[Temperament and alcoholic behavior in medical students]. / Tempérament et conduites alcooliques chez l'étudiant en médecine.

BACKGROUND AND AIMS: The Tunisian medical student population is very heterogeneous. This heterogeneity is based among other things on temperamental differences between different students and their behavior and strategies to cope with the requirements and constraints of medical studies. We conducted this study aiming: to determine the prevalence of alcohol use in a student population at Sfax Medical School and to evaluate the factors associated with this use; to study the relationship between affective temperament and alcohol consumption among medical students. METHODS: We conducted a descriptive and analytical cross-sectional study in the form of a survey between July 2018 and January 2019 among students (aged between 19 and 30) recruited from the University of Medicine of Sfax during their internship in the psychiatric department "A" at CHU HédiChaker of Sfax. We used a pre-established survey sheet based on data from the substance use and affective temperament literature in medical students that was completed by the participants after obtaining their consent and having explained the anonymity and confidentiality of the questionnaire. This sheet included a section on student sociodemographic characteristics, a second part describing the history and characteristics of alcohol consumption and the evaluation of alcohol consumption through the Alcohol Use Disorders Identification Test (AUDIT), and a third part for temperament evaluation by TIME-A, and Temperament Evaluation of Memphis Pisa Paris and San Diego Auto-questionnaire. RESULTS: We recruited 136 students with an average age of 21.63 years (SD 2 years) and a sex ratio (H/F) of 0.66. Alcohol consumption was observed among 19.8 % of students surveyed, 19.1 % of whom were occasional consumers. The mean age of onset of alcohol consumption was 18.48 years (SD 1.76 years). All consumer students had started using alcohol with their friends. The desired effect was euphoric in 74 % of cases, sleeping for 14.8 % of them. Among consumers, the average score at AUDIT was 6.44 (SD 5.3). Among them 70.4 % had a risk-free consumption (score of 8 or less); 14.8% were at risk (score 9-12) and 11.1 % were likely alcohol dependent (score>12). The evaluation of affective temperament according to TIME-A showed that the cyclothymic and hyperthymic temperament scores were the highest among the students (respectively 5.5 and 4). The analytical study showed that alcohol dependence was significantly more common among male students (p=0.048). Alcohol use was significantly more common among students who smoke (p<0.001) and those who consume cannabis (p<0.001). The bivariate correlation showed that more students had a high score of depressive temperaments (r=0.18, p=0.032), irritable (r=0.2, P=0.018) and hyperthymic (r=0.27, p=0.001). The higher their scores on the AUDIT scale, the more alcohol-dependent they were. CONCLUSION: The implementation of preventive measures is an obvious emergency. In the same way, taking into account the difference in affective temperaments in this very particular population could have a grandiose importance and an interesting impact both in the screening and in the care of these students.

[Depression and coping strategies in the elderly with type 2 diabetes]. / Dépression et stratégies de coping chez les sujets âgés atteints de diabète de type 2.

INTRODUCTION AND OBJECTIVES: Elderly patients with diabetes have been shown to have more diabetes-related complications, and they are more likely to develop somatic and psychiatric comorbidities including cognitive dysfunction and depression. Several studies have shown a close association between diabetes and depression. This comorbidity may lead to functional disability and quality of life deterioration. Thus, the elderly will face various constraints through the coping strategies. In this context, we conducted our study to assess the prevalence of depressive symptoms in elderly patients with diabetes as well as its associated factors, and to investigate their coping strategies. METHODS: We conducted a cross-sectional, descriptive and analytic study among 50 elderly patients (age≥65 years) being followed for type 2 diabetes at the outpatient department for chronic diseases of the Regional Hospital of Aguereb, Sfax, Tunisia. We used the "Activity of Daily Living" to assess the dependence level, the "Geriatric Depression Scale" to screen for depressive symptoms, and the "Brief Coping with Problems Experienced" to investigate the coping strategies. RESULTS: The mean age of patients was 73.3 years, with a sex-ratio (M/F) of 0.62. Smoking and alcohol consumption were reported respectively in 20% and 4% of participants. The mean duration of diabetes was 7.7 years. Diabetes complications were noted in 70% of participants. Somatic comorbidities were noted in 94% of cases (hypertension 84%; dyslipidemia 34%). Psychiatric histories were reported in 18% of patients who suffered from anxio-depressive symptoms. No patient among those with mental disorder histories benefited from any psychiatric management prior to the study. Three patients (6%) had previously presented suicidal ideations but none of them had attempted suicide. The mean "Activity of Daily Living" score was 4.9 points. Patients were autonomous in 28%, and dependent in 4% of cases. The mean "Geriatric Depression Scale" score was 9.8 points. According to this scale, the prevalence of depressive symptoms was 34%. They were correlated with: smoking (P=0.04), psychiatric histories (P=0.031), absence of leisure activity (P=0.035), "Activity of Daily Living" score (P=0.028), long duration of diabetes (P=0.04) and the presence of suicidal ideation (P=0.013). According to the « Brief Coping with Problems Experienced ¼, the problem-focused coping strategies were the most frequently used (44%), followed by emotion-focused (38%) and passive strategies (18%). Participants with depressive symptoms are significantly more likely to adopt emotion-focused coping strategies (P=0.01). CONCLUSION: Our study highlighted a high prevalence of depressive symptoms among elderly patients with diabetes. This relationship seems to be bi-directional and may increase somatic complications and alter the quality of life, and then darken the prognosis. Thus, besides pharmacological treatment, regular depression screening and psychological support are essential to ensure a better control of diabetes and to improve well-being.

[Post-partum depressive symptoms: Prevalence, risk factors and relationship with quality of life]. / Symptomatologie dépressive du post-partum : prévalence, facteurs de risque et lien avec la qualité de vie.

OBJECTIVE: The objective of our study was to estimate the prevalence of the post-partum depressive symptomatology in a sample of Tunisian women, to study associated factors and to assess its relationship to quality of life. PATIENTS AND METHODS: This is a prospective study carried out in two stages: during the first week (T1), then between sixth and eighth week post-partum (T2). Depressive symptomatology and quality of life were assessed respectively by the Edinburgh Postnatal Depression Scale and the World Health Organization Quality of Life scale. RESULTS: In the first stage, the prevalence of depressive symptomatology in the total sample (150 women) was 14.7% and was related to age above 35 years, low school level, personal psychiatric history, multiparity, caesarean delivery or forceps in the previous pregnancy and unplanned pregnancy. This prevalence was 19.8% among the 126 women reviewed in T2 and was correlated with the exaggerated sympathetic signs during pregnancy, namely perversion of taste and fatigue. Quality of life was strongly correlated with depressive symptoms in T1 and T2. CONCLUSION: Post-partum depressive symptoms were common in our sample and were correlated with quality of life. Therapeutic measures should be proposed for women with post-partum depressive symptoms and particularly with several risk factors in order to improve their quality of life.

[Reticence towards electroconvulsive therapy: a study of 120 caregivers in a teaching hospital in Tunisia]. / De la réticence face à l'électroconvulsivothérapie : enquête auprès de 120 personnels soignants dans un centre hospitalo-universitaire en Tunisie.

INTRODUCTION: Electroconvulsive therapy (ECT) is rarely practiced in Tunisia. We have tried to explain this lack of use by studying theoretical knowledge, the perception and the attitudes of health professionals towards ECT. METHODS: We conducted a survey in the region of Sfax in Tunisia. It included 120 participants. The latter were divided into four groups, composed of 30 members, in order to compare them: group 1 (psychiatrists); group 2 (neurologists and anaesthetists); group 3 (psychiatric nurses); group 4 (paramedics in the neurological and anaesthesia wards). The surveyed people answered an auto-questionnaire including essentially 16 items: 11 had binary answers (true or false) related to theoretical knowledge about ECT, and five others explored the perception and attitudes concerning this therapy. RESULTS: Concerning the items exploring theoretical knowledge, 67.5% of people were not able to answer, in conformity with the consensual scientific data, a minimum of 75% questions. The rates were significantly lower among the paramedics (P<0.001) and those who work outside the psychiatric wards (P=0.003). The answers of psychiatric caregivers were less frequently incorrect compared to those of non psychiatric ward workers. The latter more frequently thought that the psychiatrists easily resorted to ECT (P=0.003). The paramedics perceived, more frequently than doctors, the ECT as a violent therapeutic means (P=0.001), and more frequently refused to give their consent to apply ECT to a relative (P=0.044). The rate of doctors who refused to give their consent to apply ECT to a relative was statistically higher in group 2 than in group 1 (P=0.017). Doctors with higher scores in theoretical knowledge, consent more frequently to apply ECT to a member of their families (P=0.001). DISCUSSION: The comparison of theoretical knowledge between the two groups of doctors (group 1 versus group 2) revealed significantly more frequent incorrect answers among non-psychiatrists. The latter more frequently had erroneous ideas: that ECT was performed without anaesthesia; that it wasn't possible to apply it to pregnant women, nor to people suffering from Parkinson's disease, nor as a preventive treatment of recurrence, and that the vital risk was higher with ECT than medical treatments. Compared to the caregivers of group 3, group 4 had significantly more frequent negative attitudes toward ECT; concerning the application without anaesthesia, the lack of tolerance and the over-mortality rate related to ECT. This therapy seems to create an apprehension and a reticence among health professionals, mainly the paramedics and the non-psychiatrists. The lack of valid scientific knowledge concerning ECT allows prejudiced ideas to tarnish the image of this therapy. CONCLUSION: Information and training would play a primordial role in the improvement of the perception and attitudes concerning ECT, and lower the reticence towards this therapy, which in spite of its proven efficacy, always maintains a pejorative image.

[Multiple arterial thrombosis in Behçet's disease]. / Thromboses artérielles multiples au cours de la maladie de Behçet.

Behcet's disease (BD) is a multisystemic vasculitis. Its etiopathogeny remains unknown. Vascular involvement in BD is frequent and venous thrombosis is the most common manifestation (30% of cases). Arterial involvement is rare (2.7 to 7%). The latter is often severe and considered as a life threatening complication. Pathogenesis of thrombosis occurring in BD remains unclear. We report a 45-year-old man, from south of Tunisia, who presented a BD with a bifocal arterial involvement: right internal carotid thrombosis and bilateral proximal thrombosis of the two pulmonary arteries. Therapeutic strategies to address this multiple arterial involvement and the pathogenesis of thrombosis raise many questions.

Tunisian hereditary spastic paraplegias: clinical variability supported by genetic heterogeneity.

Hereditary spastic paraplegias (HSP) constitute a clinically and genetically heterogeneous group of neurodegenerative disorders characterized by slowly progressive spasticity of the lower extremities. We performed the first clinical, epidemiological and genetic study of HSP in Southern Tunisia. We investigated 88 patients belonging to 38 unrelated Tunisian HSP families. We could establish the minimal prevalence of HSP in the district of Sfax at 5.75/100,000. Thirty-one percent of the families had a pure HSP, whereas 69% had a complicated form. The mode of inheritance was almost exclusively compatible with an autosomal recessive trait (97%, 37/38). Taking into account previously published results and new data generated in this work, genetic studies revealed significant or putative linkage to known HSP loci in 13 families (34.2%) to either SPG11 (7/38, 18.4%), SPG15 (4/38, 10.5%) or to SPG4 and SPG5 in one family each. The linkage results could be validated through the identification of two recurrent truncating mutations (R2034X and M245VfsX246) in the SPG11 gene, three different mutations (Q493X, F683LfsX685 and the novel S2004T/r.?) in the SPG15 gene, the recurrent R499C mutation in the SPG4 gene as well as the new R112X mutation in the SPG5 gene. SPG11 and SPG15 are the major responsible HSP genes in Tunisia.

[Cardiac involvement in Steinert myotonic dystrophy]. / Les anomalies cardiaques au cours de la dystrophie myotonique de Steinert.

INTRODUCTION: Myotonic dystrophy type 1 (DM1) is an autosomal dominant multisystemic disease with frequent cardiac involvement that may cause sudden death. This study was performed to determine the various cardiac manifestations in DM1, their frequency and the relevance of cardiac electrophysiological study in this disease. METHODS: Ten patients with DM1, five men and five women, mean age 44.3+/-7.8 years underwent neurological and cardiac assessments. RESULTS: The most frequent electrocardiographic findings were conduction abnormalities, essentially by intraventricular conduction defects (eight out of ten cases) such as bundle branch or fascicular blocks. Echocardiography showed alterations in systolic left ventricular function in two cases. Invasive electrophysiology testing showed sub-hisien block in three patients, requiring cardiac pacemaker implantation. These three patients had normal duration of PR interval and normal width of QRS complex. CONCLUSIONS: We recommend that all patients with DM1 should undergo cardiac investigation to detect subclinical cardiac involvement.

[Relevance of plasma exchange in the treatment of myasthenia gravis: study of 11 cases]. / Apport des échanges plasmatiques dans le traitement de la myasthénie: étude de 11 cas.

BACKGROUND: The use of plasma exchange (PE) constituted an advance in the treatment of myasthenia. The objective of our study was to determine the relevance of PE in the treatment of myasthenia and to study the different complications which can be observed during PE. PATIENTS AND METHODS: We studied retrospectively 11 patients who have generalized myasthenia and underwent PE. We used an intermittent flow cell separator and we performed PE three times a week. Biological assessment was performed before and after PE for all patients. The exchange volume was calculated according to the patient weight, gender and the value of hematocrit. RESULTS: Our series included six women and five men. The mean age at onset of the disease was 41.4+/-14.1 years (range: 18 to 68). Indication of PE was myasthenia crisis (eight cases), resistance to classic treatment (two cases) and exacerbation after thymectomy (one case). An improvement was observed rapidly in five cases and delayed in three cases. The remaining three patients did not improve. The most frequent side effects of PE were hypotension (four cases), heart arrhythmia (two cases) and hypoglycemia (one case). Three patients dead in the seven days after the first PE. CONCLUSION: PE represents an interesting tool to treat severe forms of myasthenia and improve prognosis. High incidence of complications in our series can be explained by the initial disease severity, the used method of PE, the existence of associated illness, and a long stay in intensive care unit.

Combining WMN and FOCUSS recursive approach to estimating the current density distribution in the brain.

In this paper we present a new approach which combines the two methods of cerebral electric activity's localization: "Weighted Minimum Norm" (WMN) and the iterative method "FOCal Underdetermined System Solver" (FOCUSS). Our idea is to use the current density distribution estimated by the WMN method in order to initialize the weighting matrix necessary for the localization with FOCUSS method. We compare the found results with those of the traditional WMN and FOCUSS methods in term of computing time and resolution matrix. The presented results show that our approach gives a good localization of the active sources in the brain.

[Familial spastic paraplegia with severe amyotrophy of the hands. (Silver syndrome?)]. / Paraplégie spastique familiale avec amyotrophie sévère des mains (syndrome de Silver?).

Familial spastic paraplegia (FSP) with severe muscular atrophy of hands and feet is exceptional. Autosomal dominant forms were initially described by Silver in 1966. We report two cases, from the same Tunisian family, presenting FSP with severe amyotrophy of the hands. A brother and his sister, aged respectively 37 and 36 years old, presented practically the same clinical picture. Their parents were cousins. The female patient was hospitalized. Both patients developed gait disorders around the age of three years. Muscular atrophy of the hands arose much later, around the age of 20 years. The neurological examination disclosed a spastic gait with distal amyotrophy, severe in the hands and moderate in the feet. Sensitivity was preserved and there was no fasciculation. The spinal cord and cerebral MRI was normal. Electromyography (EMG) showed a neurogenic pattern in the distal muscles. Stimulation of the median, ulnar and sciatica nerves was ineffective. The somatosensory evoked potentials (EP) were delayed (upper limb) or desynchronised (lower limb). The auditory and visual EP were normal. The cerebrospinal fluid contained 1 mononuclear cell/mm3 and 10 mg protein/100 ml. Abnormalities of the cranio-vertebral junction, Arnold-Chiari malformation, syringomyelia and familial juvenile amyotrophic lateral sclerosis (ALS) were excluded and the diagnosis of Silver's syndrome was evoked.

Polysaccharide storage myopathy--case report and literature review.

Polysaccharide myopathy is a rare form of storage muscular disorder. The clinical picture of this particular form of myopathy is unspecific. We report a 62-year-old woman with late-onset progressive weakness and wasting, affecting proximal muscles of the four limbs and the girdles. No myalgia, dysphagia nor symptoms of cardiac failure were observed. Muscle biopsy revealed a vacuolar myopathy with accumulation of amylopectin-like polysaccharide. This material was strongly PAS-positive and diastase-resistant. At electron microscopy, the deposits were composed of non-membrane-bound filamentous and granular material surrounded by numerous mitochondria. No enzyme deficiency was found. Clinical presentation of our patient was similar to the 16 cases reported in the literature. She did not have myocardiopathy and her survival is much longer. Hypothetic mechanisms of polysaccharide accumulation are reviewed.

[Subacute myelitis revealed by human immunodeficiency virus infection]. / Myélite subaiguë révélatrice d'une infection par le virus de l'immunodéficience humaine.

A 35 year-old heterosexual man had a six months history of cervical myelitis with progressive paraplegia, leg weakness and paresthesia of the four extremities. Spinal cord MRI showed a high T2 signal intramedullary lesion wide from the bulbo-medullary junction to D4. Post gadolinium T1 sequence revealed an enhancement in front of C3-C4 vertebrae. VIH serology was positive. Corticosteroid treatment achieved a marked improvement. In addition to vacuolar myelopathy, well-known at the advanced stages of the HIV infection (AIDS), myelitis and clinical pictures simulating multiple sclerosis were described during early stages of the infection. These inflammatory lesions of the central nervous system and sometimes of the peripheral nervous system seems to be related to the immune response dysfunction induced by the VIH.

Correlation between nerve conduction studies and clinical scores in diabetic neuropathy.

Polyneuropathy, a frequent complication of diabetes, can be assessed clinically and electrophysiologically. Neurological examination can be quantified by validated scores, e.g., the neuropathy symptom score (NSS) or the neuropathy disability score (NDS). Such scores exclude electrophysiological aspects of the neuropathy. A software tool was designed to convert electrophysiological data into one single index of polyneuropathy (IPN). This index was calibrated to grade the severity of a polyneuropathy from 0.00 to 1.00. In a series of 38 diabetic patients, we have calculated NSS, NDS, and IPN. We found correlations between these variables, NDS and IPN exhibiting the more significant association. The use of IPN allowed us to demonstrate that nerve conduction values correlated with clinical scores in diabetic polyneuropathy. Such a software tool, by providing a single electrophysiological index, may facilitate clinico-electrophysiological assessment in large descriptive studies or therapeutic trials of diabetic polyneuropathy.

[Clinical, biological and genetic study of 24 patients with ataxia telangiectasia from southern Tunisia]. / L'ataxie-télangiectasie. Etude clinique, biologique et génétique de 24 cas du sud Tunisien.

Ataxia telangiectasia is a multisystem disease with an autosomal recessive inheritance. It is characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, humoral and cellular immunodeficiencies and high incidence of neoplasia and radiosensitivity. A 5 year retrospective survey included 24 patients belonging to 17 families. Cerebellar ataxia was the first clinical symptom and was usually noticed when the child began to walk. Mean age of onset was 2.9+/-1.8 years. Oculocutaneous telangiectasia was present in 17 cases and appeared between 2 and 8 years and then spread in a characteristic symmetrical pattern. When ocular telangiectasia was absent (6 cases), the diagnostic of ataxia telangiectasia was retained on oculomotor apraxia (2 cases), recurrent sinopulmonary infections (3 cases) and/or a sib with typical ataxia telangiectasia (1 case). Recurrent sinopulmonary infections, absence or low serum level of IgA (78 p.100) and lymphopenia revealed immunodeficiency. Among 12 patients, chromosomal instability was observed in 5. Balanced rearrangements involving chromosomes 2, 7, 14, 22, 1, 3 and 11. The responsible gene, ATM, encodes a large protein kinase with a phosphatidylinositol 3-kinase-like domain. Ataxia telangiectasia patients have a 100 fold higher risk of cancer than the general population. We reported, in the same family two patients who developed neoplasia, (lymphoma and leukemia). During follow-up, a progressive worsening was observed in all cases. Three patients have died.

A new locus for autosomal dominant pure spastic paraplegia, on chromosome 2q24-q34.

Hereditary spastic paraplegia (HSP) comprises a group of clinically and genetically heterogeneous disorders causing progressive spasticity and weakness of the lower limbs. We report a large family of French descent with autosomal dominant pure HSP. We excluded genetic linkage to the known loci causing HSP and performed a genomewide search. We found evidence for linkage of the disorder to polymorphic markers on chromosome 2q24-q34: a maximum LOD score of 3. 03 was obtained for marker D2S2318. By comparison with families having linkage to the major locus of pure autosomal dominant HSP (SPG4 on chromosome 2p), there were significantly more patients without Babinski signs, with increased reflexes in the upper limbs, and with severe functional handicaps.

Spectrum of SPG4 mutations in autosomal dominant spastic paraplegia.

Autosomal dominant hereditary spastic paraplegia (AD-HSP) is a group of genetically heterogeneous neurodegenerative disorders characterized by pro- gressive spasticity of the lower limbs. Five AD-HSP loci have been mapped to chromosomes 14q, 2p, 15q, 8q and 12q. The SPG4 locus at 2p21-p22 has been shown to account for approximately 40% of all AD-HSP families. SPG4 encoding spastin, a putative nuclear AAA protein, has recently been identified. Here, sequence analysis of the 17 exons of SPG4 in 87 unrelated AD-HSP patients has resulted in the detection of 34 novel mutations. These SPG4 mutations are scattered along the coding region of the gene and include all types of DNA modification including missense (28%), nonsense (15%) and splice site point (26.5%) mutations as well as deletions (23%) and insertions (7.5%). The clinical analysis of the 238 mutation carriers revealed a high proportion of both asymptomatic carriers (14/238) and patients unaware of symptoms (45/238), and permitted the redefinition of this frequent form of AD-HSP.

Frequency of the DYT1 mutation in primary torsion dystonia without family history.

BACKGROUND: Idiopathic torsion dystonia is a clinically and genetically heterogeneous movement disorder. A GAG deletion at position 946 of the DYT1 gene was the first mutation found, in early-onset dystonia, with an autosomal dominant transmission and reduced penetrance. OBJECTIVE: To evaluate the frequency of the DYT1 mutation in patients with idiopathic torsion dystonia but without a family history. DESIGN: Prospective cohort study. SETTING: Four botulinum toxin clinics in the Paris, France, area. PATIENTS: A French population of 100 patients with dystonia. MAIN OUTCOME: Frequency of the DYT1 mutation tested by polymerase chain reaction and enzyme restriction analysis for the 946 GAG deletion, and genotype-to-phenotype correlation. RESULTS: Only 5 mutation carriers were identified, 4 of whom were part of a group of 10 patients with generalized dystonia. Onset was between ages 5 and 12 years as in typical early-onset dystonia. All 4 patients had cranial muscle involvement, which is atypical for DYT1 mutation carriers. One had segmental dystonia. Molecular analysis of relatives in 2 families demonstrated that the lack of family history was due to reduced penetrance. CONCLUSIONS: For accurate diagnosis and genetic counseling, screening for the DYT1 deletion is of great interest in cases with generalized dystonia without a family history. In other cases, positive results are rare.