Epidemiology of multiple sclerosis in the Sultanate of Oman: A hospital based study.

BACKGROUND: The prevalence of multiple sclerosis (MS) has been changing not only globally but also in individual countries. We aim to estimate the prevalence of MS in the Omani population over the period from 2006-2019 as well as the incidence between 2015-2018. METHODS: This is a retrospective observational hospital-based study. All MS patients diagnosed, as per the revised McDonald criteria, over the period from June 2006 and until May 2019, had their information reviewed for age at disease onset, gender and year of diagnosis. We obtained the population of Oman from the national census data. RESULTS: A total of 422 patients were diagnosed with MS during the study period and the population of Oman as per the 2019 census data was 2,652,199. The estimated crude prevalence was 15.9 (95% confidence interval: 14.4 - 17.5) per 100,000 and the female to male ratio was 2.17:1. The mean age at disease onset was 27.3 ± 7.7 (range: 9 - 59) years in which 83% of the patients had the first clinical manifestation at the age of 19 - 40 years, while only 9% had a disease onset at <19 years. The annual incidence increased from 1.00 case per 100,000 in 2015 to 1.38 cases per 100,000 in 2018. CONCLUSION: The prevalence of MS in the Omani population is 15.9 per 100,000 placing Oman as a medium risk zone.

Clinico-electrographic characteristics and classification of genetic generalized epilepsy in Oman.

PURPOSE: There is a lack of information on the annual incidence of genetic generalized epilepsy (GGE) in the Arab countries, especially Oman. Ascertaining the true burden of illness has crucial implications for health policies and priorities. We aim to study the clinico-electrographic characteristics, classification, and annual incidence of GGE in Oman. METHOD: Using the cross-sectional data of EEGs obtained from all patients with GGE who presented to Sultan Qaboos University hospital (major referral center for epilepsy in Oman) from January 2007 to June 2014. Analyses were performed using univariate statistics. RESULTS: Approximately 10,423 patients had EEG studies during the study period of which 376 patients (3.6 %) had EEG abnormalities suggestive of GGE. Forty two percent of the 376 GGE patients were male with ages ranging from 3 to 58 years. We were able to classify 273 patients to one of the GGE syndromes. Forty-three percent of 130 patients had a positive family history of epilepsy in their first or second-degree relatives. The generalized tonic-clonic seizure was the most common seizure type observed in 242 patients (64 %; 95 %CI: 59.2 %-68.9 %). Juvenile myoclonic epilepsy was the most common epilepsy syndrome (41 % of the total GGE patients) encountered in our region. A significant female predominance (9.7 % vs 2.5 %; p = 0.016) was observed in juvenile absence epilepsy. Certain interictal focal EEG abnormalities did not exclude a diagnosis of GGE. An average annual GGE incidence of 2.9 % (95 % CI: 2.6 %-3.2 %) was observed during the study period. CONCLUSION: This hospital-based study is the first of its kind in the Arabian Gulf region, classifying the different subcategories of GGE. Our results indicate that GGE is a common epilepsy subtype in Oman. A prospective population-based epidemiological study is required to estimate the precise frequency of GGE in Oman.

Neuromyelitis optica spectrum disorders in Arabian Gulf (NMOAG); establishment and initial characterization of a patient registry.

OBJECTIVE: To describe the clinical and radiological characteristics of neuromyelitis optica spectrum disorders (NMOSD) patients from the Arabian Gulf relative to anti-aquaporin 4 antibody serostatus. METHODS: Retrospective multicentre study of hospital records of patients diagnosed with NMOSD based on 2015 International Panel on NMOSD Diagnosis (IPND) consensus criteria. RESULTS: One hundred forty four patients were evaluated, 64.3% were anti-AQP4 antibody positive. Mean age at onset and disease duration were 31±12 and 7 ±â€¯6 years respectively. Patients were predominantly female (4.7:1). Overall; relapsing course (80%) was more common than monophasic (20%). Optic neuritis was the most frequent presentation (48.6%), regardless of serostatus. The proportion of patients (54.3%) with visual acuity of ≤ 0.1 was higher in the seropositive group (p = 0.018). Primary presenting symptoms of transverse myelitis (TM) were observed in 29% of patients, and were the most significant correlate of hospitalization (p<0.001). Relative to anti-APQ4 serostatus, there were no significant differences in terms of age of onset, course, relapse rates or efficacy outcomes except for oligoclonal bands (OCB), which were more often present in seronegative patients (40% vs.22.5%; p = 0.054). Irrespective of serostatus, several disease modifying therapies were instituted including steroids or immunosuppressives, mostly, rituximab and azathioprine in the cohort irrespective of serostatus. The use of rituximab resulted in reduction in disease activity. CONCLUSION: This is the first descriptive NMOSD cohort in the Arabian Gulf region. Seropositive patients were more prevalent with female predominance. Relapsing course was more common than monophasic. However, anti-AQP4 serostatus did not impact disease duration, relapse rate or therapeutic effectiveness. These findings offer new insights into natural history of NMOSD in patients of the Arabian Gulf and allow comparison with patient populations in different World regions.

Spinocerebellar ataxia with axonal neuropathy type 1 revisited.

Spinocerebellar ataxia with axonal neuropathy type 1 (SCAN1; OMIM #607250), an exceedingly rare disorder having been documented in only a single family from Saudi Arabia, is the result of an unusual mutation in the tyrosyl DNA phosphodiesterase 1 gene (TDP1). We performed high-throughput sequencing (whole exome and ataxia gene panel) in two apparently unrelated Omani families segregating sensorimotor neuropathy and ataxia in an autosomal recessive fashion. Following validation by Sanger sequencing, all affected subjects (n = 4) were confirmed to carry the known SCAN1 pathogenic homozygous variant in the TDP1 gene, NM_001008744.1:c.1478A > G (p.His493Arg). In keeping with the initial description, our patients demonstrated progressive ataxia, cerebellar atrophy and disabling axonal sensori-motor neuropathy (n = 4), hypercholesterolemia (n = 2) and elevated serum alpha fetoprotein (n = 3). In addition, our patients also had mild cognitive deficits in multiple domains (n = 3), a feature not previously reported. Our findings independently revalidate the phenotype of TDP1 mutation and expand the clinical spectrum to include mild cognitive deficits. Haplotype sharing, as determined by DNA microarray (CytoScan HD), attests to a possible common founder mutation in the Arab population.

Encephalopathy mimicking non-convulsive status Epilepticus.

Primary hyperammonemic encephalopathy due to urea cycle disorders (UCD) typically manifests with episodic unresponsiveness and this clinical entity is not often included in the differential diagnosis of presumed non-convulsive status epilepticus (NCSE). However, this diagnostic consideration has therapeutic implications. In this report, we document the therapeutic importance of elucidating the specific cause of hyperammonemic encephalopathy that closely mimicked NCSE through 2 unique illustrative cases.

Prevalence of Sleep-Disordered Breathing During Routine Electroencephalogram (EEG): A Hospital-Based Descriptive Study.

Sleep-disordered breathing (SDB) in adults is a common condition that is associated with a range of medical problems including hypertension, cardiovascular complications, and increase of seizure frequency in susceptible individuals. Polysomnography (PSG) is considered the gold standard measure in the diagnosis of SDB. This is an observational study on the frequency of SDB in adult patients referred for routine EEG. We found that routine EEG was capable of detecting moderate to severe symptoms of SDB in 14% of adult patients (95% confidence interval = 8.1-19.9%). The state of sleep during a routine EEG recording could help in assessing a SDB pattern and could provide an opportunity for further diagnostic sleep consultation if the patient has not previously reported problems with sleep or if SDB was not considered by the referring physician. This study underscores the need for a practice approach to ensure that patients suffering from SDB are properly referred to a sleep specialist. In the context of this report, some training and experience in PSG can be an added advantage for EEG technologists in the detection of SDB.

Intravenous levetiracetam vs phenytoin for status epilepticus and cluster seizures: A prospective, randomized study.

PURPOSE: Status Epilepticus (SE) is a common medical emergency carrying a high morbidity and mortality. Levetiracetam (LEV) is a novel anticonvulsant effective against varied seizures. Few prospective studies have addressed its use in SE. We aimed to examine the efficacy of intravenous LEV in controlling SE and cluster attacks of seizures (CS), in comparison with IV phenytoin (DPH), using a prospective, randomized study design. METHOD: Adult patients with SE or CS, following an initial dose of IV benzodiazepine to control ongoing seizure, were randomized to receive either medication. Rates of seizure control over 24h, adverse effects and outcomes were compared. A logistic regression model was used to identify outcome predictors. RESULTS: 52 patients with SE and 63 with CS received either LEV or DPH. In the SE group, LEV was effective in18/22(82%) and DPH in 22/30(73.3%) patients in controlling seizures. Among patients with CS, LEV was effective in 31/38(81.6%) and DPH in 20/25(80%). With the use of LEV, DPH or both, SE and CS were controlled among 92% and 96% of patients respectively. Adverse events included hypotension (in 2 on DPH) and transient agitation (2 on LEV). CONCLUSIONS: IV Levetiracetam controls status epilepticus or cluster seizures with an efficacy comparable to that of phenytoin. Use of these two agents consecutively may control >90% of all such conditions without resort to anaesthetic agents. Further studies should explore its efficacy in larger cohorts of epileptic emergencies.

Idiopathic intracranial hypertension presenting as postpartum headache.

Postpartum headache is described as headache and neck or shoulder pain during the first 6 weeks after delivery. Common causes of headache in the puerperium are migraine headache and tension headache; other causes include pre-eclampsia/eclampsia, post-dural puncture headache, cortical vein thrombosis, subarachnoid hemorrhage, posterior reversible leukoencephalopathy syndrome, brain tumor, cerebral ischemia, meningitis, and so forth. Idiopathic intracranial hypertension (IIH) is a rare cause of postpartum headache. It is usually associated with papilledema, headache, and elevated intracranial pressure without any focal neurologic abnormality in an otherwise healthy person. It is more commonly seen in obese women of reproductive age group, but rare during pregnancy and postpartum. We present a case of IIH who presented to us 18 days after cesarean section with severe headache and was successfully managed.

Predictive Value of Short-Term EEG Recording in Critically ill Adult Patients.

We assessed the EEG patterns and their prognostic significance in critically ill adult patients with encephalopathy, by digital EEGs lasting lip to 1 hour Of the 110 patients (age: 43.8 ± 19.4 years, male: female:1.6:1) studied, 32% had hypoxic ischemic encephalopathy (HIE), 17% severe infections, and 14.5% stroke. Observed EEG patterns were diffuse slowing (41%), low-voltage cerebral activity (LVCA, 18%), nonconvulsive status epilepticus (NCSE, 13.6%), and periodic abnormalities (9.1%). LVCA, age, Glasgow Coma Score (GCS) < 8, HIE, and modified Hockaday's EEG grades of IV and V were associated with poor outcome (p < 0.005) at hospital discharge; generalized slowing was associated with a relatively good outcome (p = 0.003). On multivariate analysis, factors independently predictive of mortality were LVCA, older age, and poor GCS. In conclusion, LVCA and generalized background slowing were common EEG patterns among critically ill intensive care unit (ICU) patients with encephalopathy of varied etiologies. While LVCA was associated with a poor outcome, generalized background slowing predicted better prognosis. Conventional short-duration, bedside EEG studies could aid in the recognition of electrographic patterns of prognostic importance in facilities where continuous EEG monitoring is lacking.

Cerebral angiography. Unusual connection and disconnection.

A 61-year-old woman underwent MRI scan of brain (Figure 1A), followed by conventional angiography (Figures 1B & 1C) and magnetic resonance cerebral angiography (Figure 1D, conventional angiography was performed for more specific details regarding the findings of the initial MR cerebral angiography) as part of the ischemic stroke work-up for recurrent episodes of right hemiparesis and expressive aphasia.

Varicella zoster virus pneumonitis and brainstem encephalitis without skin rash in an immunocompetent adult.

Varicella zoster virus (VZV) pneumonitis and brainstem encephalitis developed in an immunocompetent adult without rash. Chest computed tomography exhibited nodularity; lung biopsy revealed multinucleated giant cells, Cowdry A inclusions, VZV antigen, and DNA. Varicella zoster virus central nervous system disease was verified by cerebrospinal fluid (CSF) anti-VZV IgG antibody with reduced serum/CSF ratios.

The nature of progression in Parkinson's disease: an application of non-linear, multivariate, longitudinal random effects modelling.

BACKGROUND: To date, statistical methods that take into account fully the non-linear, longitudinal and multivariate aspects of clinical data have not been applied to the study of progression in Parkinson's disease (PD). In this paper, we demonstrate the usefulness of such methodology for studying the temporal and spatial aspects of the progression of PD. Extending this methodology further, we also explore the presymptomatic course of this disease. METHODS: Longitudinal Positron Emission Tomography (PET) measurements were collected on 78 PD patients, from 4 subregions on each side of the brain, using 3 different radiotracers. Non-linear, multivariate, longitudinal random effects modelling was applied to analyze and interpret these data. RESULTS: The data showed a non-linear decline in PET measurements, which we modelled successfully by an exponential function depending on two patient-related covariates duration since symptom onset and age at symptom onset. We found that the degree of damage was significantly greater in the posterior putamen than in the anterior putamen throughout the disease. We also found that over the course of the illness, the difference between the less affected and more affected sides of the brain decreased in the anterior putamen. Younger patients had significantly poorer measurements than older patients at the time of symptom onset suggesting more effective compensatory mechanisms delaying the onset of symptoms. Cautious extrapolation showed that disease onset had occurred some 8 to 17 years prior to symptom onset. CONCLUSIONS: Our model provides important biological insights into the pathogenesis of PD, as well as its preclinical aspects. Our methodology can be applied widely to study many other chronic progressive diseases.

Treatable Neuropsychiatric Syndrome of Catatonia: A Case Review from Oman.

Catatonia is a potentially treatable neuropsychiatric syndrome less commonly encountered in developed countries these days. This review presents a case of a 19-year-old male with catatonic signs and symptoms compounded within a spectrum of a mood disorder, as well as literature review of the current treatment guidelines for this condition. There was no structural brain lesion or abnormality on cranial magnetic resonance imaging. The patient demonstrated favorable therapeutic response to benzodiazepine. This report discusses the management approach for catatonia through the case illustration, in an attempt to improve awareness and prompt recognition of this important disorder among physicians in Oman.