Retinal changes in multiple sclerosis: An optical coherence tomography and angiography study.

BACKGROUND: Multiple sclerosis (MS) is an autoimmune demyelinating disease of the central nervous system with neuroaxonal damage. It is the principal driver of non-traumatic disability in young adults. Visual symptoms are common and optic neuritis (ON) may be the revealing feature in up to 30% of cases. Structural optical coherence tomography (OCT) represents a biomarker of central nervous system neurodegeneration in MS. OCT-angiography (OCT-A) is a noninvasive tool allowing the study of retinal vasculature and the detection of microvascular damage in neuro-retinal diseases. In this study, we aimed to assess structural and microvascular retinal changes in patients with MS with and without ON and to correlate the findings with visual function and MS disability. METHODS: We conducted a cross-sectional study including patients diagnosed with MS according to the 2017 McDonald criteria. All patients underwent complete neurological examination with evaluation of the Expanded Disability Status Scale (EDSS) and the Multiple Sclerosis Severity Score (MSSS) and an ophthalmological examination including OCT and OCT-A. Patients were compared with age- and sex-matched healthy subjects. The primary endpoints were assessment of retinal nerve fiber layer (RNFL) thickness, ganglion cell layer (GCL+), and ganglion cell complex (GCL++) thicknesses on OCT. Vascular densities in the superficial capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillaris (CC) were assessed on OCT-A, as well as central avascular zone (CAZ) parameters, lacunarity and fractal dimension. RESULTS: A total of 160 MS eyes with and without a previous history of ON and 64 age- and gender-matched healthy eyes were analyzed. Among 160 eyes with MS, 69 had a history of ON. We observed a decrease in RNFL and GCL++ thickness in all 12 quadrants in MS patients when compared to healthy controls. Multivariate analysis by linear regression noted a significant correlation for temporal GCL++ and inferonasal RNFL thickness that were decreased in the MS group. A greater decrease in retinal layers thickness was identified in MS patients with a history of ON. On OCT-A, vascular density in (SCP) was significantly reduced in the MS group (P<0.002). A significant correlation between RNFL thickness and retinal vascular density was found but only in less than half of the hourly quadrants. A significant correlation was noted between visual acuity and CC density (P<0.0001). We also noted an inverse correlation between EDSS scores and CC density (P=0.02 and r=-0.275) and between MSSS and RNFL/GCL++ thicknesses. CONCLUSIONS: RNFL and GCL++ layers were thinner in MS patients with a history of ON and were reversely correlated with disease severity. Moreover, retinal vascular changes were observed in MS even in eyes without ON, and CC was reversely correlated with visual function and current disability. Thus, structural OCT coupled with OCT-A could represent a noninvasive and dynamic biomarker of MS severity and progression.

Arabic adaptation of the Edinburgh cognitive and behavioural Amyotrophic lateral sclerosis screen (ECAS-AR).

Current screening batteries for assessing neuropsychological function are not specific for Amyotrophic Lateral Sclerosis (ALS) and are considered as limited tools due to the physical disabilities associated with ALS. The Edinburgh Cognitive and Behavioural ALS screen (ECAS) was developed to detect the specific cognitive and behavioral changes that may occur among ALS patients. This study presents the ECAS developed for Arabic-speaking ALS patients (ECAS-AR) for use by healthcare professionals. ECAS was translated and modified to refined variety of Arabic language. Eighty-five ALS patients were included. Normative data were collected from 200 healthy controls (among them 97 were matched). Subjects were administered the ECAS-AR and two conventional cognitive screening batteries, Frontal Assessment Battery (FAB) and Mini-Mental State Examination (MMSE). ECAS-AR discriminated well between healthy controls and ALS patients. Significant differences were noted in language, executive functions, memory, and visuospatial domains between the two groups. The most prevalent deficit occurred in language and executive functions in ALS-specific functions. Whereas memory was more readily impaired in the lower and middle education groups concerning ALS non-specific functions. Verbal fluency tended to be preserved. Positive correlations were found between ECAS-AR and the standard cognitive tests supporting its full validity. The ECAS-AR version proposed will provide rapid, efficient and sensitive tools for healthcare professional to determine the cognitive-behavioural profile in Arabic-speaking ALS patients.

[Neuropsychiatric manifestations ushering pernicious anemia]. / Manifestations neuropsychiatriques inaugurant une maladie de Biermer.

Biermer disease or pernicious anemia is an autoimmune atrophic gastritis characterized by the lack of secretion of gastric intrinsic factor. This leads to an insufficient absorption of vitamin B12 in the ileum. Clinical manifestations are mainly hematologic. Neuropsychiatric manifestations are known but are less frequent especially early in the disease. Inaugural neuropsychiatric arrays are rare and various thus making diagnosis difficult. In this article, we report through two clinical cases different neuropsychiatric manifestations revealing pernicious anemia. Mrs. C.O., aged 56, presented after surgery for gallstones, an acute psychiatric array associated with gait disorders. She had no history of neurological or psychiatric problems. The psychiatric interview revealed delirious syndrome, depressive symptoms and anxiety. Neurological examination noted a flaccid paraplegia with peripheral neuropathic syndrome and myoclonus in the upper limbs. At the full blood count, a macrocytosis (VGM: 112.2fl) without anemia was found. The level of vitamin B12 in the blood was low. Cerebro-spinal MRI was suggestive of a neuro-Biermer and showed hyper signal in the cervical cord on T2-weighted sagittal section. In axial section, hyper signal appears at the posterior columns in the form of V. There were no brain abnormalities. A sensorimotor axonal polyneuropathy was diagnosed. The patient received vitamin B12 intramuscularly for ten days associated with neuroleptic treatment. Mrs. R.M., aged 40, was brought to the psychiatry consultation for acute behavioral disorders progressively worsening over a month. An anxiety syndrome, depressive syndrome and delirious syndrome were identified. Neurological examination showed a posterior cordonal syndrome with quadripyramidal syndrome. Full blood count showed a macrocytic anemia. Serum B12 level was collapsed. Cerebro-spinal MRI was normal. She received vitamin B12 with clinical and biological improvement. Features of pernicious anemia vary according to studies and age range. Digestive and hematological manifestations are well known. Neurological and psychiatric manifestations of pernicious anemia were also described in the early literature. They can be the initial symptoms or the only ones. However, inaugural neuropsychiatric features are often unrecognized. The most common psychiatric symptoms were depression, mania, psychotic symptoms, cognitive impairment and obsessive compulsive disorder. Neurological involvement includes mainly combined spinal sclerosis, peripheral neuropathy and dementia. Cerebellar ataxia and movement disorders are reported less often. Severity of neuropsychiatric features and therapeutic efficacy depends on the duration of signs and level of B12 deficiency. Macrocytic anemia may lack. Neuropsychiatric manifestations could be isolated or be the first manifestation of vitamin deficiency and occur without any hematological or gastrointestinal context. Pernicious anemia and serum B12 assay should be discussed in all patients with organic mental disorders, atypical psychiatric symptoms and fluctuation of symptomatology. Nevertheless, B12 level could be normal in genuine pernicious anemia diseases and macrocytic anemia may lack. Substitutive vitaminotherapy is required when diagnosis is strongly suspected and etiologic assessment is negative.

Depression in multiple sclerosis.

INTRODUCTION: Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system. Prevalence of depression in MS is significant. Existence of mood disorders alters the patients' life quality. OBJECTIVE: To determine the prevalence of depression in MS and establish the relationship between the severity of the disease, the onset of depression and their correlation with neuroimaging. METHODS: Fifty patients with MS aged 20 to 50 years followed at the Neurology Department of Charles-Nicolle Hospital from 2008 to 2012 participated in this study. The evaluation included a neuropsychological assessment, physical examination with EDSS and MRI. RESULTS: Association between MS and depression is common and known. In our study, prevalence of depression was 65%. Coexistence of other psychiatric disorders was found in around 10% of patients. Mood disorder was inaugural in some cases and delayed in others. This suggests that depression in MS may be linked to the disease or a result of a functional disability process. Occurrence of depression was not directly related to disease severity in all cases studied. CONCLUSION: Depression is a possible manifestation of MS. This mood disorder is due to the demyelinating brain damage or to a genetic susceptibility. However, a fortuitous association cannot be excluded.

Consensus recommendations for diagnosis and treatment of Multiple Sclerosis: 2023 revision of the MENACTRIMS guidelines.

With evolving diagnostic criteria and the advent of new oral and parenteral therapies for Multiple Sclerosis (MS), most current diagnostic and treatment algorithms need revision and updating. The diagnosis of MS relies on incorporating clinical and paraclinical findings to prove dissemination in space and time and exclude alternative diseases that can explain the findings at hand. The differential diagnostic workup should be guided by clinical and laboratory red flags to avoid unnecessary tests. Appropriate selection of MS therapies is critical to maximize patient benefit. The current guidelines review the current diagnostic criteria for MS and the scientific evidence supporting treatment of acute relapses, radiologically isolated syndrome, clinically isolated syndrome, relapsing remitting MS, progressive MS, pediatric cases and pregnant women. The purpose of these guidelines is to provide practical recommendations and algorithms for the diagnosis and treatment of MS based on current scientific evidence and clinical experience.

Tunisian version of the brief international cognitive assessment for multiple sclerosis: Validation and normative values.

BACKGROUND: The Brief International cognitive assessment for Multiple sclerosis (BICAMS) is a specific batterie used to identify cognitive impairment in Multiple Sclerosis (MS) in a reliable and easy way. To date, for the Arabic-speaking Tunisian MS patients, there is no consensus for the use of specific cognitive batteries in MS. OBJECTIVE: The aim of our work was to develop and validate the Tunisian version of the BICAMS (T-BICAMS) and to determine our own normative values. MATERIAL AND METHODS: Patients diagnosed with MS and followed up in the department of Neurology of Razi Hospital were recruited and matched to healthy controls according to age, sex and educational level. T-BICAMS validity was established by comparing MS and healthy controls for symbol digit modalities test (SDMT), brief visual memory test (BVMT-R) and Tunisian verbal learning tests (TVLT) which was used instead of the California verbal learning test (CVLT-II). RESULTS: The 104 MS patients and 104 healthy controls were comparable for age, sex and educational level. The MS group exhibited lower performances in all T-BICAMS domains compared to healthy controls: SDMT (x003Dp<10-3), BVMT-R (p = 0.002) and TVLT (p x003D<10-3). T-BICAMS Cronbach alpha value was 0.741. Normative values were identified for patients with MS: SDMT [39-40], BVMT-R [26-27] and TVLT [43-44]. Cognitive impairment was identified among 76 patients (73.1%). Males, lower educational levels and progressive MS were associated with a more severe cognitive impairment. CONCLUSIONS: The current study has established the BICAMS as a valid and reliable tool for the identification of cognitive impairment in the Tunisian MS population.

Clinical predictors of disease progression in a cohort of Tunisian progressive Multiple Sclerosis.

BACKGROUND: Knowledge about progressive Multiple Sclerosis (MS) is mainly based on Caucasian studies. In our North-African context, MS exhibits particular characteristics that are mainly related to a more severe phenotype. Given the limited data available, there is an imminent need to characterize progressive MS in our latitudes. OBJECTIVE: To describe the specificities of progressive MS and identify the inherent clinical predictors of disability accrual with a Tunisian cohort. METHODS: A retrospective, hospital-based study was conducted in the department of neurology of Razi hospital. Patients, who had been diagnosed with MS, were divided into relapsing MS (RRMS), secondary progressive MS (SPMS) and primary progressive MS (PPMS). Epidemiological, clinical and paraclinical data were compared among the three groups. RESULTS: Of the 504 patients, a progressive MS was described among 115 patients. This percentage of (22.8%) is divided into 13.9% SPMS and 8.9% PPMS. During the first clinical attack, motor symptoms have revealed to be predominant during PPMS (91.1%). For SPMS onset, the median time was 10 years, and was significantly delayed for patients with visual onset or full recovery from the first relapse. Patients with progressive MS exhibited a more rapid disability accumulation. CONCLUSION: Compared to Caucasians, Tunisians exhibited a faster rate of conversion to SPMS. According to our natural progressive MS history, early clinical features are predictors of MS disability accrual.

Multiple sclerosis 2017 McDonald criteria are also relevant for Tunisians.

BACKGROUND: Validation of the 2017 revised McDonald criteria was based on data from Caucasians. Among North Africans, Multiple Sclerosis prevalence, clinical phenotype and differential diagnosis are different. Hence, verifying the relevance of the latest revised criteria applied in North Africans was recommended. The aim of our study was to investigate the applicability and reliability of the revised 2017 McDonald criteria, compared to the 2010 version, with the relevance to the diagnosis of Multiple sclerosis in a Tunisian cohort. METHODS: Data from patients, with a typical clinically isolated syndrome, were re-analyzed retrospectively. Also, clinical, immunological and imaging characteristics were reviewed, according to the 2010, then 2017, McDonald criteria. Sensitivity, specificity, accuracy, positive predictive value and negative predictive value were evaluated to analyze the impact of the new criteria in everyday clinical practice. RESULTS: A total of 98 patients were included. Eighty-eight patients developed a definite Multiple Sclerosis, while ten had a different diagnosis. With relevance to the 2010 criteria, 41 patients (42%) were diagnosed with Multiple Sclerosis, after the first clinical attack. The 2017 revised criteria allowed to diagnose 32 more cases (73 patients = 74%). Sensitivity of the 2017 criteria was higher (77% versus 44%), but specificity was lower (33% versus 63%). CONCLUSIONS: Compared to the 2010 version, the 2017 McDonald criteria highlighted higher sensitivity, but lower specificity for Tunisians.

Thromboembolic events in inflammatory bowel disease.

GOAL OF THE STUDY: Inflammatory bowel disease (IBD) is associated with an increased risk of thromboembolic events (TEE) during outbreaks, increasing morbidity and mortality. The aim of our study was to specify the prevalence of TEE in IBD patients and to determine their epidemiological, clinical and evolutionary characteristics. MATERIEL AND METHODS: This is a retrospective study collecting all patients with IBD, who had a thromboembolic complication confirmed by imagery, between January 2012 and December 2018. RESULTS: One hundred patients with IBD were diagnosed during the study period. A TEE occurred in 6 patients (5.9%). These patients had an average age of 41 years, divided into 4 women and 2 men. Five patients had Crohn's disease and one patient had ulcerative colitis. The IBD was active in all patients. Five patients were already hospitalized and under preventive heparin therapy. Patients had deep venous thrombosis of the lower limbs in 3 cases, associated with pulmonary embolism in 1 case, cerebral venous thrombosis in 2 cases and pulmonary embolism isolated in 1 case. Thrombophilia investigations were negative in all patients. Evolution under medical treatment was favorable in 4 patients and fatal in 2 patients. CONCLUSION: In our study, the prevalence of TEE in patients with IBD was 5.9%. Thrombosis occurred during the active phase of IBD in all cases.

Spontaneous skin necrosis revealing protein S deficiency in Crohn's disease.

Inflammatory bowel diseases are associated with a state of hypercoagulability secondary to several mechanisms, protein S deficiency being one of these. It can be revealed by spontaneous skin necrosis in children. This condition is rare in adults with Crohn's disease. We are reporting a case of a 35-year-old woman with active Crohn's disease who presented a protein S deficiency responsible for an extensive spontaneous skin necrosis. The evolution was favourable after vascular filling, curative anticoagulation, antibiotic therapy, as well as a high-dose of corticosteroid therapy. We are reporting this case in order to emphasize the importance of considering skin necrosis as a possible cutaneous manifestation of inflammatory bowel diseases.

Evolution of iron speciation during hydration of C4AF.

It is now well accepted and demonstrated that calcium silicate, calcium aluminate and calcium sulfo aluminate (ettringite, AFm) phases exhibit a good capability to fix metals and metalloids. Unfortunately the role of minor phases and especially calcium-ferric aluminate phase, shorthand C4AF is not well defined. In other systems like in soils or sediments iron phases play a key role in the fixation of pollutant. In cement sorption isotherms, indicated that various metals can be retained by the C4AF hydrated products. Therefore the capabilities of those phase to retain heavy metal should not be neglected. Previous investigations have shown that the minerals formed during the hydration of C4AF are similar to those formed from C3A (pure tri-calcium aluminate) under comparable conditions. Nevertheless no investigation was conducted at the molecular level and there is still a controversy whether Fe substitutes for Al in the hydrated minerals in whole or in part, or if it forms FeOOH clusters scattered throughout the matrix. In this context we have conducted XAS experiments using synchrotron radiation. It was found that the hydration of C4AF forms C3AH6 (hydrogarnet) in which Fe randomly substitutes for Al as well as an amorphous FeOOH phase. Intermediate products like AFm (i.e., an ill organized lamellar phase) are also formed but rapidly evolve to C3AH6; iron does not seem to be incorporated in the AFm structure.

Unusual intraatrial thrombus in a neonate with coarctation of the aorta.

Left atrial thrombus in neonates is uncommon. We describe a newborn with coarctation of the aorta, in whom a thrombus confined to the left appendage was discovered on the first day of life and was thought to be a myxoma.

[Pulmonary fibrosis as a presentation of Wegener's granulomatosis]. / Une fibrose pulmonaire révélant une granulomatose de Wegener.

INTRODUCTION: Pulmonary fibrosis secondary to antineutrophil cytoplasmic antibodypositive vasculitis is rarely reported. Most cases have occurred in association with microscopic polyangiitis. CASE REPORT: We report the case of an 82-year-old man presenting with pulmonary fibrosis. The CT scan showed honeycombing and traction bronchiectasis with peripheral and basal predominance. The diagnosis of Wegener's granulomatosis was based on the association of positive antineutrophil cytoplasmic antibodies, urinary sediment abnormalities and renal biopsy findings. CONCLUSION: We emphasize, through this case, the rarity of interstitial fibrosis as a pulmonary manifestation of vasculitis and we discuss the pathogenesis of this association.