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Autism is heterogeneous multifactorial neurodevelopmental and neuropsychiatric disorder with repetitive and limited behaviors as well as communication deficits. Prevalence of autism in males is predominant than females, but their genetic association is unclear. The study was performed to investigate Y-chromosome haplotypes, significant risk variants and susceptibility genes associated with autistic Saudi young males with autism. Exome genotyping microarray analysis was performed in Saudi young boys with autism (cases, n = 47) and without autism and other genetic or neurodevelopmental disorders (control, n = 43) to identify the functional exonic risk variations among 243,345 exonic variations. The most significant single nucleotide polymorphisms (SNPs) of protein coding associated with autism in Saudi young boys were studied for functional enrichment. Y-chromosome haplotyping analysis of 6 SNPs such as rs1865680, rs2032624, rs2032658, rs2032631, rs9786153 and rs13447352 uncovered the most significant protective (ACGACA p = 2.94 × 10−9) among the controls and the high risk Y-haplotype (GAAGTC p = 6.85 × 10−6) among autistic boys. Exome association study revealed 6 susceptible genes, MCC, AUTS2, VSX1, SETBP1, CNTN3, and PCDH11Y that were known for autistic disorder. The significant predisposed genes with functional variants of Y-chromomere are strongly connected with spermatogenic failure (p = 8.02 × 10−8), azoospermia (p = 6.32 × 10−7), partial chromosome Y deletion (p = 7.66 × 10−6), HDMs demethylate histones pathway (p = 3.55 × 10−4) and immune system diseases (p = 4.11 × 10−3). Y-haplotypes and highly significant pathogenic exonic variants in MCC, AUTS2, VSX1, SETBP1, CNTN3 and PCDH11Y genes are more influential genetic factors for developing autism in boys of Arab origin.

Autism is a complex disease with genetic predisposition factors. Real factors for treatment and early diagnosis are yet to be defined. This study integrated transcriptome and exome genotyping for identifying functional variants associated with autism spectrum disorder and their impact on gene expression to find significant variations. More than 1800 patients were screened, and 70 (47 male/23 female) with an average age of 7.56 ± 3.68 years fulfilled the DSM-5 criteria for autism. Analysis revealed 682 SNPs of 589 genes significantly (p < 0.001) associated with autism among the putative functional exonic variants (n = 243,345) studied. Olfactory receptor genes on chromosome 6 were significant after Bonferroni correction (α = 0.05/243345 = 2.05 × 10−7) with a high degree of linkage disequilibrium on 6p22.1 (p = 6.71 × 10−9). The differentially expressed gene analysis of autistic patients compared to controls in whole RNA sequencing identified significantly upregulated (foldchange ≥0.8 and p-value ≤ 0.05; n = 125) and downregulated (foldchange ≤−0.8 and p-value ≤ 0.05; n = 117) genes. The integration of significantly up- and downregulated genes and genes of significant SNPs identified regulatory variants (rs6657480, rs3130780, and rs1940475) associated with the up- (ITGB3BP) and downregulation (DDR1 and MMP8) of genes in autism spectrum disorder in people of Arab ancestries. The significant variants could be a biomarker of interest for identifying early autism among Arabs and helping to characterize the genes involved in the susceptibility mechanisms for autistic subjects.

Neurocysticercosis is a neurologic disease caused by infection with larvae of Taenia solium. It is most commonly transmitted by consumption of pork, water contaminated with T. solium or poor hygiene habits. As only few cases of neurocysticercosis have been documented in Saudi Arabia, the authors report a case of neurocysticercosis in a young Indian female residing in Saudi Arabia who presented with generalized tonic-clonic seizures 6 days after a normal vaginal delivery. Her physical and laboratory investigations as well as chest X-ray and electroencephalogram were all normal. Computed tomography of the head revealed multiple calcified nodular lesions, and magnetic resonance imaging showed ring-enhancing lesion in the left frontoparietal area. Serum enzyme-linked immunosorbent assay (qualitative) was positive for immunoglobulin G antibodies for cysticercosis. A diagnosis of neurocysticercosis was made, and the patient was treated with dexamethasone and levetiracetam for 4 days before discharge. At the 3-month follow-up, the patient's condition had significantly improved, and her seizures had not recurred. This report recommends considering neurocysticercosis as a differential diagnosis in patients presenting with new-onset seizures, even if the symptoms do not initially indicate neurocysticercosis or if the patient resides in an area where the disease is rare.

Multiple sclerosis (MS) is a debilitating chronic inflammatory demyelinating disease that affects the central nervous system. The underlying cause of multiple sclerosis is unknown, yet there are various predisposing factors. Currently, bariatric surgery is an increasingly common solution for obesity. It has been reported that bariatric procedures result in miscellaneous neurological complications. To the best of our knowledge, this is the first case series to report the occurrence of MS following bariatric surgery. Here we report the cases of five patients who were recruited for inclusion following neurological examination, complete cerebrospinal fluid (CSF) study, enhanced cranial as well as spinal cord magnetic resonance (MR) images. Diagnosis of multiple sclerosis was made based on McDonalds criteria (2010). All cases were diagnosed with relapsing-remitting form of multiple sclerosis (RRMS) after various types of bariatric surgeries.

Background: Multiple sclerosis (MS) is one of the neuroinflammatory disorders that commonly affect the young. Injectable interferons beta-1a (Rebif 22-44, Betaseron 250 and Avonex 30) are one of the most important first line disease modifying therapies with less side effects compared to others. But the injection site reaction (ISR) is an important side effect, which can decrease the patient’s compliance to the medications that will lead to the disease progression. Objective: The purpose of this study was to compare the ISR in the three types of interferon (2 subcutaneous Rebif 22-44, Betaseron 250 and one intramuscular Avonex 30) and determine whether the route of administration (subcutaneous versus intramuscular) affects the frequency of ISR, and to reduce this side effect and ensure compliance. Methods: This was a single center, prospective observational study of 300 patients at King Fahad University Hospital (Al Khobar, Saudi Arabia) form September 2015 to August 2016 with a relapsing remitting form of multiple sclerosis for which 114 patents were receiving injectable interferon. A questionnaire was filled out by the participants including type of interferon, type of ISR and pain severity. Patients were then evaluated in our MS clinic and dermatology clinic after 1 week and 3 months. Data were analyzed by IBM-SPSS version 21. Appropriate statistical tests will be mandated per the collected data. Statistical significance is determined at p-value

Background Laryngeal cancer is one of the most commonly occurring cancers in head and neck malignancies with the majority of tumors being squamous cell carcinomas. Despite sharing similarities with other head and neck tumors, laryngeal tumors require a special approach in treatment due to the need for organ preservation. Non-surgical procedures, including chemotherapy, radiotherapy and novel targeted therapies are alternative options. However, the selection of appropriate treatment modality is crucial for recurrence-free survival. With the availability of targeted therapies, the biomarker expression pattern has become a vital tool in the selection of treatment and prognosis. The present study aims to study the variation of protein [epidermal growth factor receptor (EGFR), phosphatase and tensin homolog (PTEN) and p16INK4] expression and human papillomavirus (HPV) infection status in larynx carcinoma patients and correlate it with histopathological and clinical parameters. Methods This is a retrospective study comprising laryngeal carcinoma tumor samples from 18 patients. The samples were analyzed for EGFR, PTEN and p16INK4 expression by immunohistochemistry and HPV status by chromogenic in-situ hybridization (CISH). Results The EGFR over expression was observed in the 83.3% of patients. This high EGFR expression was associated with lymph node positivity (P=0.045) and advanced disease stage (P=0.035). Furthermore, the smoking status of the patients is correlated with a better prognosis (P=0.048). The PTEN was negatively expressed in 94.4% patients. Conclusions The association of EGFR over expression with lymph node status and advanced stage of the disease supports the role that EGFR plays in the tumor metastasis and invasion in laryngeal carcinoma patients. Therefore, the use of anti-EGFR targeted therapy in cases of laryngeal carcinoma may improve the prognosis of these patients. The other studied proteins p16INK4, PTEN and HPV infection did not show any correlation with prognosis and other clinical parameters.

Background: Post-stroke fatigue is a common symptom which needs to be assessed by a psychometrically sound tool.Objectives: To investigate the psychometric properties of an Arabic version of the fa...

Objectives: To determine if there is a relationship between acute stress and either the onset or relapse of multiple sclerosis (MS) and to discover how different types of acute stressors may be involved. Methods : This study was carried out in Saudi Arabia between September 2017 and June 2018 and involved King Fahad University Hospital in Eastern province, Arfa Multiple Sclerosis Society in the Central and Western province of Saudi Arabia. A cross-sectional descriptive study was performed using an Arabic self-constructed questionnaire consisted of 4 sections: 1) demographic data and time of diagnosis; 2) emotional/psychological stressors; 3) environmental/physical stressors; and 4) 4 specific stressors measuring their effect on the severity and recurrence of attacks. Results : A total of 370 patients participated in the study. Almost half of patients reported no effect of family problems on their disease, whereas the other reported that family problems have an impact on the onset or relapse of the disease. Majority of patients reported that work and social life stressors affect the recurrence of attacks. Cold weather showed no effect on MS; however, hot weather and physical activity increased the number of attacks. Continuous thinking about social stress and problems, mood swings, and sleep deprivation showed an impact on the severity and recurrence of attacks. Financial problems showed no effect. Conclusion : Study indicates that an association exists between acute stress and relapse in MS but not the disease onset. Saudi Med J 2019; Vol. 40 (4): 372-378 doi: 10.15537/smj.2019.4.24010 How to cite this article: AlZahrani AS, Alshamrani FJ, Al-Khamis FA, Al-Sulaiman AA, Al Ghamdi WS, Al Ghamdi OA, et al. Association of acute stress with multiple sclerosis onset and relapse in Saudi Arabia. Saudi Med J . 2019 Apr;40(4):372-378. doi: 10.15537/smj.2019.4.24010.

Objectives To mitigate the incidence of recurrent stroke in patients, dual antiplatelet therapy comprising aspirin and clopidogrel is usually administered. Clopidogrel is a prodrug and its bioactivation is catalyzed by cytochrome P450 (CYP)2C19. The main objective of this work was to determine the prevalence of CYP2C19*2 carriers in Saudi ischemic stroke patients and assess the suitability of using genotyping to guide antiplatelet therapy in a university hospital setup. Methods This prospective (2018–2019) study was conducted on 256 patients (age 61 ± 12.5) clinically diagnosed with ischemic stroke who were genotyped using Spartan RX CYP2C19 assay. Results From the total patient group (256), upon admission, 210 patients were prescribed either aspirin, clopidogrel or dual antiplatelet therapy. Of the 27 patients with the CYP2C19*2 allele who were prescribed clopidogrel (18) or dual antiplatelet therapy (9), only 21 patients could be followed up for a period of six months post stroke event, in addition to 21 age- and sex-matched patients with the normal allele. The CYP2C19*2 allele carriers had a statistically significant increased risk of recurrent stroke compared to patients carrying the normal allele. Conclusions This study shows the suitability of using genotyping to guide antiplatelet therapy in ischemic stroke patients in a clinical setting.

Objective: Our study aims to evaluate the etiologic and clinical features of cerebral venous sinus thrombosis (CVST) in Saudi Arabia, and secondarily whether gender plays a role in CVST. Materials and Methods: Data were collected retrospectively from the stroke registry during the period from January 2008 to April 2018, and the patients with the diagnosis of CVST were identified, and data were analyzed for any gender-specific differences in clinical presentation and etiology of cerebral venous thrombosis. Results: There were 15 females while 11 males with a female:male ratio of 1.4:1. The mean age was 29.4± standard deviation 8.9 with the age range of 15–49. Headache was the most common and usually the first presenting symptoms present in 65% followed by hemiparesis and cranial nerve palsies. The first neurological examination was normal in 9/26 (34.6%) of the patients, while the common abnormality was cranial nerve palsies. Infections and trauma played an important part in risk factor analysis of our patient after the pregnancy- and hormone-related conditions. Some significant differences between the clinical presentation and risk factors among males and females were noted as age at presentation was higher in females while trauma and infections were common in male patients, although the involvement of the sinuses and response to treatment did not prove to be statistically significant. Conclusion: The results of this study were similar to the available literature with few differences. The relatively higher proportion of males in our study can be explained partly with more cases of traumatic CVST. Some important differences were noted between the risk factors and clinical presentation among genders. Large-scale prospective studies are needed to further clarify these differences.

Context: Medication nonadherence is a significant barrier in achieving seizure freedom in patients with epilepsy. There is a deficiency of data about the reasons for nonadherence in Saudi population. Aims: The aim of this study is to prove the existence of nonadherence to antiepileptic drugs (AEDs) in patients with epilepsy and identify the responsible factors. Setting and Design: This is a prospective, cross-sectional study carried in the Department of Neurology at King Fahd Hospital of the University affiliated with Imam Abdulrahman Bin Faisal University. Subjects and Methods: Patients of all ages diagnosed to have epilepsy as mentioned in their medical record and taking antiepileptic medications were interviewed using a questionnaire. Statistical Analysis Used: Statistical analysis was performed using IBM Statistical Package for the Social Sciences version 21 (IBM Corp., Armonk, NY, USA). Statistical significance was defined as two-tailed with a P ≤ 0.05. Results: Among 152 participants, 52.6% were male and 47.4% were female. Mean age of the patients was 28 ± 14.3 (mean ± standard deviation) years. Of 152 patients, 48.7% were found to be nonadherent to their AED therapy. The most commonly identified factor was forgetfulness. Nonadherence was significantly associated with poor seizure control (P = 0.002). Conclusion: Nonadherence to the AED is common among patients with epilepsy and affects seizure control adversely.

Cerebral vein and dural sinus thrombosis (CVST) is an uncommon cause of stroke, but its delayed diagnosis carries significant morbidity and mortality. Several studies have reported higher incidence of CVST than that previously reported. The clinical presentation of CVST varies and can be atypical. Advancement in neuroimaging modalities has made it possible to make an early diagnosis and initiate management with a wide range of therapeutic options, including direct oral anticoagulants and endovascular treatment. This narrative review summarizes the epidemiology, clinical aspects, diagnosis and management of CVST.

Idiopathic hypertrophic pachymeningitis (IHP) can resemble other disorders associated with spinal compression. It is a rare inflammatory fibrosing disease of the dura of unidentified etiology and is considered a diagnosis of exclusion. We present a case of idiopathic hypertrophic spinal pachymeningitis occupying a long segment of cervical dura. This is a case of 38-year-old female patient, who suffered progressive neck pain for 2-year duration. Examination revealed spasticity in all four limbs, plus three symmetric reflexes all over, and the sensory level at T4. Magnetic resonance imaging showed spinal cord compression by a thickened anterior and posterior dura adjacent to the cord from C2 to C7. The diagnosis of spinal IHP was confirmed through biopsy. The patient improved after treatment with corticosteroids. Early surgical intervention with postoperative corticosteroid therapy is a known treatment for this disease, as a way to prevent irreversible neurological damage.

Objectives: To assess the burden and describe the pattern of neurological disorders requiring admissions in a teaching hospital of Al Khobar. Methods: This is a retrospective, cross sectional study, carried out in the Neurology Department of King Fahd Hospital of the University from January 2009 to December 2016. Neurological disorders were grouped as ischemic stroke, intracerebral hemorrhage, transient ischemic attack, cerebral venous sinus thrombosis, seizure disorders, central nervous system infection, multiple sclerosis, neuropathies, myopathies, headache, dementia and miscellaneous group. Data was entered and analyzed by Statistical Package for the Social Science (SPSS) version 22.0 (IBM Corp., Armonk, NY, USA). Results: The records of 1,317 patients admitted under Neurology Service were analyzed. Out of that, 740 (56.2%) were male and 577 (43.8%) were female. Mean age was 46.9±24 years (mean±standard deviation). Ischemic stroke was the most common diagnosis (32%) followed by seizures (20%). Multiple sclerosis accounted for around 8% and central nervous system infections 5% of neurological admission. Conclusion: Ischemic stroke was found to be the most common etiology for hospitalization in our study. The results of our study are similar to previous literature. An urgent need to control major risk factors such as diabetes and hypertension is warranted to minimize the burden of stroke.

Introduction Repetitive nerve stimulation (RNS) showing ≥ 10% decrement is considered the cutoff for myasthenia gravis (MG), but this has never been validated. The objective of this study was to find an optimal validated cutoff value for decrement on RNS. Methods We performed retrospective chart review of patients who had electrophysiological assessment for possible MG from 2013 to 2015. Results A total of 122 patients with MG and 182 controls were identified. RNS sensitivities for generalized and ocular MG using the traditional ≥10% cutoff value were 46% and 15%, respectively, for frontalis recordings, and 35% and 19%, respectively, for nasalis recordings. Using a decrement cutoff value of 7% for frontalis and 8% for nasalis increased the sensitivities by 6-11%, with specificities of 95-96%. Conclusions For RNS in facial muscles, we suggest a cutoff value of 7-8%, which increases test sensitivity by 6-11%, while preserving high specificity for the diagnosis of MG. Muscle Nerve, 2016 Muscle Nerve 55: 166-170, 2017.

Objectives To explore the increased diagnostic yield of repetitive nerve stimulation (RNS) following activation for myasthenia gravis (MG) diagnosis, and compare with testing an additional muscle at rest only. Methods We performed a retrospective chart review of patients diagnosed with MG attending the neuromuscular clinic from 2013 to 2015 and extracted data on electrophysiological studies, including frequency of decrement of 10% or above, with facial RNS at rest, and following activation. Results The total cohort included 102 patients with MG, 65 with generalized, and 37 with ocular MG. Facial RNS sensitivities for diagnosing MG were 32–46% and 14–19% respectively. The increase in RNS sensitivity following muscle activation was 6–8% for frontalis muscle recordings, and 0–2% for nasalis muscle recordings. Recording from both muscles at rest only, increased the sensitivity by 9–15%. Conclusion RNS is a valid method for confirming a clinical diagnosis of MG. The increased diagnostic yield of RNS following activation is controversial. Our study shows that the increased diagnostic yield of facial RNS following activation is modest, and is less than performing facial RNS in a second muscle. Significance Performing facial RNS in an additional muscle at rest appears to be more sensitive than facial RNS in a single muscle after activation.

Objective: To identify the different subtypes of acute ischemic stroke, and estimate the frequency of various risk factors among these patients. Methods: In this retrospective, cross-sectional study, we reviewed the medical records of patients admitted with the diagnosis of acute ischemic stroke at King Fahd Hospital of the University, Al-Khobar, Saudi Arabia from March 2008 till December 2015. The demographic characteristics, subtypes of stroke, risk factors (hypertension, diabetes mellitus, hyperlipidemia, coronary artery disease, atrial fibrillation, valvular heart disease) and other relevant data were documented on pre-defined data sheets. Results: The records of 343 patients were included in the study; 64.4% were male and 35.6% were female. The mean age was 59.3+13.6 (mean+SD) years for males, and 66.8+14.9 years for females. Small vessel occlusion was the most common etiologic subtype of ischemic stroke (32.1%), followed by cardio embolic (21.9%), and large artery atherosclerosis (14.6%). The middle cerebral artery was the most commonly affected territory. Hypertension was found in 78.1%, diabetes mellitus in 62.7%, hyperlipidemia in 54.8%, and ischemic heart disease in 24.2% of patients. Conclusion: Small vessel occlusion was the most common etiology in our cohort. The onset of stroke at a relatively younger age group in the male population is of great concern and needs to be verified by further epidemiological studies. Adequate control of modifiable risk factors may help in reducing the disease burden caused by stroke.

Sickle cell anemia (SCA), a disorder with an important inflammatory component, where vasoocclusion is major contributor to the disease pathophysiology. Pro-inflammatory cytokines play an important regulatory role in the process of inflammation. We investigated the expression TL1A/DR3/DcR3 cytokine signaling pathway in peripheral blood mononuclear cells (PBMC) and their corresponding plasma levels in SCA subjects who presented with acute painful episodes.PBMC were isolated from the blood of SCA subjects and normal healthy controls. RNA isolated from PBMC was used for real time gene expression of TL1A/DR3/DcR3. Gene expression was compared in subgroups within SCA subjects with co-inherited fetal hemoglobin (HbF) or alpha-globin gene deletions. Plasma prepared from blood was used for determination of TL1A/DR3/DcR3 proteins by ELISA assays.In the PBMC of SCA subjects, expression of TL1A and DcR3 is elevated, while DR3 expression is lowered in comparison to normal control PBMC. In SCA subjects with HbF 10%, TL1A/DcR3 expression is lower, while HbF 10% is associated with increased TL1A/DcR3 expression. Moreover, subjects with HbF 10% appear to have significantly fewer pain episodes in comparison to those with HbF 10%. Deletion of alpha-globin genes appears to have no significant effect on TL1A/DR3/DcR3 expression. Circulating levels of TL1A, DR3 and DcR3 in plasma were significantly elevated in SCA subjects.Elevated TL1A and DcR3 expression in PBMC of SCA subjects during painful vasoocclusive crisis, suggest an altered TL1A expression may contribute to the pathophysiology of vasoocclusive crisis in SCA. HbF 10% appears to moderate TL1A elevation, while HbF 10% exacerbates TL1A/DcR3 responses. Furthermore, subjects with HbF 10% have significantly lower pain episodes reported as compared to subjects with HbF 10%.

One of the most common neurodevelopmental disorders worldwide is autism spectrum disorder (ASD), which is characterized by language delay, impaired communication interactions, and repetitive patterns of behavior caused by environmental and genetic factors. This review aims to provide a comprehensive survey of recently published literature on ASD and especially novel insights into excitatory synaptic transmission. Even though numerous genes have been discovered that play roles in ASD, a good understanding of the pathophysiologic process of ASD is still lacking. The protein⁻protein interactions between the products of NLGN, SHANK, and NRXN synaptic genes indicate that the dysfunction in synaptic plasticity could be one reason for the development of ASD. Designing more accurate diagnostic tests for the early diagnosis of ASD would improve treatment strategies and could enhance the appropriate monitoring of prognosis. This comprehensive review describes the psychotropic and antiepileptic drugs that are currently available as effective pharmacological treatments and provides in-depth knowledge on the concepts related to clinical, diagnostic, therapeutic, and genetic perspectives of ASD. An increase in the prevalence of ASD in Gulf Cooperation Council countries is also addressed in the review. Further, the review emphasizes the need for international networking and multidimensional studies to design novel and effective treatment strategies.

Introduction Polyneuropathy is one of the most prevalent neurologic disorders. Although several studies explored the role of the neurological examination in polyneuropathy, they were mostly restricted to specific subgroups of patients and have not correlated examination findings with symptoms and electrophysiological results. Objectives To explore the sensitivity and specificity of different neurological examination components in patients with diverse etiologies for polyneuropathy, find the most sensitive combination of examination components for polyneuropathy detection, and correlate examination findings with symptoms and electrophysiological results. Methods Patients with polyneuropathy attending the neuromuscular clinic from 01/2013 to 09/2015 were evaluated. Inclusion criteria included symptomatic polyneuropathy, which was confirmed by electrophysiological studies. 47 subjects with no symptoms or electrophysiological findings suggestive for polyneuropathy, served as controls. Results The total cohort included 312 polyneuropathy patients, with a mean age of 60±14 years. Abnormal examination was found in 95%, most commonly sensory findings (86%). The most common abnormal examination components were impaired ankle reflexes (74%), vibration (73%), and pinprick (72%) sensation. Combining ankle reflex examination with vibration or pinprick perception had the highest sensitivity, of 88%. The specificities of individual examination component were generally high, excluding ankle reflexes (62%), and vibration perception (77%). Abnormal examination findings were correlated with symptomatic weakness and worse electrophysiological parameters. Conclusion The neurological examination is a valid, sensitive and specific tool for diagnosing polyneuropathy, and findings correlate with polyneuropathy severity. Ankle reflex examination combined with either vibration or pinprick sensory testing is the most sensitive combination for diagnosing polyneuropathy, and should be considered minimal essential components of the physical examination in patients with suspected polyneuropathy.

Selecting proper outcome measures is important for clinical practice and clinical studies assessing treatable neuropathies, such as chronic inflammatory demyelinating polyneuropathy (CIDP).To explore clinical characteristics and impairment and disability scores in CIDP patients, and assess their relationship to different CIDP Disease Activity Status (CDAS) classes.We performed a retrospective chart review of CIDP patients attending the neuromuscular clinic between January 2014 and December 2015, and explored clinical characteristics, and various impairment and disability scores for different CDAS classes.The total cohort included 69 CIDP patients. A higher CDAS class was correlated with more frequent motor and sensory deficits, worse sensory sum score, worse scores on the disability scales, including the Rasch-built overall disability scale (RODS) and Overall Neuropathy Limitation Scale (ONLS), and lower treatment responsiveness. ONLS and RODS scales were significantly correlated (r=-0.86, p0.0001). A change of one point or more in the ONLS score, was associated with a corresponding change of 4.1-4.5 points in the RODS score.The study results show that a higher CDAS class is correlated with a more severe neuropathy. This was reflected by the sensory sum score, RODS and ONLS. These results might help profile CIDP patients at different CDAS classes for clinical practice and future clinical studies.

Introduction Evaluation of disease status in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) is often done by a combination of clinical evaluation and electrodiagnostic studies. A CIDP disease activity status (CDAS) was developed to standardize outcomes in CIDP patients. We aimed to determine if the CDAS was concordant with classical evaluation and whether CDAS enables benchmarking of CIDP. Methods We performed a retrospective chart review of 305 CIDP patients and identified 206 patients with > 1 visit and applied the CDAS to this cohort. We examined relationships between the CDAS and classical evaluation as to outcomes and compared our cohort to other CIDP cohorts who had CDAS. Results We found that the CDAS mirrored disease severity as measured by electrophysiology and vibration perception thresholds in that CDAS class 5 had more severe neuropathy. Our results are similar to other cohorts in the middle CDAS strata with the exception of fewer subjects in CDAS 1 and more in CDAS 5. The only demographic factor predicting CDAS 5 in our cohort was age, and the overall treatment response rate using the CDAS classification was 79.3%. Conclusions CDAS appears to have sufficient face-validity as a grading system to assess disease activity in relation to treatment status. The use of CDAS appears to allow benchmarking of patients with CIDP that may be useful in subject selection for clinical trials and also to highlight differences in practice.

Repetitive nerve stimulation (RNS) showing ≥ 10% decrement is considered the cutoff for myasthenia gravis (MG), but this has never been validated. The objective of this study was to find an optimal validated cutoff value for decrement on RNS.We performed retrospective chart review of patients who had electrophysiological assessment for possible MG from 2013 to 2015.A total of 122 patients with MG and 182 controls were identified. RNS sensitivities for generalized and ocular MG using the traditional ≥10% cutoff value were 46% and 15%, respectively, for frontalis recordings, and 35% and 19%, respectively, for nasalis recordings. Using a decrement cutoff value of 7% for frontalis and 8% for nasalis increased the sensitivities by 6-11%, with specificities of 95-96%.For RNS in facial muscles, we suggest a cutoff value of 7-8%, which increases test sensitivity by 6-11%, while preserving high specificity for the diagnosis of MG. Muscle Nerve, 2016 Muscle Nerve 55: 166-170, 2017.

The prevalence of morbid obesity is increasing worldwide, which over the past decade, has resulted in an increase in the number of obese patients undergoing bariatric surgery. All surgical procedures carry a varying degree of risk; however, bariatric surgery is specifically associated with a number of metabolic, nutritional, and neurological complications. The neurological complications include encephalopathy, peripheral neuropathy, myelopathy, plexopathy, and radiculoneuropathy. This case report involves an 18-year-old female who underwent successful bariatric surgery for morbid obesity. Postoperatively, the patient developed severe painful weakness of the lower limbs and was found to have diffused sensory motor polyneuropathy. It was concluded that sensory-motor polyneuropathy was the most likely cause of the patient's weakness. Reporting this case is important as the author believes that the consequences of bariatric surgery may not be well-known to both patients and surgeons.