Egyptian adaptation and validation of the Edinburgh Cognitive and Behavioral Amyotrophic Lateral Sclerosis Screen (ECAS-EG).

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is the most common, fatal adult neuromuscular disease. It is a multi-system disorder characterized primarily by motor manifestations, but there is established evidence for cognitive and behavioral impairment, which is associated with poor prognosis, hence, the importance of tools for its assessment. The Edinburgh Cognitive and Behavioral Assessment Screen (ECAS) is an invaluable assessment tool for cognition in ALS-front temporal spectrum dementia (FTSD), as it accommodates physical challenges that usually confound traditional neuropsychological testing in those patients. OBJECTIVE AND METHODS: To validate the Egyptian Arabic version of ECAS (ECAS-EG) based on the original English scale. This is a prospective study. The ECAS was adapted and administered to 62 Egyptian ALS patients and 60 healthy controls. Patients were recruited from the Neuromuscular Unit, Ain Shams University Hospital. The ECAS was adapted to Egyptian Arabic after being translated using the back translation method. Internal consistency of the test, inter-rater reliability, and construct validity were assessed. RESULTS: The Egyptian Arabic version of ECAS (ECAS-EG) showed good internal consistency using Cronbach's alpha of 0.84. Inter-rater reliability was tested, values for all variables were compared, and no statistically significant differences were found (ICC = .997). ECAS-EG discriminated significantly between the patients from the control subjects (p-value of 0.001). There was a strong positive correlation between the ECAS-EG total score and the MoCA total score with a p-value of 0.001, thus indicating convergent validity. The test showed that 63% of Egyptian ALS patients were cognitively affected; most affected domains were executive functions and verbal fluency. CONCLUSION: The current study proves that the Egyptian version of the ECAS (ECAS-EG) is valid and reliable among Egyptian ALS patients and it would be applicable to the general Arabic-speaking population.

Diffusion and perfusion correlates of the 18F-MISO PET lesion in acute stroke: pilot study.

PURPOSE: Mapping the ischaemic penumbra in acute stroke is of considerable clinical interest. For this purpose, mapping tissue hypoxia with (18)F-misonidazole (FMISO) PET is attractive, and is straightforward compared to (15)O PET. Given the current emphasis on penumbra imaging using diffusion/perfusion MR or CT perfusion, investigating the relationships between FMISO uptake and abnormalities with these modalities is important. METHODS: According to a prospective design, three patients (age 54-81 years; admission NIH stroke scale scores 16-22) with an anterior circulation stroke and extensive penumbra on CT- or MR-based perfusion imaging successfully completed FMISO PET, diffusion-weighted imaging and MR angiography 6-26 h after stroke onset, and follow-up FLAIR to map the final infarction. All had persistent proximal occlusion and a poor outcome despite thrombolysis. Significant FMISO trapping was defined voxel-wise relative to ten age-matched controls and mapped onto coregistered maps of the penumbra and irreversibly damaged ischaemic core. RESULTS: FMISO trapping was present in all patients (volume range 18-119 ml) and overlapped mainly with the penumbra but also with the core in each patient. There was a significant (p ≤ 0.001) correlation in the expected direction between FMISO uptake and perfusion, with a sharp FMISO uptake bend around the expected penumbra threshold. CONCLUSION: FMISO uptake had the expected overlap with the penumbra and relationship with local perfusion. However, consistent with recent animal data, our study suggests FMISO trapping may not be specific to the penumbra. If confirmed in larger samples, this preliminary finding would have potential implications for the clinical application of FMISO PET in acute ischaemic stroke.

Lateralized readiness potentials can identify hemisphere of recovery in stroke patients.

BACKGROUND: Event related cortical potentials related to motor action are referred to as movement related cortical potentials. The late component of which is the readiness potential (RP) and its polarity is more negative in the hemisphere responsible for planning of motor action. This lateralized nature of RP during unilateral hand movement is studied as lateralized readiness potential (LRP) by calculating the contralateral-minus-ipsilateral difference wave for each hand. OBJECTIVE: The aim was to identify the hemisphere contributing to motor recovery in acute and chronic stroke patients through recording LRPs. METHODS: Twenty-nine cases with cerebrovascular stroke (15 acute and 14 chronic) were included in the study. EEG was recorded in response to self-cued button presses by the paretic side to obtain the averaged LRP amplitude. The hemisphere with greater negativity was considered the side of recovery. Functional recovery was assessed by Fugl Meyer test. RESULTS: In acute cases, recovery was more related to LRP activity in the contralesional hemisphere (73%), whereas lateralization was equal in chronic cases; 50% in either group. LRP amplitude was higher in the contralesional hemisphere (p = 0.02). Functional recovery assessed by the Fugl Meyer test (FM) was similar whether recovery was ipsi- or contralesional. CONCLUSIONS: Early after stroke, motor recovery is more likely to involve compensatory activity in the contralesional hemisphere, while in the chronic phase, the ipsilesional hemisphere may recover its function and become more active. Further research is needed to verify if the technique mentioned in our study could be used to guide customized NIBS protocols tailoring the optimal site and parameters for each patient.

Assessment of diagnostic potential of some circulating microRNAs in Amyotrophic Lateral Sclerosis Patients, an Egyptian study.

OBJECTIVE: Numerous studies have been carried out to identify the role of microRNA (miRNA) as potential biomarkers for many diseases including amyotrophic lateral sclerosis (ALS). The aim of this study was to explore the circulating levels of some miRNAs in cohort of Egyptian ALS patients in an attempt to correlate the selected miRNA profiles with disease progression. METHODS: Thirty ALS patients and 20 age and sex matched healthy controls were enrolled. Circulating miRNA levels were determined in venous blood samples, collected on EDTA, from all the study subjects. The selection of miRNA species (miR-206, miR-142-3p, miR-143-3p, miR-181a-5p, miR-106b-3p, miR-4516 and Let7f-5p) was based on their involvement in the pathophysiology of ALS and was further confirmed by data mining of specific miRNA databases (miRBase and miRDB). RESULTS: As compared to the control group, significant consistent upregulation was found in the levels of miR-206, miR-143-3p and to a lesser extent in miR-142-3p. An elevation trend, although not significant, was also found in the levels of miR-181a-5p, miR-106b-3p, and miR-4516. Interestingly, we found that the levels of miR-142-3p were elevated in familial cases, while that of miR-4516 were significantly increased in sporadic cases. Furthermore, the levels of Let7f-5p, although were generally lowered in ALS patients but were also decreased in familial cases as well as in spinal onset ALS as compared to bulbar onset. CONCLUSION: This is the first study investigating miRNA profiles in Egyptian ALS patients. We found that some miRNAs are significantly altered in ALS patients, and some may be used to distinguish familial and sporadic cases and bulbar and spinal onset. Larger study is needed, in which we will conduct a correlation of miRNA levels against variations in disease onset, progression as well as systemic inflammatory responses and the extent of neuromuscular involvement in Egyptian ALS patients in an attempt to identify environmental/occupational risk factors.

Watershed infarcts in transient ischemic attack/minor stroke with > or = 50% carotid stenosis: hemodynamic or embolic?

BACKGROUND AND PURPOSE: Watershed ischemia is a significant cause of stroke in severe carotid disease, but its pathophysiology is unsettled. Although hemodynamic compromise has long been regarded as the main mechanism-particularly with deep watershed infarction-there is some contradictory evidence from clinical and pathological studies for a role of microembolism, thought to result from plaque inflammation. However, no study so far has directly addressed these conflicting scenarios. METHODS: In 16 consecutive patients with recent transient ischemic attack/minor stroke and ipsilateral 50% to 99% carotid stenosis, we prospectively obtained (1) plaque inflammation mapping with (18)F fluorodeoxyglucose positron emission tomography; (2) brain MRI and perfusion MR; and (3) transcranial Doppler detection of microembolic signals (MES). Patients were excluded if on dual antiplatelets or with a potential cardiac source of emboli or contralateral MES. RESULTS: We found the expected significant relationship between (1) degree of stenosis and severity of distal hemodynamic impairment in the watershed areas; and (2) degree of in vivo plaque inflammation and rate of MES/hr. Deep watershed infarcts were present in 8 patients and MES in 8 (3 with both). There was no systematic association between the presence of deep watershed infarcts and either hemodynamic impairment or MES, but deep watershed infarcts were present only when either hemodynamic impairment or MES was present (P=0.01). CONCLUSIONS: This pilot study supports the idea that in symptomatic carotid disease, deep watershed infarcts result either from hemodynamic impairment secondary to severe lumen stenosis or from microembolism secondary to plaque inflammation. There was no direct evidence that both mechanisms act in synergy.

Stroke attributable to a calcific embolus from the brachiocephalic trunk.

BACKGROUND AND PURPOSE: Calcific brain embolization is a rare event that is usually secondary to cardiac valve calcification. We present a case of stroke caused by embolization of calcific material from the brachiocephalic trunk, probably induced by radiotherapy. SUMMARY OF CASE: A 56-year-old right-handed female developed left-sided hemiparesis, hemihypesthesia, and sensory inattention. She had a history of right breast carcinoma that was excised 8 years previously followed by radiotherapy. She had no other history of note. Computed tomography of the head and magnetic resonance imaging confirmed a calcific embolus in right middle cerebral artery and an acute infarction in the corresponding territory. Plain chest radiography, carotid ultrasonography, transthoracic and transoesophageal echocardiography failed to demonstrate the source of calcific embolism. Computed tomography of the thorax revealed heavy calcification of the brachiocephalic trunk and the origin of the right common carotid artery. CONCLUSIONS: Undertaking a vigilant systematic search for the source in cases of calcific embolization is necessary. The aorta and its main branches are possible, yet unusual, sources of calcific emboli that merit investigation.

Restless legs syndrome in Egyptian medical students using a validated Arabic version of the Restless Legs Syndrome Rating Scale.

OBJECTIVE: Restless legs syndrome (RLS) is a common movement disorder that has a variable prevalence and impact reported from different countries and specific populations. The current study validated an Arabic version of the International Restless Legs Syndrome Study Group (IRLSSG) rating scale (IRLS) and investigated the prevalence and impact of RLS in medical students at Ain Shams University in Cairo. METHODS: Translation of IRLS was done according to standard recognized guidelines provided by the publisher. A total of 389 medical students (217 female and 172 male) participated in the study and answered four questions to detect RLS as proposed by the IRLSSG. Subjects who answered positively the first three questions were recruited for face-to-face interview to exclude RLS mimics and to answer the IRLS. RESULTS: A total of 46 subjects (11.8%; 27 female and 19 male) met the four criteria for RLS. Of these, 39 subjects (10%) had idiopathic RLS. Five subjects (1.3%) and two subjects (0.5%) reported association with history of anemia and diabetes mellitus respectively. Their mean total IRLS score was 16.33 ± 5.3, with moderate severity (11.62 ± 3.9) and low impact (3.1 ± 1.8). The prevalence of individuals who had two or more episodes of RLS of at least moderate severity per week was 5.9%. CONCLUSION: In this specific population of Egyptian medical students, a within-average prevalence of RLS was found with low impact on quality of life similar to worldwide reported populations. RLS sufferers were of high prevalence among this cohort. The Arabic version of IRLS is reliable and valid for further research in Arabic countries.

Arabic cross cultural adaptation and validation of the National Institutes of Health Stroke Scale.

INTRODUCTION: The National Institutes of Health Stroke Scale (NIHSS), the most commonly used tool to quantify neurological deficit in acute stroke, was initially developed in English. We present our experience in developing and validating an Arabic version of the NIHSS (arNIHSS). RESULTS: In 6months, 137 patients were recruited (mean age±standard deviation 62±12years; 48 women). For interrater agreement, weighted kappa value ranged from 0.36 to 0.66 and intraclass correlation coefficient (ICC) for the whole scale was excellent at 0.95 (95% confidence interval [CI] 0.94-0.97). For intrarater agreement, weighted kappa ranged from 0.52 to 1.0 and the ICC was 0.94 (95% CI 0.87-0.98). The construct validity of the arNIHSS is demonstrated by its correlation with the DWI-ASPECT and the 3months mRS score (Spearman correlation -0.46 and 0.58 respectively; P<0.001 for both). CONCLUSION: We developed and validated a culturally adapted Arabic version of the NIHSS. Further validation in other Arab countries is recommended.

Imaging the cellular biology of the carotid plaque.

Carotid atherosclerotic disease is a significant preventable cause of stroke. Clinical decision-making in current practice is based primarily on detection of the severity of luminal stenosis, as determined by ultrasound or conventional angiographic imaging modalities. New insights in the biology of atherosclerosis now suggests that the morphological characteristics of the carotid plaque as well as the molecular and cellular processes occurring within it may be more important markers of plaque vulnerability and stroke risk. This review summarizes emerging applications in the molecular imaging of atherosclerosis and detection of the vulnerable carotid plaque. We discuss how advances in imaging platforms and biochemical technology (e.g. targeted contrast agents) have driven some exciting and promising novel diagnostic imaging approaches from bench to bedside.

Imaging the penumbra in acute stroke.

Imaging continues to have a huge impact on the understanding of the ischemic penumbra and the management of acute stroke. Determinants of penumbral tissue fate, such as age, hyperglycemia, hematocrit, and oxygen concentration, are increasingly being recognized using neuroimaging. The significance of the penumbra in the white matter and in posterior circulation stroke is also becoming clearer. Neuroimaging is also making invaluable contributions to clinical decision making in acute stroke, especially in relation to reperfusion therapies in the 3- to 6-hour time window. Despite ongoing questions over the choice of parameters to identify the penumbra and their respective clinical usefulness, imaging is gaining widespread use in acute stroke management. However, definitive evidence of its benefit is still lacking. This review explores the recent progress and controversies relating to imaging of the penumbra.