Use and representations of intrauterine device in Martinique, F.W.I.: A cross-sectional survey.

INTRODUCTION: Abortion rate is higher in Martinique than in metropolitan France. Difference in pattern of contraception may be involved, particularly regarding IUD use. IUD use is often hampered by misconceptions. The aim of the study was to evaluate IUD use in a non-selected population in Martinique and to explore knowledge and acceptance of the method. MATERIAL AND METHODS: Women were submitted a self-questionnaire about their contraceptive history, appropriate candidates for, effectiveness, side effects and perceived risks of IUD. Acceptance of the method among men and women and reasons for rejecting it were also surveyed. We included 112 men and 136 women. Descriptive statistics and comparison of answers between ever and never IUD-users were realized. RESULTS: Past or current IUD use was found in 26.5% [19.3-34.8] of women and the method was known by 90.4% of women and 85.1% of men. Pain at insertion (important for 22%), infectious risk (increased for 23.6%), and eligible candidates for the method (possible in nulliparous for only 56.9%) were the main misconceptions recorded. Knowledge was better among ever than never IUD-users. Theoretical acceptance of the method was high (87.5% among women of reproductive age, 82.5% among men). Rejection of the method was mainly motivated by doubts on efficacy and fear from pain, whereas religious barriers were seldom set forward. DISCUSSION: IUD use seems comparable in Martinique and France but misconceptions are still common. Information may help diffusion of IUD in a targeted population at higher risk of unintended pregnancy.

High abortion rate in Martinique (F.W.I.) in spite of a large contraceptive availability. What are the determinants?

INTRODUCTION: Despite a similar health insurance coverage and access to contraception, Martinique F.W.I. has a higher rate of abortion than continental France (25.6 versus 15.3 per thousand respectively). The reasons are still largely unknown. A different contraceptive pattern, a lower use of emergency contraception in Martinique may be involved. We sought to explore the contraceptive pattern, to evaluate the use of emergency contraception and the reasons leading to an unwanted pregnancy among women seeking for an abortion. MATERIAL AND METHODS: An anonymous cross-sectional survey about the contraceptive use in the year and in the month before abortion, the use of emergency contraception and the reasons why, to patients' point of view, they became pregnant albeit not willing it was proposed to women seeking for an abortion in the dedicated unit of the university hospital of Martinique. RESULTS: Six hundred patients were included. A total of 83.7% of patients declared having a regular contraceptive use during the year preceding. Just before abortion, 61.1% of women had no contraception or a low reliable method (fertility awareness, withdrawal…). Lack of recognition of pregnancy risk was the main reason leading to abortion, accounting for 51.1% of cases, explaining a low use of emergency contraception (13.8%), whereas a difficult access to contraception was rarely quoted. Emergency contraception was used in 27.8% of relevant situations. CONCLUSION: Insufficient estimation of pregnancy risk is the main reason leading to unwanted pregnancy. Availability of emergency contraception does not reduce the risk.

Topiramate in childhood epileptic encephalopathy with continuous spike-waves during sleep: A retrospective study of 21 cases.

OBJECTIVE: Encephalopathy with continuous spike-wave during sleep (CSWS) is a particularly difficult-to-treat childhood epileptic syndrome. This study sought to present the EEG improvement and clinical efficacy of topiramate (TPM), a broad spectrum antiepileptic drug (AED), in a series of 21 children with CSWS encephalopathy. METHODS: We retrospectively reviewed the EEG results and clinical data of children with CSWS followed-up in our institution and treated with TPM. Sleep EEGs were performed 0-3 months prior to TPM introduction and then at 3 and 12 months. The exclusion criteria were (1) introduction of another AED and (2) withdrawal of a potentially aggravating AED during the first 3 months of treatment. In addition to spike index (SI), the severity of EEG abnormalities was rated using an original scale that also considered the spatial extent of interictal epileptiform discharges. RESULTS: 21 patients were included (18 males, 4-14y, three symptomatic cases). At 3 months, sleep EEG was improved in 14 and normalized in four (TPM doses: 2-5.5 mg/kg/day). Among these 18 patients, 16 manifested cognitive or behavioural improvement. In a subgroup of seven patients with frequent seizures, five became seizure-free and one had over 75% decrease in seizure frequency. At the one-year follow-up, 20 children were still on TPM and 10 exhibited persistent EEG improvement without any other AED being introduced, most of them with clinical benefits. CONCLUSION: TPM can decrease EEG abnormalities in epileptic encephalopathy with CSWS, achieving clinical improvement in the majority of patients. However, relapse may occur in the long-term in nearly half of cases. Otherwise, TPM has proven particularly useful in reducing seizure frequency in refractory cases.

[<< Early stimulation >> programs evaluation]. / Evaluation des programmes d' << intervention précoce >>.

Early intervention include educational and neuroprotection strategies. Early educational strategies are based on the cerebral plasticity concept. Neuroprotection, initially reserved for molecules preventing cell death phenomena, can be extended now to all actions promoting harmonious development and preventing handicaps, and include organisational, therapeutic and environmental aspects. Early stimulation programs have been first devised in United States for vulnerable children who belong to an unfavorable socio-economic category ; positive effects were recorded in school failure rates and social problems ; programs have also been launched in several countries for premature infants and infants with a low birth weight, population exposed to a high risk of deficiencies. The programs are targetted either to the child, or to the parents, or combined to provide assistance for both the child and the parents. The programs given the best evaluation are NIDCAP Program in Sweden (Newborn Individualized Developmental Care and Assessment Program), intended for babies < 1500 g in neonatal intensive care units, then a longitudinal, multisite program, known as IHDP (Infant Health and Development Program). It was launched in United States for infants < 37 weeks or < 2500 g. Results show that combined parent-child programs are the most useful. Effects on parent- child relationships and on child's cognitive development are especially effective if stimulation is maintained and when mothers have a low level of education.

The association between developmental handicaps and traumatic brain injury during pregnancy: an issue that deserves more systematic evaluation.

AIMS: Trauma during pregnancy is commonly viewed as benign for the foetus when the delivery occurs normally. This study revisits that point of view. METHOD: We included eighteen patients having a neurological handicap with an anamnesis of an accident during pregnancy and a follow-up sufficient to determine a definite outcome. RESULTS: Pregnancy outcome and observed management. Foetal abnormalities were detected in six cases between the first and the thirteenth day after the trauma. Emergency delivery or rapid birth after signs of foetal distress occurred in five cases. One baby died soon after birth. One-third of cases were not submitted to any investigation. VARIOUS NEUROLOGICAL HANDICAPS WERE RECORDED: Congenital microcephaly (three patients), congenital hydrocephalus (three), Infantile cerebral hemiplegy (six), quadriplegy with severe encephalopathy (four), diplegy (one), clumsiness with cerebellar atrophy (one), Moebius syndrome (one), mental retardation with autistic features (two), learning disability (one) auditory agnosia (one). Cerebral imaging showed macroscopic abnormalities in fourteen patients, evoking various pathogenetic hypotheses. CONCLUSION: The association between maternal trauma and foetal brain lesions lacks sufficient investigation in many cases. Prospective studies are needed to clarify both medical and legal issues. Guidelines are proposed for obstetrical and paediatric management after significant maternal trauma.

Delayed white matter injury in a murine model of shaken baby syndrome.

Shaken baby syndrome, a rotational acceleration injury, is most common between 3 and 6 months of age and causes death in about 10 to 40% of cases and permanent neurological abnormalities in survivors. We developed a mouse model of shaken baby syndrome to investigate the pathophysiological mechanisms underlying the brain damage. Eight-day-old mouse pups were shaken for 15 seconds on a rotating shaker. Animals were sacrificed at different ages after shaking and brains were processed for histology. In 31-day-old pups, mortality was 27%, and 75% of survivors had focal brain lesions consisting of hemorrhagic or cystic lesions of the periventricular white matter, corpus callosum, and brainstem and cerebellar white matter. Hemorrhagic lesions were evident from postnatal day 13, and cysts developed gradually between days 15 and 31. All shaken animals, with or without focal lesions, had thinning of the hemispheric white matter, which was significant on day 31 but not earlier. Fragmented DNA labeling revealed a significant increase in cell death in the periventricular white matter, on days 9 and 13. White matter damage was reduced by pre-treatment with the NMDA receptor antagonist MK-801. This study showed that shaking immature mice produced white matter injury mimicking several aspects of human shaken baby syndrome and provided evidence that excess release of glutamate plays a role in the pathophysiology of the lesions.

Adenylosuccinase deficiency: an unusual cause of early-onset epilepsy associated with acquired microcephaly.

Adenylosuccinase deficiency, an autosomal recessive inborn error of purine synthesis, was first described in 1984 by Jaeken and Van den Berghe (reviewed in J Inher Metab Dis 20;1997:193). The cardinal features are variable psychomotor delay often accompanied by epilepsy and autistic features. Diagnosis is made by detection of abnormal purine metabolites in body fluids. We report a girl who presented with early onset epilepsy, associated with acquired microcephaly and severe psychomotor retardation, as the most prominent symptoms.

Glycine antagonist and NO synthase inhibitor protect the developing mouse brain against neonatal excitotoxic lesions.

The prevention of cerebral palsy and neuroprotection of the immature brain continue to be health care priorities. The pathophysiology of perinatal brain lesions associated with cerebral palsy seems to be multifactorial and includes pre- and perinatal factors such as preconceptional events, hormone and growth factors deficiencies, maternal infections with production of cytokines, and hypoxic/ischemic perfusion failures. Excitotoxic cascade could represent a common pathway that leads to neural cell death and subsequent brain damage. Brain injuries induced by ibotenate, a glutamatergic analog, which are essentially mediated through the N-methyl-D-aspartate receptor, mimic some aspects of the white matter cysts and transcortical necrosis observed in human perinatal brain damage. The purpose of the present study was to assess the protective role of several pharmacological agents, administered in conjunction with ibotenate, against induced excitotoxic lesions. We injected ibotenate in the developing mouse brain 5 d postnatally, after the full settlement of neuronal layers. Co-treatment with kynurenic acid, an antagonist of the facilitating glycine site of the N-methyl-D-aspartate receptor, or with N(G)-nitro-L-arginine, an inhibitor of nitric oxide synthesis, induced a dose-dependent neuroprotective effect. Conversely, zinc gluconate, a blocking agent of the channel linked to the N-methyl-D-aspartate receptor, and a free radical scavenger (U74389F), were unable to protect the developing brain against excitotoxic attack. These data help to clarify some molecular mechanisms involved in excitotoxic lesions of the developing mouse brain and permit us to envision new strategies in the prevention of cerebral palsy.

[Prenatal diagnosis and outcome of patients with spina bifida]. / Le diagnostic prénatal et le devenir des patients atteints de spina bifida.

The frequency of new cases of myelomeningocele has largely decreased in the last 10 years in several countries of western Europe; the emergence of widespread prenatal diagnosis is probably one of the main reasons. However, the outcome of spina bifida which eludes the prenatal diagnosis, or in cases where termination was refused by parents, remains badly known. To our knowledge, there is no recent data on the outcome of children with spina bifida. Moreover, available data, depending on their European or American origin, indicate different functional outcomes. This led us to re-evaluate the mechanisms involved in the prenatal diagnosis of spina bifida and the outcome of our patients with spina bifida. The aim of this paper is to give a short description of the "rules and principles" of prenatal diagnosis as it is applied in our centres, as well as a brief overview of the outcome of 137 cases of spina bifida. In the retrospective study, three major aspects were evaluated: 1) frequency and types of surgeries and their benefits; 2) socio-psychological adaptation of the patients; 3) educational level and learning disabilities.

Whipple disease confined to the central nervous system in childhood.

In a case of pediatric Whipple disease confined to the central nervous system, white matter lesions initially appeared as areas of very low signal intensity on T1-weighted MR images and as areas of hyperintensity on proton density-weighted and T2-weighted images, and showed slight peripheral enhancement on delayed contrast-enhanced T1-weighted images. On MR studies obtained 3 and 6 months after antibiotic therapy, the lesions had decreased in size and no longer enhanced. They became progressively less hypointense on T1-weighted images and less hyperintense on T2-weighted images.

Outcome and prognosis of whiplash shaken infant syndrome; late consequences after a symptom-free interval.

Long-term follow-up five to 13 (mean seven years) of 13 cases of whiplash-shaken-infant syndrome (WSIS) demonstrated long sign-free intervals. Full clinical appearance of neurological deficits takes four months for the interruption of brain growth, six to 12 months for lesions of the central nervous system long pathways, up to two years for epilepsy, and three to six years for behavioural and neuropsychological signs. In our series, WSIS occurred at a mean postnatal age of 5.5 months and caused intracranial, retinal and preretinal haemorrhages, intracranial haematomas, oedema, contusional tears, and developmental disturbances interfering with the growth and differentiation of neural tissue and with synaptic stabilisation. These mechanisms account for the long sign-free interval that makes its impossible to formulate a precise and final neurological prognosis before the age of school entrance. Only one of our patients seems to have remained normal even several years after the shaking.

Prolonged dysphagia caused by congenital pharyngeal dysfunction.

We describe two patients with severe, isolated, congenital dysphagia caused by paralysis of the pharyngeal muscles, who recovered at the ages of 40 months and 20 months, respectively. No other evidence of neurologic or muscular dysfunction was present except for a transient paralysis of the adductors of the vocal cords in one child. Radiocinematographic studies showed paralysis of the pharyngeal stage of swallowing, with minimal involvement of the oral stage. One child refused oral feeding for several months after apparent radiologic recovery. Two other patients with a similar disorder died of tracheal aspiration at the ages of 8 months and 4 months, respectively. Autopsies showed no abnormality of the central nervous system, and the cranial nerves involved in swallowing were normal. Only five other well-studied cases of this syndrome have been reported. These observations demonstrate the existence of a type of severe, idiopathic, congenital dysphagia related to paralysis of the constrictor muscles of the pharynx, with a propensity to recover after several months or years if properly managed. The cause of the disorder is obscure, but it is probably related to a dysfunction of the central nervous system.

[Epilepsy disclosing neuroborreliosis]. / Epilepsie révélatrice d'une neuroborréliose.

After two seizures, a 13 year-old boy experienced headache, fatigue and loss of appetite over a period of 3 weeks. There was a bilateral papilledema with normal visual acuity. CT and MRI disclosed two ischemic foci, that were interpreted as evidence of vasculitis. High serum levels of IgG and IgM antibodies specific to Borrelia burgdorferi, were present. The patient had attended an outdoor scout camp in a area, in south-east Belgium, known to be endemic for tick-born borreliosis. The clinical symptoms, the levels of the specific antibodies and the radiologic abnormalities responded dramatically to treatment. We believe that seizures in this case were related to cerebral vasculitis. This case confirms the extreme diversity of the neurological manifestations of Borreliosis.

Leiomyoma of the suprarenal gland in a child with ataxia-telangiectasia.

We report the occurrence of a leiomyoma of the suprarenal gland in a 10-year-old girl with ataxia-telangiectasia (A-T). Muscle cell tumors are very uncommon in this gland as they are in A-T. Possible reasons for developing nonhematologic tumors in this syndrome are reviewed. A defect in DNA repair mechanisms probably favors, in young children, the expression of tumors normally expected in the aged.

Effects of changes in the periphery on development of the corticospinal motor system in the rat.

Effects of changes in the periphery on development of the corticospinal (CS) motor system were studied in the rat. Unilateral forelimb restraint between ages 5 and 30 days resulted in an increase in the number of CS neurons which persisted in the adult. The effect was most marked ipsilateral to limb restraint where both crossed and uncrossed CS connections were increased, but it also occurred to a lesser extent on the contralateral side. Animals with limb restraint had enlargement of the areas of cerebral cortex in which CS neurons occurred. The enlargement of motor cortex regions and increase in CS neurons closely resembled the changes found in the remaining cerebral hemisphere after neonatal hemispherectomy. The findings in animals with forelimb restraint differed markedly from those after forelimb amputation, where little change occurred in either number or location of CS neurons. Limb restraint initiated at the time of postnatal hemispherectomy had no effects on location or number of CS neurons beyond those of hemispherectomy alone. It is proposed that transient CS axons that occur normally in the postnatal rat may be recruited for formation of permanent connections under very diverse conditions, i.e. hemispherectomy and limb restraint. Failure to observe an additional effect of limb restraint in hemispherectomized animals may be due to the fact that after hemispherectomy all available transient fibers in the remaining hemisphere are recruited for innervation of the side of the spinal cord that has lost its cortical input.

Prenatal and early postnatal intoxication by inorganic mercury resulting from the maternal use of mercury containing soap.

A case of slight renal tubular dysfunction associated with cataract and anaemia was diagnosed in a 3-month-old black boy in whom high levels of mercury were found in blood and urine. Several arguments suggest that the renal, ocular and haematological defects may have resulted from exposure to mercury during foetal life and the 1-month lactation period due to the extensive use of inorganic mercury containing cosmetics by the mother.