Clinical, radiological and electrophysiological predictors for drug-resistant epilepsy.

Background: Epilepsy is the third chronic brain illness worldwide. About a third of the epileptic patients will be drug resistant. Early identification of these patients is critical for appropriate treatment selection and prevention of the devastating consequences of recurrent seizures. The objective of this study aims to detect clinical, electrophysiological, and radiological predictors for drug-resistant epilepsy patients. Results: One hundred fifty-five patients were included in this study, divided into a well-controlled epilepsy group (103 patients) and a drug-resistant group (52 patients). Both groups were compared regarding clinical, electrophysiological, and neuro-radiological data. Younger age at onset, history of delayed milestones, history of perinatal insult (especially hypoxia), mental retardation, neurological deficits, depression, status epilepticus (SE), complex febrile seizures, focal seizure to bilateral tonic-clonic convulsion as well as multiple seizures and high seizure frequency (daily) at onset, poor response to first anti-seizure drug (ASD), structural and metabolic etiology, abnormal brain imaging, and slow background and multifocal epileptiform discharges in EEG were significant risk factors for the development of drug-resistant epilepsy. Conclusion: MRI abnormalities are the most significant predictor for drug-resistant epilepsy. Drug-resistant epilepsy is associated with clinical, electrophysiological, and radiological risk factors that can be used to diagnose drug-resistant patients early and choose the best treatment option and time.

Fasting and post fasting effect of Ramadan on different seizure types in patients with active epilepsy.

BACKGROUND AND OBJECTIVES: Fasting is the basis for the ketogenic diet, and intermittent fasting is emerging as a treatment for epilepsy. There are no available data about the role of Islamic fasting on seizure control. This study aims to assess the effect of Ramadan fasting on the frequency of different seizure types. METHODS: This was a prospective observational study on Muslim patients with active epilepsy intending to fast during Ramadan in the year 2019, with an average of 16 fasting hours per day. Seizure frequency for each seizure type was followed over three months, one month before (Shaaban), during Ramadan and one month after (Shawwal), after ensuring drug compliance. RESULTS: Three hundred and twenty one Muslim patients with active epilepsy with median age of 33 years were included (some patients had more than one type of seizure). In Ramadan, 86 out of 224 patients with focal seizures, 17 out of 38 patients with myoclonic seizures and 6 out of 10 patients with absence seizures showed ≥ 50% reduction. In Shawaal, such improvement continued to include 83, 13 and 4 patients with focal, myoclonic and absence seizures. Focal and myoclonic seizures were significantly improved in the months of Ramadan and Shawaal compared to Shaaban. However, absence seizures were significantly improved only in Ramadan compared with Shaaban. The frequency of generalized tonic-clonic seizures did not significantly differ between the three months. DISCUSSION: Ramadan fasting may have an improving effect, as well as a post-fasting effect, on active focal, myoclonic and absence seizures.

The aquaporin4-IgG status and how it affects the clinical features and treatment response in NMOSD patients in Egypt.

BACKGROUND: In Egypt, the characterization of Neuromyelitis Optica Spectrum Disorder (NMOSD) is lacking. OBJECTIVES: To determine the demographics, clinical features, aquaporin4 antibodies (AQP4-IgG) status, and neuroimaging of Egyptian NMOSD patients. METHODS: Retrospective analysis of 70 NMOSD patients' records from the MS clinic, Kasr Alainy hospital, between January 2013 and June 2018. RESULTS: Patients' mean age was 34.9 ± 9.2 years, and the mean at disease onset was 28.9 ± 10.5 years. Fifty-nine patients had an initial monosymptomatic presentation. AQP4-IgG was measured using either enzyme-linked immunosorbent assay (ELISA) (22 patients) or cell-based assay (CBA) (34 patients). Six and 29 patients had positive results, respectively (p < 0.001). 84% had typical NMOSD brain lesions. Longitudinally extensive myelitis was detected in 49 patients, and 9 had either short segments or normal cords. Treatment failure was higher in seropositive patients. Rituximab significantly reduced the annualized relapse rate (ARR) compared to Azathioprine with a percentage reduction of (76.47 ± 13.28) and (10.21 ± 96.07), respectively (p = 0.04). Age at disease onset was the only independent predictor for disability (p < 0.01). CONCLUSION: Treatment failure was higher in seropositive patients. However, there was no difference in clinical or radiological parameters between seropositive and seronegative patients. Patients, who are polysymptomatic or with older age of onset, are predicted to have higher future disability regardless of the AQP4-IgG status.

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.

Managing Disease-Modifying Therapies and Breakthrough Activity in Multiple Sclerosis Patients During the COVID-19 Pandemic: Toward an Optimized Approach.

The emergence of the novel coronavirus disease 2019 (COVID-19) pandemic has become a major public health challenge of global concern since December 2019, when the virus was recognized in Wuhan, the capital city of Hubei province in China and epicenter of the COVID-19 epidemic. Given the novelty of COVID-19 and the lack of specific anti-virus therapies, the current management is essentially supportive. There is an absence of consensus on guidelines or treatment strategies for complex disorders such as multiple sclerosis (MS), in which the risk of infections is higher than in the general population. This is due to the overall impairment of the immune system typical of autoimmune diseases, in addition to accumulation of disabilities, and the iatrogenic effect generated by corticosteroids and the recommended disease-modifying therapies (DMTs). DMTs have different modes of action, but all modulate and interfere with the patient's immune response, thereby raising concerns about adverse effects, such as an increased susceptibility to infections. In this review, we analyze the evidence for use of DMTs during the current critical period and ratify an algorithmic approach for management to optimize care between keeping DMTs, with their infection hazards, or coming off them, with the risk of disease activation. We also provide an algorithmic approach to the management of breakthrough activity during the COVID-19 pandemic.

Predictors of successful Ramadan fasting in Muslim patients with epilepsy: A prospective study.

PURPOSE: Ramadan fasting represents a challenge for both Muslim patients with epilepsy (MPWE) as well as their treating neurologists who aim to minimize the risk of fasting-related seizures. Several factors may contribute to the risk of fasting-related seizures such as the half-life of antiepileptic drugs (AEDs), seizure control before Ramadan, and sleep fragmentation. The aim of this work was to investigate these factors. METHODS: An observational prospective study included all MPWE who completed Ramadan fasting in 2019, about 16 h per day for 30 days. They were assessed regarding seizure control, AEDs, and sleep alterations using The Pittsburgh Sleep Quality Index. RESULTS: The study included 430 MPWE. The majority of patients (75.58%) completed Ramadan fasting without breakthrough seizures. Patients achieved successful Ramadan fasting were significantly younger, had shorter disease duration, longer periods of seizure freedom before Ramadan, more efficient and longer sleep hours. There was no significant difference between patients receiving monotherapy regimens with short versus intermediate long t½. Maximum seizure freedom before Ramadan and sleep hours were identified as independent predictors of successful Ramadan fasting, using multivariate analysis. Every extra week of being seizure free before Ramadan and every extra hour of sleep was associated with an increase in the probability of successful Ramadan fasting by 10% and 30%, respectively. CONCLUSION: Neurologists should guide their MPWE who wish to fast Ramadan about the risks and precautions. Proper seizure control and ensuring adequate sleep duration can increase the probability of a successful Ramadan fasting.

Childhood onset of acquired neuromyotonia: association with vitamin D deficiency.

Purpose/Aim: Acquired neuromyotonia or Isaacs syndrome is a type of peripheral nerve hyperexcitability of autoimmune origin. It may occur as an isolated, paraneoplastic or accompanied with some autoimmune diseases. This report describes acquired neuromyotonia in a child with a new reported association with vitamin D deficiency. Case report: A 9-year-old child, in whom the diagnosis of acquired neuromyotonia was made by clinical and typical electromyographic findings. All paraneoplastic and autoimmune workup was normal, except for a vitamin D deficiency state. A dramatic improvement was recorded on both clinical and electrophysiological base after vitamin D replacement. Conclusion: An in-depth future analysis of vitamin D status in patients with neuromyotonia will help to establish whether the association of neuromyotonia with vitamin D deficiency is casual or whether these two conditions may be causally related.

Evaluating sex hormones and cytokine profile in Egyptian females with relapsing-remitting multiple sclerosis.

BACKGROUND: Sexual dimorphism shown in multiple sclerosis suggests an interaction between immune system and sex hormones. The objective of this study is to determine the hormonal profile and serum cytokine levels in Egyptian female patients with relapsing-remitting MS (RRMS) compared with healthy controls and their associations with disease disability. METHODS: This study was conducted on 40 female patients with RRMS and 20 age-matched controls subjected to measurements of the hormonal profile (estrogen, testosterone) and cytokine levels (interleukin 10 and 4 and tumor necrosis factor alpha) and disability assessment using Expanded Disability Status Scale (EDSS). RESULTS: Levels of estrogen, testosterone, interleukin 10 and 4 (IL-10 and IL-4), and tumor necrosis factor alpha (TNF-α) were higher in patients compared to control with no statistically significant difference. Estrogen levels were positively correlated with interleukin 10 and interleukin 4 levels and negatively correlated with tumor necrosis factor alpha (TNF-α), but there was no statistically significant correlation between hormonal profile or cytokine profile (IL-10, IL-4, and TNF-α) and EDSS. CONCLUSIONS: It is suggested that estrogen has an anti-inflammatory effect on cytokine milieu; therefore, it can be tried as a treatment option in multiple sclerosis.

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.

Pediatric-onset multiple sclerosis in Egypt: a multi-center registry of 186 patients.

INTRODUCTION: Although the frequency of pediatric-onset multiple sclerosis (POMS) has increased in recent decades, it is still highly uncommon, which creates a need for the involvement of more registries from various clinical centers. OBJECTIVE: To characterize the demographic, clinical, and paraclinical features of Egyptian patients with POMS. PATIENTS AND METHODS: A retrospective chart review study was undertaken on 237 Egyptian patients with demyelinating events which started before the age of 18 years who attended one of five tertiary referral centers in Cairo, Egypt. RESULTS: Multiple sclerosis was diagnosed in 186 patients, 47 (25.27%) patients had disease onset before the age of 12 years; "early-onset pediatric multiple sclerosis (EOPMS)". The mean age of disease onset was (14.13±2.49 years), with a female:male ratio of 1.62:1, none of the enrolled patients had a primary progressive course (PPMS), whereas 10 patients (5.38%) had a secondary progressive form. Approximately two-thirds of the patients had monofocal disease onset, and less than 10% presented with encephalopathy; most of them had EOPMS. Motor weakness was the presenting symptom in half of the patients, whereas cerebellar presentation was detected in 34.95%, mainly in EOPMS. Seizures (not related to encephalopathy) were more frequent in those with EOPMS. Initial brain magnetic resonance images were positive in all patients, with detected atypical lesions in 29.03%, enhanced lesions in 35.48%, black holes in 13.98%, and infratentorial in 34.41%. Cervical cord involvement was found in 68.28%. More than two-thirds of the patients received either immunomodulatory or immunosuppressant (IS) treatment throughout their disease course, and about half of them received their treatment within the first year from symptoms onset, with a more favorable outcome, and patients with highly active disease received natalizumab, fingolimod, or other IS. CONCLUSION: The results from this registry - the largest for MS in the Arab region to date - are comparable to other registries. Immunomodulatory therapies in POMS are well tolerated and efficacious and they can improve the long-term outcome in children.

Ultrasound evaluation of internal jugular valve incompetence (IJVI) in Egyptian patients with idiopathic intracranial hypertension (IIH).

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a clinical syndrome with no identified causative factor. Internal jugular valve incompetence (IJVI) has been linked to many neurological disorders such as idiopathic intracranial hypertension (IIH), transient global amnesia and cough-induced headache. Intact valves prevent efficiently retrograde flow into the internal jugular vein. AIM: The aim of this study is to evaluate the competence of the jugular vein valves and its relationship to age, BMI, opening CSF pressure and MRV findings in IIH patients. SUBJECTS AND METHODS: Twenty-five Egyptian female patients diagnosed with IIH according to the modified Dandy criteria, and 24 female controls, matched for age and BMI, were included and examined using color-coded duplex for IJVI during the Valsalva maneuver. The patients underwent lumbar puncture to measure the opening pressure, MRV, ophthalmic examination and laboratory work-up. RESULTS: There was no statistically significant difference in the proportion of IJVI among the patients and controls (P=0.7). There was a statistically significant increase in the opening pressure and proportion of MRV abnormalities in the patients with IJVI compared to the patients without IJVI (P=0.03 and 0.007, respectively), but there were no statistically significant difference with regard to age, BMI, grade of papilledema and perimetry findings. CONCLUSION: This study showed that there is no relationship between IJVI and IIH; thus, IJVI would be a rather normal finding. Further studies are recommended to confirm or rule out a possible relationship.

Diagnostic value of aquaporin 4 antibody in assessing idiopathic inflammatory demyelinating central nervous system diseases in Egyptian patients.

Neuromyelitis optica immunoglobulin G (NMO-IgG) binds selectively to aquaporin 4 (AQP4). We aimed to evaluate the frequency of AQP4 antibody in Egyptian patients. We retrospectively evaluated 39 consecutive Egyptian patients with suspected idiopathic inflammatory demyelinating central nervous system disease (IIDCD) who visited the multiple sclerosis clinic at Kaser Al-Aini Hospital. The patients were diagnosed with NMO, other NMO spectrum disorders, or multiple sclerosis using the respective current diagnostic criteria. For the anti-AQP4 antibody assays, serum samples from all patients and 16 healthy matched controls were evaluated. The coded sera were tested for AQP4 antibody using an enzyme-linked immunosorbent assay kit. The relations between the clinical diagnosis and the AQP4 antibody serologic status were studied. Among the 39 patients, 21 (53.85%) were AQP4 antibody-positive. NMO spectrum disorders patients had a significantly higher level of AQP4 antibody compared with MS patients and controls (p<0.001). Only eight patients (36.36%) met the Wingerchuk 2006 criteria for NMO diagnosis excluding AQP4 antibody-seropositive status. AQP4 antibody was highly prevalent (almost 54%) in Egyptian IIDCD patients. Our research revealed that we must maintain a high index of suspicion for NMO spectrum disorders.

Possible induction of acute disseminated encephalomyelitis (ADEM)-like demyelinating illness by intrathecal mesenchymal stem cell injection.

We report a 27-year-old woman with an episode of encephalitis and optic neuritis, followed by autologous bone marrow mesenchymal stem cell transplants and possible induction of acute disseminated encephalomyelitis-like demyelinating illness.

Vitamin D deficiency in women with fibromyalgia in Saudi Arabia.

BACKGROUND: The relation between low levels of 25-hydroxyvitamin D and nonspecific musculoskeletal pain, including fibromyalgia syndrome, is debatable. Many studies have reported "a positive relation" and others "found no relation." OBJECTIVES: To determine the prevalence of vitamin D deficiency among patients with fibromyalgia in a neurology clinic in the Kingdom of Saudi Arabia (KSA). METHODS: This study was done at a neurology clinic of Bugshan Hospital, Jeddah, KSA, from January to April 2011. Thirty female patients were diagnosed with fibromyalgia according to new clinical fibromyalgia diagnostic criteria; their serum vitamin D levels were screened. Vitamin D deficiency is defined as <20 ng/mL, vitamin D insufficiency is defined as 21-29 ng/mL, and vitamin D sufficiency is equal to or >30 ng/mL. RESULT: Thirty female patients were included in the study. The mean age was 34.56 ± 8.1 years. Mean vitamin D level was 4.76 ± 1.46 ng/mL. A significant negative correlation between vitamin D level and widespread pain index was found. Thirty percent of the patients were Saudi Arabian of whom 100% were veiled; 70% were non-Saudi Arabian of whom 47.6% were veiled and 52.4% wore long pants and/or full sleeved clothes. Vitamin D deficiency was equally prevalent among veiled (4.77 ± 1.37 ng/mL) and nonveiled (4.75 ± 1.68 ng/mL). Treatment with high-dose vitamin D resulted in clinical improvement in all patients. CONCLUSION: Vitamin D deficiency is often seen in patients diagnosed with fibromyalgia in our population. This was equally true in veiled and nonveiled, but conservatively dressed populations. Effective treatment with high-dose vitamin D could lead to resolution of almost all symptoms. Further study of these populations and fortification of foods with vitamin D may be essential.