Optimal Assessment and Quantification of Iodine Nutrition in Pregnancy and Lactation: Laboratory and Clinical Methods, Controversies and Future Directions.

Iodine intake must be boosted during pregnancy to meet the demands for increased production and placental transfer of thyroid hormone essential for optimal foetal development. Failure to meet this challenge results in irreversible brain damage, manifested in severity from neurological cretinism to minor or subtle deficits of intelligence and behavioural disorders. Attention is now being focused on explaining observational studies of an association between insufficient iodine intake during pregnancy and mild degrees of intellectual impairment in the offspring and confirming a cause and effect relationship with impaired maternal thyroid function. The current qualitative categorisation of iodine deficiency into mild, moderate and severe by the measurement of the median urinary iodine concentration (MUIC) in a population of school-age children, as a proxy measure of dietary iodine intake, is inappropriate for defining the degree or severity of gestational iodine deficiency and needs to be replaced. This review examines progress in analytical techniques for the measurement of urinary iodine concentration and the application of this technology to epidemiological studies of iodine deficiency with a focus on gestational iodine deficiency. We recommend that more precise definitions and measurements of gestational iodine deficiency, beyond a spot UIC, need to be developed. We review the evidence for hypothyroxinaemia as the cause of intrauterine foetal brain damage in gestational iodine deficiency and discuss the many unanswered questions, from which we propose that further clinical studies need to be designed to address the pathogenesis of neurodevelopmental impairments in the foetus and infant. Agreement on the testing instruments and standardization of processes and procedures for Intelligence Quotient (IQ) and psychomotor tests needs to be reached by investigators, so that valid comparisons can be made among studies of gestational iodine deficiency and neurocognitive outcomes. Finally, the timing, safety and the efficacy of prophylactic iodine supplementation for pregnant and lactating women needs to be established and confirmation that excess intake of iodine during pregnancy is to be avoided.

A new therapy prevents intellectual disability in mouse with phenylketonuria.

Untreated phenylketonuria (PKU) results in severe neurodevelopmental disorders, which can be partially prevented by an early and rigorous limitation of phenylalanine (Phe) intake. Enzyme substitution therapy with recombinant Anabaena variabilis Phe Ammonia Lyase (rAvPAL) proved to be effective in reducing blood Phe levels in preclinical and clinical studies of adults with PKU. Aims of present study were: a) to gather proofs of clinical efficacy of rAvPAL treatment in preventing neurological impairment in an early treated murine model of PKU; b) to test the advantages of an alternative delivering system for rAvPAL such as autologous erythrocytes. BTBR-Pahenu2-/- mice were treated from 15 to 64 post-natal days with weekly infusions of erythrocytes loaded with rAvPAL. Behavioral, neurochemical, and brain histological markers denoting untreated PKU were examined in early treated adult mice in comparison with untreated and wild type animals. rAvPAL therapy normalized blood and brain Phe; prevented cognitive developmental failure, brain depletion of serotonin, dendritic spine abnormalities, and myelin basic protein reduction. No adverse events or inactivating immune reaction were observed. In conclusion present study testifies the clinical efficacy of rAvPAL treatment in a preclinical model of PKU and the advantages of erythrocytes as carrier of the enzyme in term of frequency of the administrations and prevention of immunological reactions.

[Epilepsy and Pregnancy]. / Epilepsie und Schwangerschaft.

BACKGROUND: On average, female patients with epilepsy have 0.9 children, which is below the birth rate of healthy women. One reason is insufficient counselling. OBJECTIVES: To summarize the current data relevant to counselling pregnant women with epilepsy. MATERIALS AND METHODS: Discussion of research and recommendations concerning seizure control during pregnancy, pregnancy and birth complications, congenital malformations, and breastfeeding. RESULTS: Changes in seizure frequency during pregnancy are variable and partly due to changes in the serum concentrations of antiepileptic drugs. Epilepsy patients have a slightly higher risk for some pregnancy and birth complications including spontaneous abortion, pre- and postpartum bleeding, induction of labour, and caesarean section. In particular, the administration of valproic acid can lead to congenital malformations and a lower IQ of the child. Folic acid seems to have a protective effect. Data concerning breastfeeding are insufficient. CONCLUSIONS: If possible, epilepsy patients should be treated with a low-dose monotherapy during pregnancy and valproic acid should be avoided. Treatment with lamotrigine requires frequent control of serum concentration. Supplementary folic acid (5 mg daily dose) is recommended. Epilepsy is not an indication for a caesarean section.

Proceso de atención integrada para niños con necesidades especiales (PAINNE) / Process for integrated care for children with special needs (PAINNE)

Introducción y objetivos: la prevención de las deficiencias infantiles resulta más eficiente cuando coordinamos recursos, implicamos a los profesionales e incorporamos herramientas para la detección e intervención temprana en las deficiencias infantiles y en situaciones de riesgo biológico, psicológico y/o social. Material y métodos: presentamos los primeros resultados de la implantación del Proceso de atención integrada en niños con necesidades especiales (PAINNE), de la Organización Sanitaria Integrada Bilbao-Basurto (Bilbao, España), siguiendo el modelo de atención temprana del País Vasco, en una población de 20 655 menores de seis años. Aplicando metodología cualitativa, incorporamos nuevos datos básicos del paciente sobre desarrollo infantil en la historia clínica electrónica, junto con los indicadores del proceso, recogidos en la guía PAINNE 2013. Resultados: el 75% de los pediatras y el 56% de los profesionales de enfermería participaron previamente en actividades de formación en atención temprana. Las propuestas desde los equipos de Pediatría se validaron por el equipo técnico de valoración en atención temprana de la Diputación Foral de Bizkaia (EVAT), en el 93,27% de los casos. La edad media de derivación a salud mental infantil descendió en ocho meses, hasta los tres años, en casos de enfermedad mental grave, mejorando el diagnóstico precoz y el inicio de los programas de intervención. Conclusiones: los resultados obtenidos en el primer año indican que los equipos de Pediatría se consolidan como gestores válidos de 516 niños con necesidades especiales de salud en programas de atención temprana entre 0 y 6 años (AU)

Introduction and objectives: the prevention of childhood deficits is more efficient when resources are coordinated amongst the professionals responsible for the child's care and screening tools are utilized to detect and provide early intervention to address situations of biological, psychological, and/or social risk. Material and methods: we present the initial results of the implementation of the process for integrated care for children with special needs, PAINNE, in the integrated health organization Bilbao-Basurto, following the model of early intervention in the Basque Country, with a population of 20 655 children under the age of 6 years old. Applying the qualitative methodology, we have incorporated developmental screening tools in the electronic medical record, along with the other process indicators, as described in the guide PAINNE 2013. Results: 75% of the pediatricians and 56% of the nurses participated in continuing educational activities regarding early intervention. The referrals for early intervention were accepted in 93.27% of the cases by the Basque Early Intervention Team (EVAT). The median age for referral to mental health services for children with severe mental health problems decreased by 8 months to 3 years old, showing improvement in the early detection and implementation of services. Conclusions: the results obtained in the first year indicated that the Pediatric Primary Care teams have reliably identified and referred 516 children with special needs, ages 0-6 years, to early intervention programs (AU)

Midwives' comprehension of care for women with intellectual disability during pregnancy and childbirth: An open-ended questionnaire study in Sweden.

BACKGROUND: Few studies have investigated midwifery care for women with intellectual disability (ID). AIM: To gain a deeper understanding of midwives' comprehension of care for women with ID during pregnancy and childbirth. METHODS: A cross-sectional study among 375 midwives at antenatal clinics and delivery wards in Sweden. Findings 2476 quotations were sorted into six categories: information; communication and approach; the role of the midwife; preparing for and performing interventions and examinations; methods and assessments; and organisation of care. The midwives affirmed that individual, clear and repeated information together with practical and emotional support was important for women with ID. The midwives planned the care as to strengthen the capacity of the women, open doors for the unborn child and reinforce the process of becoming a mother. Extra time could be needed. They tried to minimise interventions. The midwives felt a dual responsibility, to support the mother-child contact but also to assess and identify any deficits in the caring capacity of the mother and to involve other professionals if needed. CONCLUSIONS: The midwives described specially adapted organisation of care, models of information, practical education and emotional support to facilitate the transition to motherhood for women with ID. They have a dual role and responsibility in supporting the woman, while making sure the child is properly cared for. Healthcare services should offer a safe and trusted environment to enable such midwifery care. When foster care is planned, the society should inform and co-operate with midwives in the care of these women.

Rich micronutrient fortification of locally produced infant food does not improve mental and motor development of Zambian infants: a randomised controlled trial.

It is uncertain whether multiple micronutrients benefit the mental and psychomotor development of young children in developing countries. We conducted a randomised double-blind controlled trial to evaluate the effect of a richly micronutrient-fortified v. a basal fortified porridge on mental and psychomotor development in Zambian infants. Infants (n 743) were randomised at age 6 months to receive either the richly fortified or the basal fortified infant food and were followed up until 18 months of age. All the infants were evaluated monthly for achievement of a series of developmental milestones. The Bayley scales of infant development II were administered to a subsample of 502 infants at 6, 12 and 18 months. Rich micronutrient fortification had no significant benefit on the following: (a) number of developmental milestones achieved (rate ratio at 12 months = 1·00; 95 % CI 0·96, 1·05; P = 0·81, adjusted for sex, socio-economic status and maternal education, with similar results at 15 and 18 months); (b) ages of walking unsupported (hazard ratio (HR) 1·04; 95 % CI 0·88, 1·24; P = 0·63, adjusted for the above covariates) and of speaking three or four clear words (HR 1·01; 95 % CI 0·84, 1·20; P = 0·94, adjusted for the above covariates); (c) mental development index (MDI) and psychomotor development index (PDI) of the Bayley scales (scores difference adjusted for baseline scores, age at the assessment, sex, socio-economic status, maternal education, language, age and HIV status: MDI 0·3 (95 % CI - 0·5, 1·1), P = 0·43; PDI - 0·1 (95 % CI - 0·9, 0·7), P = 0·78). In conclusion, the results do not support the hypothesis that rich micronutrient fortification improves Zambian infants' mental and motor development.

Iodine deficiency in pregnancy: the effect on neurodevelopment in the child.

Iodine is an integral part of the thyroid hormones, thyroxine (T(4)) and tri-iodothyronine (T(3)), necessary for normal growth and development. An adequate supply of cerebral T(3), generated in the fetal brain from maternal free T(4) (fT(4)), is needed by the fetus for thyroid hormone dependent neurodevelopment, which begins in the second half of the first trimester of pregnancy. Around the beginning of the second trimester the fetal thyroid also begins to produce hormones but the reserves of the fetal gland are low, thus maternal thyroid hormones contribute to total fetal thyroid hormone concentrations until birth. In order for pregnant women to produce enough thyroid hormones to meet both her own and her baby's requirements, a 50% increase in iodine intake is recommended. A lack of iodine in the diet may result in the mother becoming iodine deficient, and subsequently the fetus. In iodine deficiency, hypothyroxinemia (i.e., low maternal fT(4)) results in damage to the developing brain, which is further aggravated by hypothyroidism in the fetus. The most serious consequence of iodine deficiency is cretinism, characterised by profound mental retardation. There is unequivocal evidence that severe iodine deficiency in pregnancy impairs brain development in the child. However, only two intervention trials have assessed neurodevelopment in children of moderately iodine deficient mothers finding improved neurodevelopment in children of mothers supplemented earlier rather than later in pregnancy; both studies were not randomised and were uncontrolled. Thus, there is a need for well-designed trials to determine the effect of iodine supplementation in moderate to mildly iodine deficient pregnant women on neurodevelopment in the child.

[Iodine deficiency in infancy - a risk for cognitive development]. / Jodmangel im Säuglingsalter - ein Risiko für die kognitive Entwicklung.

Severe iodine deficiency during pregnancy seriously influences fetal brain development and in the worst case induces cretinism. Recent studies have shown that even a mild iodine deficiency during pregnancy and during the first years of life adversely affects brain development. The World Health Organisation (WHO) considers iodine deficiency as the most common preventable cause of early childhood mental deficiency. In this context, the insufficient production of the four iodine atoms containing thyroxine seems to play a causal role, i. e., due to the iodine substrate deficiency the neuronally particularly relevant free-thyroxine level falls. Due to the very limited iodine storage capacity, the infantile thyroid is eminently dependent on an adequate and steady iodine supply. In the first month of life, when milk is the only energy- and nutrient provider, infants fed a commercial formula regularly have a sufficient iodine supply. However, breastfed infants, who depend on maternal iodine status, frequently show an inadequate iodine intake. Furthermore, iodine intake is critical when complementary food (CF) is introduced. Especially homemade CF is poor in iodine, but also commercial CFs are only partly fortified. A simultaneous inadequate iodine supply of the breastfeeding mother and the preferential use of mostly iodine-poor organic milk cannot ensure an adequate iodine supply of the infant. In terms of an improvement of nutrient supply, especially concerning an unhindered brain development, the corresponding German reference value for iodine intake of infants until age 4 month should be raised from currently 40 microg/d to at least 60 microg/d (WHO-reference: 90 microg/d).

Profile of iodine content of salt and urinary iodine excretion levels in selected districts of Tamil Nadu.

OBJECTIVE: Iodine deficiency disorders (IDD) is the most common cause of preventable mental retardation in world today. Out of 29 districts in Tamil Nadu, 24 have been surveyed and all have been found to be endemic to IDD. There is a complete ban on the sale of non iodised salt in the state since 1995. The present study was conducted to assess the iodine content of salt and urinary iodine excretion levels amongst the beneficiaries in the state to help the government to strengthen the existing Universal salt iodisation (USI) programme activities. METHODS: The study was undertaken in 24 districts of Tamil Nadu during the year 2001. The guidelines recommended by WHO/UNICEF/ICCIDD for a rapid assessment of salt iodisation in a district was adopted. A minimum of 150 salt samples were collected from each district by utilising the uniform sampling methodology. The iodine content of salt samples was analysed using the standard iodometric titration method. At least 60 children from each school were randomly selected and were requested to provide "on the spot" casual urine samples. The urinary iodine excretion (UIE) levels were analysed using the wet digestion method. RESULTS: It was observed that 62.3% of the families were consuming iodised salt with more than 5 ppm of iodine. It was found that district Perambalour had deficient iodine nutriture as revealed by the median UIE levels less than 100 micro g/l. CONCLUSION: The findings of the present study reveals the success of USI programme and highlights need for continued monitoring of the quality of salt provided to the population in order to achieve the goal of IDD elimination.

Treatment of Crigler-Najjar type 1 disease: relevance of early liver transplantation.

BACKGROUND: Crigler-Najjar syndrome type 1 (CNS1) is characterized by severe unconjugated hyperbilirubinemia from birth, caused by total failure of UDP-glucuronyltransferase activity. Only orthotopic liver transplantation (OLT) offers the prospect of cure. However, because the onset of neurologic deficits is unpredictable, timing of OLT remains difficult. METHODS: In our transplant center, 3 patients underwent early OLT for CNS1. Two of them (7 yr, 12 yr) showed mild to moderate neurologic deficits only few weeks before OLT, another patient (4 yr) had no signs of bilirubin encephalopathy. All patients required extensive phototherapy to control bilirubin levels. Thus, OLT was performed shortly after the onset of neurologic symptoms or as a prophylactic procedure, respectively. RESULTS: OLT was uneventful in all recipients. One of the symptomatic patients (7 yr) completely recovered from neurologic deficits at 36 months on OLT, whereas the other patient (12 yr) significantly improved symptoms at 27 months of OLT. These patients, including the 4-year-old boy, attend school at appropriate grades now. CONCLUSIONS: Irreversible brain damage (kernicterus) may occur in the course of CNS1 disease. Because no alternative treatment options are available at this time, OLT should be performed as a preventive procedure to counteract severe CNS-related complications.

Congenital hypothyroidism: a review of current diagnostic and treatment practices in relation to neuropsychologic outcome.

Because thyroid hormone is essential for normal brain development, children born with congenital hypothyroidism who lack thyroid hormone during a circumscribed period of early development are at risk of brain damage and mental retardation. Since the advent of newborn screening programs in the 1980s, the diagnosis and treatment of this condition are now provided in the first 2-3 weeks of birth in most regions. While this is usually sufficient to prevent mental retardation, the children so identified attain mildly reduced IQs from expectation, and may still experience subtle and specific neurocognitive deficits. Their particular deficits are related to the brief period of thyroid hormone insufficiency they undergo, especially factors reflecting the severity of hypothyroidism at the time of diagnosis, the duration of hypothyroidism in infancy, and thyroid hormones at time of testing. In this article, we review the specific kinds of deficits demonstrated by children with congenital hypothyroidism who were diagnosed by screening and treated early, as well as the factors associated with their disease and its management that contribute to these deficits. The disease-related factors that will be reviewed will include the etiology of hypothyroidism and severity of disease at the time of diagnosis, while the treatment-related factors will include age at onset of therapy, starting and subsequent dose levels, compliance, and treatment-adequacy issues. Also examined will be the effects of hormone levels at the time of testing. In addition, the role of moderating variables such as social, genetic, and environmental influences, as well as the child's gender, will be discussed. Furthermore, several new issues including the quality of subsequent management, ultimate outcome, and pregnancy will be additionally reviewed. In conclusion, while outcome in congenital hypothyroidism is substantially improved by screening, affected children do still experience mild neuropsychologic deficits. To reduce the impact of persisting deficits, further research is needed to determine the optimal starting dose for the different etiologies, guidelines for subsequent management, and alternative therapies. Moreover, now that the original samples are reaching adulthood and, in females, childbearing age, further research is also needed regarding treatment during pregnancy in women with congenital hypothyroidism, as is research to determine how this population ultimately fares in adulthood.

[Prevention of mental retardation]. / Prevención del retraso mental.

OBJECTIVE: To present within the general field of the conditions causing mental retardation, the preventive strategies for specific application available at the present time. DEVELOPMENT: In spite of the fact that in the majority of cases of mental retardation the etiology is unknown, and for that reason, in them it is not possible to establish preventive strategies, within the last three decades, important research advances have helped to prevent thousands of cases of mental retardation of illnesses caused by Haemophilus influenzae B, measles encephalitis, Rh disease and severe jaundice in newborn infants, congenital hypothyroidism, phenylketonuria and congenital rubella; as well as removing lead from the environment, intervention programs for the proper use of seat belts, child safety seats, and motorcycle and bicycle helmets; early and adequate prenatal care, dietary supplementation with folic acid beginning before conception to reduce the risk of neural tube defects, avoidance of toxic substances during pregnancy like alcohol, and the use of newborn screening tests. CONCLUSION: The primary and secondary prevention of conditions that cause mental retardation continue being a challenge. Require of a review of the present strategies, that frequently inform about the problem, but are not practice in an every day bases (ej. intake of alcohol during pregnancy, the universal use of seat belt and child safety seats during automobile travel). In the future we may have the possibility of prenatal gene therapy.

Iodised salt for preventing iodine deficiency disorders.

BACKGROUND: Iodine deficiency is the main cause for potentially preventable mental retardation in childhood, as well as causing goitre and hypothyroidism in people of all ages. It is still prevalent in large parts of the world. OBJECTIVES: To assess the effects of iodised salt in comparison with other forms of iodine supplementation or placebo in the prevention of iodine deficiency disorders. SEARCH STRATEGY: We searched the Cochrane Library, Medline, the Register of Chinese trials developed by the Chinese Cochrane Centre, and the Chinese Med Database. We performed handsearching of a number of journals (Chinese Journal of Control of Endemic Diseases, Chinese Journal of Epidemiology, Chinese Journal of Preventive Medicine, and Studies of Trace Elements and Health up to February 2001), and searched reference lists, databases of ongoing trials and the Internet. Date of latest search: November 2001. SELECTION CRITERIA: We included prospective controlled studies of iodised salt versus other forms of iodine supplementation or placebo in people living in areas of iodine deficiency. Studies reported mainly goitre rates and urinary iodine excretion as outcome measures. DATA COLLECTION AND ANALYSIS: The initial data selection and quality assessment of trials was done independently by two reviewers. Subsequently, after the scope of the review was slightly widened from including only randomised controlled trials to including non-randomised prospective comparative studies, a third reviewer repeated the trials selection and quality assessment. As the studies identified were not sufficiently similar and not of sufficient quality, we did not do a meta-analysis but summarised the data in a narrative format. MAIN RESULTS: We found six prospective controlled trials relating to our question. Four of these were described as randomised controlled trials, one was a prospective controlled trial that did not specify allocation to comparison groups, and one was a repeated cross-sectional study comparing different interventions. Comparison interventions included non-iodised salt, iodised water, iodised oil, and salt iodisation with potassium iodide versus potassium iodate. Numbers of participants in the trials ranged from 35 to 334; over 20,000 people were included in the cross-sectional study. Three studies were in children only, two investigated both groups of children and adults and one investigated pregnant women. There was a tendency towards goitre reduction with iodised salt, although this was not significant in all studies. There was also an improved iodine status in most studies (except in small children in one of the studies), although urinary iodine excretion did not always reach the levels recommended by the WHO. None of the studies observed any adverse effects of iodised salt. REVIEWER'S CONCLUSIONS: The results suggest that iodised salt is an effective means of improving iodine status. No conclusions can be made about improvements in other, more patient-oriented outcomes, such as physical and mental development in children and mortality. None of the studies specifically investigated development of iodine-induced hyperthyroidism, which can be easily overlooked if just assessed on the basis of symptoms. High quality controlled studies investigating relevant long term outcome measures are needed to address questions of dosage and best means of iodine supplementation in different population groups and settings.

[A preliminary meta-analysis of 36 studies on impairment of intelligence development induced by iodine deficiency].

OBJECTIVES: Since 1980 s', numerous studies on intelligence quotient (IQ) have documented, in the areas prevalent with severe, moderate and even mild iodine deficiency, which is a risk factor for retardation in mental development there. It was purposed for this paper to quantify the relationship between iodine deficiency and mental development in children and to explore the etiological role of iodine deficiency in mental retardation and the protective effects on children's intelligence of iodine supplementation. METHODS: A total of 59 independent investigations published during 1980 and 1998 were selected for meta-analysis, including 20 studies on intelligence determined by Raven's Test and 39 by China Benit Scale. RESULTS: Homogeneity test showed that there was no significant difference in baseline features between two groups (P > 0.05). The hypothesis testing showed that the homogeneity of each study group was obviously statistically significant (P > 0.05). The results demonstrated that IQ in children at risk for iodine deficiency showed a marked drop by 8.94 points with Raven's Test and by 10.80 points with China Binet Scale, respectively, with an average drop of 10 points. Substantial evidence now available has showed that mental retardation can be prevented by effective correction of iodine deficiency through iodine supplement either iodized salt or iodized oil, which is confirmed by an obvious increase in 11.5 points of IQ in average, and in 11.85 by Raven's Test and 11.64 by China Binet Scale, respectively. CONCLUSIONS: Iodine nutrition plays an important and positive role in brain development. Iodine deficiency leads to loss of 10 points of IQ and 11.5 points can be gained for children in the iodine deficiency areas after significant iodine supplement.

[Comparison of two strategies for the early detection of congenital hypothyroidism]. / Comparação entre duas estratégias para a detecção precoce do hipotiroidismo congênito.

OBJECTIVE: Compare two different strategies in newborn screening for congenital hypothyroidism, primary TSH in the umbilical cord blood (method 1) and primary T4 in blood collected from the heel in the 2nd day of life (method 2). METHODS: We compared both strategies in 10,000 newborns, measuring TSH by a sensitive immunofluorimetric assay and T4 by a radioimmunoassay. RESULTS: Both strategies detected all cases of hypothyroidism (4 cases, 1/2,500 newborns). The recalling index owing to insufficient amount of blood to perform the assays was zero in method 1 and 8.5% (850 newborns) in method 2. The recalling index for confirmation of the results was 0.06% (6 newborns) in method 1 and 2.25% (225 newborns) in method 2; when method 2 included supplementary TSH, the recalling index was reduced to 1.63% (163 newborns). CONCLUSION: Our data indicate the technical superiority of the umbilical cord blood compared to heel and primary TSH compared to primary T4 in the neonatal thyroid screening for congenital hypothyroidism.

Nutritional deficiencies and subsequent effects on mental and behavioral development in children.

Nutritional deficiencies at all stages of growth, both pre- and post-natal, can affect a child's physical, mental and behavioral development. In this paper, we review literature about how mental development is affected by the following nutritional conditions: low birth weight, mild, moderate, and severe protein-energy malnutrition, iodine deficiency, and iron deficiency.

Glutamate in pyridoxine-dependent epilepsy: neurotoxic glutamate concentration in the cerebrospinal fluid and its normalization by pyridoxine.

BACKGROUND: Pyridoxine-dependent epilepsy is a rare autosomal recessive disorder. Untreated patients suffer from a progressive encephalopathy with mental retardation, intractable epilepsy, and progressive neurological signs and symptoms. Lifelong supplementation with vitamin B6 is the treatment of choice. However, despite early treatment, many patients develop mental retardation. OBJECTIVES: To assess the role of glutamate as an excitatory neurotransmitter and neurotoxin in pyridoxine-dependent epilepsy. METHODS: We examined cerebrospinal fluid (CSF) levels of glutamate, gamma-aminobutyric acid, and pyridoxal-5'-phosphate in a patient with pyridoxine dependency while on and off vitamin B6 treatment. RESULTS: Off vitamin B6 the glutamate level was two hundred times normal. An intermediate dose of vitamin B6 (5 mg/kg BW/day) caused normalization of the EEG and remission of the seizures, but the CSF glutamate concentration was still ten times normal. With a higher dose of pyridoxine (10 mg/kg BW/day) the CSF glutamic acid normalized. CONCLUSIONS: The results indicate that control of epilepsy might not suffice as the therapeutic aim in treating of pyridoxine dependency. In view of the evidence for the role of excitatory amino acids in destruction of CNS nerve cells, the optimal treatment must counteract the raised levels of CSF glutamate and the dosage of vitamin B6 must be adjusted accordingly. The development of mental retardation might theoretically be prevented by adjusting the dose of vitamin B6 to achieve not only remission of epilepsy but also normalization of CSF glutamate.

Strengthening mental health services in Head Start. A challenge for the 1990s.

Steps must be taken to strengthen mental health services by building on Head Start's philosophy and by translating innovations in mental health services for older children and adolescents into improved services for young children and their families. Recommendations for strengthening Head Start's mental health program include creating a unified vision that reaffirms a holistic, family-focused, and comprehensive services approach; ensuring that mental health services are responsive to the diversity in families served; increasing coordination of mental health services and linkages with new initiatives; increasing resources and providing assistance in gaining access to new sources of funding; supporting innovation; and integrating the new paradigm for children's mental health services into more traditional approaches to intervention within Head Start.

Substance abuse in adolescent mothers: strategies for diagnosis, treatment and prevention.

Adolescent drug addiction and alcohol abuse are on the rise in this country. Teenagers of all races, socioeconomic groups and both genders have become avid experimenters with a full spectrum of substances, including alcohol, marijuana, stimulants, cocaine and hallucinogens. A diagnosis of addiction requires familiarity with the normative trends of adolescent behavior, as well as deviations from these trends. Moreover, an adolescent mother with a drug addiction problem confronts the additional burden of fostering adaptive developmental patterns in her infant. Treatment techniques such as previewing, which empowers through the representation and enactment of future outcomes, have helped addicted teenage mothers relinquish drug use and adopt more mature caregiving behaviors.