[Clinical and laboratory features of recessive Limb Girdle Muscular dystrophies in the Department Neurology of University Hospital of Point G]. / Aspects Cliniques et Paracliniques des Myopathies des Ceintures Récessives au Service de Neurologie du CHU Point G.

INTRODUCTION: Limb-Girdle Muscular dystrophies (LGMD) is a group of inherited diseases characterized by predominantly proximal and limb muscle weakness. These are rare diseases that have not been well studied in sub-saharan Africa. The aim of our was the clinical and paraclinical characterization of patients with recessive LGMD at the Department of Neurology of the Teaching Hospital of Point G. PATIENTS AND METHODS: We conducted a longitudinal prospective study which took place from March 2014 to May 2019. Patients with recessive LGMD phenotype were enrolled. Sociodemographic, clinical and laboratory data were analyzed. RESULTS: We enrolled 46 families (67 patients), i.e. a frequency of 16.7% among the neurodegenerative diseases seen in the service. Among them, 45.6% came from the Sikasso region. Autosomal recessive inheritance pattern was suspected in 67.4% of the families. Symptoms appeared mainly in the first decade of life. Proximal muscle weakness was found in almost all patients. Cardiac examination showed dilated cardiomyopathy in 4.5% of cases. CONCLUSION: Limb-Girdle muscular dystrophy is a disabling disease that is found in Mali. Further study of these cases could elucidate the underlying genetic defects.

Network visualization for outbreak response: Mapping the Ebola Virus Disease (EVD) chains of transmission in N'Zérékoré, Guinea.

BACKGROUND: During the 2014-2015 Ebola Virus Disease (EVD) outbreak in N'Zérékoré, Forested Guinea, modes of transmission remained unexamined for a number of new cases. We used network visualization to investigate EVD transmission chains (TC) in seven sub-prefectures of N'Zérékoré in order to adapt outbreak response. METHODS: Between August 2014 and February 2015, the EVD outbreak response team including the World Health Organization (WHO) and local health authorities routinely collected information among new cases regarding hospital visits, cases within a household, participation in burials, as well as dates of symptom onset, serial intervals (SI) and exposure to EVD. SI's were defined as the interval between symptom onset in an index case and symptom onset in a secondary case infected by that index case. Cases who reported hospital visits, contact with a case in the household or participating in burials were attributed to these exposures. RESULTS: We identified seven TC (two urban and five rural) gathering characteristics of 109 probable/confirmed cases. Overall, 61% (66 cases, SI range: 7-20 days) were household related, 32% (35 cases, SI range 8-30 days) were household or burial related and 7% (8 cases, SI range: 4-20 days) were hospital-related. In the urban chains (18 cases, SI range: 7-20 days), 12 cases were household related and 6 cases were hospital related, none where household or burial related. In the rural chains (84 cases, SI range: 7-30 days), 60% (50 cases) were household related, 1% (1 case) was hospital related and 39% (34 cases) were household or burial related. No cases reported multiple exposures. CONCLUSIONS: Network visualization during field response is crucial in enhancing local control strategies, refining outbreak response and aiding rapid response teams in insuring psychosocial and socio-economic recovery. Urban settings need to focus on reducing hospital EVD transmission whereas rural settings should focus on raising awareness of transmission within a household and safeguarding EVD burials.

[Neurological disorders related to vitamin B12 deficiency in prisons in Guinea: a 22-case study]. / Manifestations neurologiques associées à la carence en vitamine B12 en milieu carcéral en Guinée : étude de 22 observations.

Neurological disorders related to vitamin B12 deficiency are common in prisons of tropical Africa. We collected 22 cases (20 men and 2 women). They all showed vitamin B12 deficiency associated with neurological signs that were represented by sclerosis combined with bone marrow (n = 9), peripheral neuropathy (n = 6), cerebellar syndrome (n = 2), a pyramidal syndrome of the lower limbs (n = 4) and optic neuropathy (n = 1). Laboratory tests showed a mean hemoglobin concentration of 7.2 ± 1.5 g/dl, mean 104 ± 28 fl, macrocytic anemia in 10 patients. Biermer's disease was identified in 9 patients, 3 patients showed the syndrome of non dissociation of vitamin B12, a gastrectomy in 2 patients and no etiology was identified in 8 patients.

[Clinical and tomographic aspects of 29 cases of phakomatosis in Guinea]. / Aspects clinique et scannographique de 29 observations de phacomatoses en Guinée.

The purpose of this report is to describe 29 cases of phakomatosis including 18 cases of tuberous sclerosis (Bourneville) and 11 cases of neurofibromatosis (von Recklinghausen) observed over a 10-year period at the Neurology Department of the University Hospital Centre in Conakry, Guinea. Findings during this period were consistent with those classically reported in the literature: high frequency of advanced skin lesions coalescing into massive tumours, occurrence of seizures of all types and development of a wide variety of complications as a result of late diagnosis. Our experience underscores the need for follow-up and surveillance of these patients by somatic studies based on neurological, ophthalmologic and tomographic data depending on clinical findings.

[Permanent disability and the insurance estimation process]. / Estimation de l'incapacite partielle permanente et du pretium doloris dans le cadre judiciaire et dans le cadre des procedures conseil d'assurances.

INTRODUCTION: The casualties are indemnified according to two processes. First by transaction on rate proposition of insurance physicians, and the second process on rate proposition by a medical expert assigned by law-court. Indemnification scale failure justifies the Interafrican Conference of insurance Markets code adoption. MATERIAL AND METHOD: Six insurance societies and the Automotive Guarantee Fund were debtors. Only 627 victims had been indemnified between 1986 and 2003. Expert valuations done at forensic medicine service were the support of the investigation. Inquired parameters were insurance societies, regulation type, aftermaths and the retained prejudices. The data collected on computer card have been analyzed by software Epi Info. RESULTS: The partial permanent inabilities fixed since its adoption differ to inabilities fixed before this adoption. Transaction process concerned 567 victims (90.4%). Sixty victims were indemnified by judicial way. According to process type, the rates fixed in judicial process were 61.6% middle permanent partial inabilities. After 1997, there have observed a decrease in the high and middle permanent partial inabilities in the two processes. The appreciation of the pretium doloris is more subjective but must repair the aftermaths. The middle pretium was majority in the two processes, before and after 1997 with a high decrease of the middle pretium in the transaction process (-15.07) and a small pretium increase of 10.98 points. CONCLUSION: A common scale code has decreased the judicial litigation concerning casualties in spite of scales' limits. Only the patients with important aftermaths arrive in the judicial process since 1997.

Aspects clinique et scannographique de 29 observations de phacomatoses en Guinee

Les auteurs rapportent 29 observations de phacomatoses dont 18 cas de sclerose tubereuse de Bourneville et 11 cas de neurofibromatose de Recklinghausen; observees dans le service de neuro l ogie du centre hospitalo-universitaire de Conakry; sur une periode de 10 ans. Dans cette peri o d e;les donnees classiques de la litterature sont retrouvees : frequence elevee des lesions dermatologiques evoluees atteignant de volumineuses tumeurs royales; des crises epileptiques sous toutes ses formes et les complica-tions diverses en raison du retard du diagnostic. Nos resultats soulignent la necessite de suivi et de surveillance de ces patients par un examen somatique (neurologique; ophtal- mologique) et un examen scannographique dicte par les donnees cliniques

[Sexual child abuse: correlation between medical certificates' conclusions and judiciary sanctions]. / Sevices sexuels sur les enfants: correlations entre les conclusions medicales et les peines prononcees.

Sexual child abuse, comprises of indecency attitudes and physical misbehaviours, directed towards children are dominated by rape. The objective of our study was to assess in sexual child abuse the relation between the conclusion of medical certificates and court decision. It is a retrospective study carried out from 1994 to 1998 on the clerk's office correctional repertories in Dakar regional court. An overall number of 79 cases of child abuse were collected in 5 years period. Children under 18 years old of of both sex, were concerned. Data found were correlated with a review of requisition cases received by the of gynaecology and obstetrics clinic of Aristide Dantec Hospital. This facilitates the establishement of the relationship between the offences and the pronounced sanctions, as well as the initial medical certificate and these sanctions. The sanctions were severe whenever rape had been retained. Some cases were disqualified in indecent assault and were judged as such. The judge decision, which follow the medical certificate conclusions in 11 cases out of 14 shows the importance and reliability of this medical document. All files reviewed at the medical and legal level were incomplete. The difficulty of the materiality of the rape and the psychological consequences in the long run and especially HIV infection should invite to a multidisciplinary, specialized and organized management of sexual child abuse. This study has shown the importance of a correct and complete drafting of the medical certificate, to enable the establishment by the judge the materiality of the facts.

[Comparative review of the Senegalese and French deontology codes]. / Revue comparative des codes de déontologie sénégalais et français.

The medical deontology regroups duties of the physicians and regulate the exercise of medicine. The code of medical deontology of Senegal inspired of the French medical deontology code, has not been revised since its institution whereas the French deontology code knew three revisions. Comparing the two codes of deontology titles by title and article by article, this work beyond a parallel between the two codes puts in inscription the progress in bioethics that are to the basis of the revisions of the French medical deontology code. This article will permit an advocacy of the health professionals, in favor of a setting to level of the of Senegalese medical deontology code. Because legal litigation, that is important in the developed countries, intensify in our developing countries. It is inherent to the technological progress and to the awareness of the patients of their rights.

[Impact of snakebites in rural environment: community survey in the rural development community (DRC) of Frilguiagbe, Republic of Guinea]. / Incidence des morsures de serpent: enquête communautaire dans la collectivité rurale de développement (CRD) de Frilguiagbe (République de Guinée).

An exhaustive study has been carried out in the rural development community (DRC) of Frilguiagbe, located at 115 km from Conakry in the Conakry-Kindia highway. It concerns a population of 23,442 inhabitants divided into 3,047 households. The survey was carried out at three levels:--community level;--health centres;--traditional healers. In the household survey made between 2002 and 2003, 175 snakebites cases were recorded. In health centres, only two (2) cases were recorded from 1993 to 2003. On the other hand, 80% (=140) of the cases registered in the households have consulted a traditional healer. On the whole, there were 9 deaths (5%). Such a result shows that the collect of information from the consultation registers is not appropriate to appraise the importance of envenomations in our country.

[Problems with envenomations in Guinea]. / Problématique des envenimations en Guinée.

Only a few years after its creation, the Kindia Pasteur Institute (currently Institut de Recherche de Biologie Appliquée de Guinée, IRBAG), owing to its geographical situation, began regularly supplying the Paris Pasteur Institute with snake venom for manufacturing antivenom. The diversity of the biotopes in Guinea due mainly to the diversity of vegetation (savannah, forest, mangrove) and altitudes (coastal zone, mountains of Fouta-Djalon and mountain of High Guinea), induces a wide variety of the herpetological fauna. Envenomations are consequently a significant cause of morbidity and mortality in a country where agriculture is an important economic activity. Unfortunately, precise statistics for envenomations are unavailable. A retrospective study was performed based on IRBAG registers (1980-1990). A total of 584 snakebites were reported with a 2.2% case fatality rate. Between 1997 and 1999, 379 cases of snakebites were reported with 18.2% death, mainly due to the lack of antivenom, and 2.1% amputations. By combining the fang marks and victim or relative assessments, we were able to identify most of the snakes involved in the envenomations: Naja (21.3%), Bitis (15%), Dendroaspis (12.4%) and Causus (12.9%). In 38.2% of the cases, the snake was not identified. This survey confirmed that envenomations are a problem in Guinea. However, more studies are necessary to appreciate the real importance of the herpetological Guinean fauna and its consequences on morbidity and mortality in order to improve the management of envenomations.

[Atypical neurosyphilis: 28 cases observed at the University Hospital Center of Conakry]. / Neurosyphilis atypique etude de 28 cas observes au CHU de Conakry.

This study describes a series of 28 cases of neurosyphylis presenting atypical oligosymptomatic syndromes involving epileptic attack, chronic headache, peripheral facial paralysis, deafness, isolated optic atrophy, and cerebral ataxia. Diagnosis was based on positive results (VDRL-TPHA) of serological reactions in blood samples and analysis of cerebrospinal fluid showing pleocytosis (mean lymphocyte level, 69%) and elevated protein levels. Epilepsy and chronic headache were the most common clinical manifestations. Brain scan demonstrated low-grade ventricular dilatation associated with nonspecific cerebral atrophy in 8 cases and isolated cerebral atrophy in three. In the remaining 17 cases brain scans were normal.

[Malaria prevalence survey in 24 Guinean villages in the Kindia area]. / Etude de la prévalence du paludisme dans 24 villages de la Guinée.

The authors report the results of a survey of malaria prevalence carried out from October to December 1998 in 24 villages located upstream of the Garafiri dam, in the Kindia area. The parametre used for the assessment of prevalence was the Plasmodium index. The gametocyte and spleen indexes were also recorded. The prevalence observed (34,7%) confirms the presence in this area of stable malaria mostly transmitted by Anopheles gambiae in tropical Africa. This prevalence rate is compatible with the epidemiological data of the Ministry of Health.

Theophylline kinetics and ventilatory flow in bronchial asthma and chronic airflow obstruction: influence of erythromycin.

The kinetics and the effects on the ventilatory function peak expiratory flow rate (PEFR) of a single 600-mg oral dose of theophylline were investigated in 46 adult patients with bronchial asthma (BA) and in 16 adult patients with chronic airflow obstruction (CAO). In the former, theophylline induced an early and potent bronchodilatation (60% rise in PEFR), the kinetics of which correlated with plasma concentration. Theophylline was also effective in patients with CAO, but the magnitude of its bronchodilator effect was less than in those with BA: this was despite plasma concentrations of much the same order. In adult patients with BA (but not with CAO) theophylline plasma levels and bioavailability are higher after simultaneous erythromycin dosing.