R Melbourne-Chambers

Aicardi syndrome associated with anterior cephalocele in a female infant

Aicardi syndrome is a triad of abnormalities that includes total or partial agenesis of the corpus callosum, chorioretinal lacunae, and infantile spasms. This syndrome was first described in 1965. A female infant with Aicardi syndrome associated with a nasoethmoidal cephalocele is described in this report. She presented with a history of unilateral nasal discharge since birth and seizures since age 1 week. She was microcephalic and there was visual impairment. A fleshy mass of the left nostril was noted. Ophthalmological evaluation revealed left exotropia, dysplastic optic discs and retina, ‘morning glory’ appearance of the left optic disc, and bilateral chorioretinal lacunae. Magnetic resonance imaging of the brain showed absence of the corpus callosum, dysmorphic changes of the lateral ventricles, a superiorly located third ventricle, heterotopic grey matter of the frontal lobes, a left nasoethmoidal cephalocele, and closed lip schizencephaly of the left frontal lobe. This female infant developed asymmetric infantile spasms at age 8 weeks. Surgical correction of the cephalocele was declined. She developed recurrent pneumonias secondary to aspiration of feeds and died at age 8 months during one of these events.

Pregnancy in sickle cell-haemoglobin C (SC) disease. A retrospective study of birth size and maternal weight gain

OBJECTIVE To assess pregnancy and fetal outcomes in Jamaican subjects with sickle cell-haemoglobin C (SC) disease. STUDY DESIGN A retrospective chart review over 21 years (1992-2012) of all pregnancies in SC disease and a comparison group matched by gender and date of delivery in mothers with a normal haemoglobin (AA) phenotype at the University Hospital of the West Indies, Jamaica. There were 118 pregnancies in 81 patients with SC disease and 110 pregnancies in 110 in the normal comparison group. Corrections were made for repeat pregnancies from the same mother. Outcome measures included maternal weight at 20, 25, 30, 35 and 38 weeks gestation, maternal pregnancy complications, birth weight, head circumference and crown heel length and were used to analyse possible predictors of birth weight. RESULTS First antenatal visits occurred later in women with SC disease, who also had lower haemoglobin level and lower systolic blood pressure. The prevalence of pregnancy-induced hypertension, pre-eclampsia, ante-partum or postpartum haemorrhage did not differ between genotypes. Maternal weight gain was significantly lower in SC disease and there was a significantly lower birth weight, head circumference, and gestational age. CONCLUSIONS Pregnancy in SC disease is generally benign but mothers had lower weight gain and lower birth weight babies, the difference persisting after correction for gestational age.

Parent and caregiver knowledge, beliefs, and responses to convulsive seizures in children in Kingston, Jamaica - A hospital-based survey.

OBJECTIVE The objective of this study was to determine the knowledge and beliefs about seizures and actions during seizures of parents/caregivers of Jamaican children hospitalized for convulsive seizures. DESIGN AND METHODS This was a cross-sectional study of parents and caregivers of children with acute convulsive seizures hospitalized at the Bustamante Hospital, Kingston, Jamaica between May 1 and October 31, 2013. Subjects were identified by admission records. Parents/caregivers were invited to participate. A questionnaire on the demographics, knowledge, beliefs, and response of parents/caregivers during the childs current seizure episode was administered face to face. Data were analyzed for frequencies: groups were compared using chi-square analysis for categorical variables, Students t-test for normally distributed data, and the Mann-Whitney U-test for data not normally distributed. RESULTS There were fifty participants: 39 (78%) mothers, mean (SD) age - 33.8 (10.1) years. All sought medical care first. Twenty-two (44%) had plausible beliefs about the cause of seizures. Twenty-seven (54%) knew of appropriate actions during a seizure, 10 (20%) knew of appropriate precautions, and 11 (22%) responded appropriately during the seizure. Eleven (22%) reported receiving seizure education. Witnessing a previous seizure, education level, and seizure education were associated with knowledge of seizures (p<0.05). Socioeconomic status was higher in those with plausible beliefs about seizures and lower in those who took appropriate action during a seizure (p<0.05). CONCLUSION Parents/caregivers of children with convulsive seizures have appropriate health-care seeking behavior, but most do not have appropriate knowledge about seizures. Few take appropriate action during the episode. A public education program is needed to improve parental knowledge of and response to convulsive seizures.

Traditional Antiepileptics Remain First Line Treatment for Children with Epilepsy at The University Hospital of the West Indies, Kingston, Jamaica

Objectives: To describe the demography of epilepsy in children at the University Hospital of the West Indies and to review the frequency of major seizure types and antiepileptic drug therapy used at diagnosis. Methods: The records of all children ages two months to 12 years with two or more unprovoked seizures, on at least two separate days between January 2001 and December 2005 at the paediatric in-patient unit and paediatric neurology clinic were reviewed. Descriptive analyses (Chi-squared tests) were performed utilizing the Statistical Programme for Social Sciences (SPSS version 14). Statistical significance, p < 0.05. Results: Sixty-three children were enrolled in the study; thirty-two (50.8%) were males. The median (minimum, maximum) age was 35 (2, 144) months at seizure onset. N (65%) had focal seizures of which dyscognitive seizures were the most frequent subtype. Generalized tonic clonic and myoclonic seizures were the most frequent of the generalized seizures. Nineteen (30%) children had more than one seizure type and 17 (27%) of the children were diagnosed with an epilepsy syndrome. West syndrome was the commonest of the epilepsy syndromes. Carbamazepine was the most frequently prescribed drug at initial diagnosis for focal seizures. Valproic acid was the drug of choice for generalized seizures. Conclusion: There was no sex predilection in epilepsy in this population. Focal seizures were commonest and Carbamazepine was the choice drug for focal seizures and Valproic acid for generalized seizures.

Acute Paralysis and Neuro-inflammation in Jamaican Children during ZIKV and Dengue Epidemics of 2016

Dengue, Chikungunya virus (CHIKV) and Zika virus (ZIKV) are all transmitted by the Aedes aegypti mosquito and are currently circulating in Jamaica. Jamaica has been experiencing a ZIKV epidemic since February 2016. At the University Hospital of the West Indies (UHWI), Kingston, Jamaica, a cluster of five cases of paralysis attributed to neuro-inflammation was noted amongst adolescents admitted to the institution. Three were diagnosed with acute myelitis and one each with acute disseminated encephalomyelitis (ADEM) and Guillain Barre syndrome (GBS). In these patients, there were common presenting symptoms, characteristic findings of peripheral nerve involvement and a history of contact with persons with symptoms of possible ZIKV in the majority. In only one case was a viral association, Dengue infection, confirmed. This case series suggests a unique clinical pattern of neuro-inflammation in Jamaican adolescents occurring during the ZIKV epidemic and questions the role of the three circulating arboviruses in the pathogenesis.