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.

Surgical rhinoplasty after prior hyaluronic acid based nose remodeling.

BACKGROUND: Medical rhinoplasty by hyaluronic acid injection (HA) has become nowadays a common practice. The number of patients requesting surgical rhinoplasty and having already undergone one or more injections of HA is increasing. However, the literature lacks publications regarding the management of these patients. OBJECTIVES: The aim of this study is to discuss the management of patients who have been treated with previous nasal HA injections and who seek surgical rhinoplasty, and elaborate a treatment protocol and algorithm to standardize surgical plans. METHODS: We are reporting case studies based on our clinical experience. We also reviewed the literature to suggest perioperative management for rhinoplasty with previous HA injections. RESULTS: Hyaluronidase injection preoperatively allows to carry out an accurate preoperative analysis of the nasal deformities to treat, in order to make an adapted treatment plan. Postoperative course is similar to other rhinoplasty cases without the use of this enzyme. CONCLUSION: Hyaluronidase should be used in all patients with nasal injections of HA (unless contraindications), who are willing to undergo a surgical rhinoplasty. The operation can be undertaken at one-week interval as soon as the edema subsides and no further treatments are necessary.

The effects of cognitive behavioral therapy on selected physical, physiological parameters, exercise and nutritional behaviors in diabetic persons.

OBJECTIVE: Due to the high prevalence of diabetes in the world, it seems that one of the most important strategies to prevent and treat this disease are lifestyle changes. The present study aimed to investigate the effects of cognitive behavioral therapy on selected physical and psychological parameters, exercise, and nutritional behaviors in diabetic patients. PATIENTS AND METHODS: This was a cross-sectional study of volunteers with type 2 diabetes. Patients were randomized into two groups: an intervention (n=32) and a control group (n=31). The Beck Physical Activity Questionnaire was used to assess physical activity in combination with the Beck Depression Inventory and the Pittsburgh Sleep Quality Index to assess the levels of depression and the quality of sleep. Cognitive behavior therapy (CBT) training consisted of six sessions of cognitive behavioral therapy interventions. The intervention consisted of a face-to-face individual counseling session. The patients received a special diet according to their basal metabolic rate (BMR). At all sessions, dietary advice was reviewed, and during the treatment period, they were asked to record their diet and physical activity. Body composition and blood samples were measured before and after three months of the experiment for both groups. RESULTS: Blood glucose, blood lipid profiles (except high-density lipoprotein), lifestyle, behavior patterns and body composition were significantly improved following CBT intervention (p≤0.05). CONCLUSIONS: CBT can play a key role in improving physiological and psychological parameters in diabetic patients.

The effects of daytime melatonin ingestion on arousal and vigilance vanish after sub-maximal exercise: a pilot study.

OBJECTIVE: Daytime melatonin ingestion is known to induce sleep at rest, which may affect arousal and vigilance. Physical exercise is known to produce an increase in core temperature and circulating cortisol which can enhance arousal and vigilance. The effect of submaximal exercise on vigilance and arousal following acute melatonin ingestion has not yet been studied. The present study aimed at investigating the effect of submaximal exercise on vigilance and arousal following daytime melatonin ingestion. PATIENTS AND METHODS: Eight physical education students undertook 45 min of submaximal exercise (at 60% of maximal aerobic speed) on a treadmill after melatonin-(6 mg) or placebo ingestion, in a randomized and counterbalanced order. RESULTS: Heart rate (HR), rectal temperature (Tre), felt arousal scale (FAS), and thermal sensations (TS) were recorded at baseline (pre-exercise), immediately after exercise (post-exercise), and after 30 min of recovery (30 min post-exercise). Blood was sampled for lactate and cortisol. At 30 min post-exercise, the Tre, HR, blood pressure, lactate, FAS, and TS were measured. The participants performed vigilance tests pre-exercise, post-exercise and 30 min post-exercise. Daytime melatonin ingestion affected arousal and vigilance in the pre-exercise period (p < 0.05) but had no effect on Tre, HR, blood pressure, lactate, TS, arousal, and vigilance measured 30 min post-exercise (p > 0.05). CONCLUSIONS: The negative effects of melatonin ingestion on vigilance and arousal vanished after a 45 min of submaximal exercise. The hypnotic effect of melatonin observed in the pre-exercise dissipated in the post-exercise period, possibly due to the significant elevation of Tre, HR, and cortisol at the end of submaximal exercise.

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.

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.

Guidelines for clinical trials of frontal fibrosing alopecia: consensus recommendations from the International FFA Cooperative Group (IFFACG).

BACKGROUND: Frontal fibrosing alopecia (FFA) has become one of the most common causes of cicatricial alopecia worldwide. However, there is a lack of clear aetiology and robust clinical trial evidence for the efficacy and safety of agents currently used for treatment. OBJECTIVES: To enable data to be collected worldwide on FFA using common criteria and assessment methods. METHODS: A multicentre, international group of experts in hair loss was convened by email to create consensus recommendations for clinical trials. Consensus was defined at > 90% agreement on each recommended part of these guidelines. RESULTS: Standardized diagnostic criteria, severity rating, staging, and investigator and patient assessment of scalp hair loss and other clinical features of FFA were created. CONCLUSIONS: These guidelines should allow the collection of reliable aggregate data on FFA and advance efforts in both clinical and basic research to close knowledge gaps in this condition.

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.

[Pyoderma gangrenosum and systemic lupus erythematosus: A rare association]. / Pyoderma gangrenosum et lupus érythémateux systémique : une association rare.

INTRODUCTION: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that is traditionally associated with systemic disorders such as chronic inflammatory bowel diseases, rheumatoid arthritis and malignant hematologic disorders. Its association with systemic lupus erythematosus (SLE) is rare and not well known. We report a case of this association with a review of the literature. CASE REPORT: A 43-year-old female patient, followed for 4 years for SLE, presented a deep ulceration of the anterior face of the left thigh with inflammatory borders, an ulcerated nodule of the right shoulder and four small ulcerations of the back of the right hand. The biopsy of the ulceration of the left thigh concluded to PG. The patient was treated by corticosteroids with complete healing of lesions. CONCLUSION: The prognosis of lupus does not seem to be aggravated by PG and the treatments of a SLE flare are usually enough for treating associated PG.

[Systemic lupus erythematosus presenting as Stevens-Johnson syndrome]. / Lupus érythémateux systémique se présentant comme un syndrome de Stevens-Johnson.

Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening dermatological conditions. Their most common cause is medication. However, in a small proportion of patients these dermatological conditions could be the first presentation of systemic lupus erythematosus. We now describe a 34-year-old patient who presented with manifestations of Stevens-Johnson as a first feature of systemic lupus erythematosus. Systemic lupus erythematosus reveled by Stevens-Johnson syndrome has been infrequently reviewed in the previous literature. This diagnosis should be considered when cutaneous adverse drug reactions occur without clear drug causality.

[SAPHO syndrome]. / Le syndrome SAPHO.

SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome is a rare entity characterized by the association of heterogeneous osteoarticular and cutaneous manifestations that have for common denominator an aseptic inflammatory process. The etiopathogeny of this disease is still a matter of debate. Although it has been related to the spondylarthritis family, an infectious origin is suggested. Diagnosis is based on the presence of at least one of the three diagnostic criteria proposed by Kahn. The treatment includes NSAIDs, antibiotics, corticosteroids, methotrexate and more recently the bisphosphonates and the TNFα inhibitors.

[Primary cutaneous osteosarcoma]. / Ostéosarcome cutané primitif.

BACKGROUND: Osteosarcoma is a malignant tumor of mesenchymatous origin that chiefly affects the metaphysis of long bones. The extraskeletal form of the disease is rare, and even rarer is a cutaneous site, whether metastatic or primary. Herein, we report a new case of primary cutaneous osteosarcoma. PATIENTS AND METHODS: A 54-year-old woman presented a hard subcutaneous nodular tumor on her left arm noted 1 year earlier. The diagnosis of cutaneous osteosarcoma was made on the basis of histological analysis of the lesion, which showed a sarcomatous dermal-hypodermal proliferation secreting osteoid. Clinical and radiological staging ruled out any extra-cutaneous spread, particularly to bone, thus confirming the primary cutaneous nature of the osteosarcoma. DISCUSSION: Primary cutaneous osteosarcoma is a rare tumor, diagnosis of which is normally based on histopathological features.

The course and prognosis of pemphigus in 47 Tunisian patients.

BACKGROUND: Pemphigus is a severe and life-threatening autoimmune bullous dermatosis. OBJECTIVE: We have analysed parameters that may influence prognosis of pemphigus (P). METHODS: It was a retrospective study (2002-2010), with pemphigus considered as severe if body surface involvement ≥ 30%. Disease control and relapse-free survival (Kaplan-Meier) were analysed and compared according to several parameters (P < 0.05). RESULTS: 47 cases of pemphigus were collected, mean age 51 years ± 16.8 (F/H = 3.27). There were 30 pemphigus profundus and 17 superficial pemphigus. The median remission period was of 9 months (1.2 months-5 years). The mean healing time was of 40 days (6 days-4 months), which did not depend on type of P, its severity or infectious complications, whereas it was shorter in aged patient (≥ 65 years) compared to non aged ones (P = 0.018). 36.2% of patients had relapsed. Relapses were significantly more frequently observed only in the presence of mucosal involvement at presentation (P = 0.015). The median overall 1st relapse-free survival was of 2.33 years. Only mucosal involvement at presentation was associated with a shorter median 1st relapse-free survival time (1.28 years vs. 3 years) (P = 0.0017). Mortality rate was of 10.6% (n = 5); in four patients the death was directly related to pemphigus and occurred rapidly after the onset of lesions. CONCLUSION: Our study illustrates the poor prognosis of pemphigus by a long duration to disease control, a high initial dose of oral steroid, a high rate of relapse and a short remission period. Only mucosal involvement at presentation was identified as a poor prognostic factor.

[Spontaneous uterine rupture after myomectomy. Case report and review of the literature]. / Rupture utérine spontanée après myomectomie. À propos d'un cas et revue de la littérature.

Uterine rupture after myomectomy is rare but serious. It can occur before onset of labor and compromise vital maternofoetal outcome. We report the case of a uterine rupture at 25-week gestation in a 38-year-old primiparous woman after myomectomy and we will present a review of the literature concerning obstetric outcome after myomectomy.

Cutaneous metastases from internal cancers.

BACKGROUND: Cutaneous metastases (CMs) from carcinoma are relatively uncommon, being usually late events in the course of internal malignancies. CMs from internal tumors seen at a secondary dermatological centre in Tunis are reported. METHODS: A retrospective review of the available clinical records and histological material of all patients with CMs from internal malignancies was performed over a 14-year period. RESULTS: Fourteen cases of CMs were recorded: 8 males and 6 females. The age range was 53 to 96 years, with a mean of 63.5 years for males and 76.5 years for females. CMs represented the first indication of an internal malignancy in 64% of cases. Firm solitary or multiple nodules were the most relevant clinical presentation (93%). Preferred sites were the thorax (50%), the abdomen (29%), and the head and neck region (21%). The common sites of primary malignancy were the lungs (38%) in males and breast (33%) in females. Four out of 14 patients died within a short follow-up period (mean 9.5 months). DISCUSSION: CMs are relatively rare in patients with internal cancers, but they may be a presenting sign of such cancers.