January 2010 to June 2013. PATIENTS AND METHODS: Collected data included 26 patients' demographics, epilepsy characteristics, seizure frequency, and treatment history. Patients with a follow-up duration of minimum 12 months were included in the analysis. The examined outcome measures were seizure reduction rates, antiepileptic drugs (AEDs) burden, and impact on patients' quality of life (QOL). RESULTS: Onset of seizures was from birth to 30 years. Patients' ages at VNS implantation ranged from 4 to 38 years (18.9 [8.7] years). Epilepsy was classified as focal in 8 patients (30%), multifocal in 9 patients (35%), and generalized in 9 patients (35%). The average number of AEDs failed before VNS was 4.2 (1.4). Greater than 50% seizure reduction was achieved in 50% of patients at 3 months, 67% at 6 months, 73% at 12 months, and 78% at 24 months. There was no significant reduction in AEDs burden during the same period. Subjective QOL improvement was reported by 72% of patients at 3 months, 83% at 6 months, 78% at 12 months, and 73% at 24 months after VNS. Minor adverse effects were reported in 27% of patients. One patient had the device replaced due to malfunction. CONCLUSION: The experience with VNS in a single center in Saudi Arabia confirms that it is a safe and effective adjunctive therapy for refractory epilepsy in adult and pediatric patients.

Cerebellar swelling due to familial hemophagocytic lymphohistiocytosis: An unusual presentation.

BACKGROUND: Cerebellar swelling with obstructive hydrocephalus is a rare but life threatening condition, associated with different etiologies, familial hemophagocytic lymphohistiocytosis (HLH) being rarely one of them. PATIENT: 2-year-7-month old boy presented with irritability, cerebellar dysfunction, and somnolence. Brain MRI showed marked diffuse cerebellar swelling and obstructive hydrocephalus with mild tonsillar herniation. Laboratory testing revealed pancytopenia, elevated liver enzymes, elevated ferritin and triglycerides levels and decreased fibrinogen. The diagnosis of familial HLH was confirmed by the presence of homozygous missense mutation of Syntaxin 11 gene. The child was treated with HLH-2004 protocol of chemotherapy followed by allogenic stem cell transplantation. His neurological condition improved significantly after treating the underlying disease. CONCLUSION: Cerebellar swelling is a rare manifestation of familial HLH. High degree of clinical suspicion may allow a timely diagnosis and appropriate therapy.

Emergency excision of cardiac myxoma and endovascular coiling of intracranial aneurysm after cerebral infarction.

Cardiac myxoma is the most common primary tumor of the heart, located mainly in the left atrium. Cerebral embolization or intracranial aneurysm formation as a consequence of left atrial myxomas has been well documented, whereas myxoma embolization causing the combination of cerebral infarction and intracranial myxomatous aneurysm is rare. We report herein, a 67-year-old female with a cardiac myxoma who experienced a left hemispheric embolic ischemic stroke and in addition was found to have right internal carotid artery aneurysm. The patient underwent emergency surgical excision of left atrial myxoma 2 hours after the stroke onset and endovascular coiling of the aneurysm a week later. Although the timing of cardiac surgery is controversial in patients who have had recent ischemic stroke, we recommend immediate resection of cardiac myxoma, if feasible, and early endovascular treatment of associated intracranial myxomatous aneurysms.