Peripheral neuropathies of childhood

Includes synopsis.Incldues bibliographical references (p. 195-220).Peripheral nerve disease was described by Galen (AD 130-200) over a thousand years ago.(3) Detailed anatomical illustrations were documented by Andreas Vesalius in his major work 'De humani corporis fabrica' in 1543.(4) Over the last two centuries an explosion in knowledge in the area has occurred, with a further exponential increase in the last 20 years mostly related to understandings in the field of molecular genetics.(5) Although some degree of diagnostic closure was possible for a number of the hereditary peripheral neuropathies, this has not been the end point of knowledge but only the beginning

Use of phenobarbitone for treating childhood epilepsy in resource-poor countries

Should the continued use of phenobarbitone for childhood epilepsy in resource-poor countries be considered a form of discrimination? Phenobarbitone was recommended by the World Health Organization (WHO) as the first-line agent for the control of seizures,1 but this has been contested on the grounds that it is biased against resource-poor countries.2 It was first used as an anticonvulsant in 1912, but now has little role to play in First-World countries where the newer generation agents are readily accessible. Phenobarbitone monotherapy has equivalent efficacy to the newer anticonvulsants (phenytoin, sodium valproate and carbamazepine) in children with partial-onset and generalised tonic-clonic seizures.3 Phenobarbitone is cheap, readily available, and easy to use and store. However, it has definite cognitive and behavioural side-effects in many children. It can exacerbate seizures in about 35% of children, and extreme caution should be taken with children who have a pre-morbid state of behavioural problems or attention deficit hyperactivity disorder (ADHD)

Sydenham's chorea - clinical and therapeutic update 320 years down the line

No Abstract. South African Medical Journal Vol. 96(9) (Part 2) 2006: 906-91

The role of ICNA in Africa

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/106854/1/j.1469-8749.2011.03972.x.pd

The impact of HIV infection on the nervous system of children

At the end of 2007, 33.2 million people including 2.5 million children were living with HIV; > 85% of HIV-infected children were in Africa. At the end of 2006, 115 000 children were on HAART, a global coverage rate of 15% with sub-Saharan Africa having the lowest regional coverage. HIV affects the immature brain causing static or progressive encephalopathy (PE). PE is characterized by acquired microcephaly, failure to attain or loss of neurodevelopmental milestones, or loss of intellectual ability, and acquired symmetric motor defects. Isolated neurodevelopmental delays and peripheral nervous system disease occur as a direct consequence of HIV infection. Susceptibility to opportunistic infection (OI), due to systemic immunodeficiency, predisposes HIV-infected children to CNS infections including acute bacterial and tuberculous meningitis, CMV co-infection, EBV-associated primary lymphoma and in older children cryptococcal meningitis. HAART may induce an immune reconstitution inflammatory response to several microorganisms including mycobacterium tuberculosis and JC virus, causing CNS deterioration. Neurological manifestations occur in 20– 80% of HIV-infected children. Complex clinical presentations may be difficult to classify with the layering effect of multiple pathologies. There are limited descriptive studies from resource-constrained countries. In one hospitalbased, cross-sectional survey (n = 80, median age = 5.2 years), 60% had a variety of neurological and neurodevelopmental deficits. The neuropathogenesis of HIV encephalopathy is incompletely understood. Several pathogenic events are involved including (1) CNS invasion following receptor/ co-receptor-mediated HIV infection of monocytes/macrophages and CD4+ T-lymphocytes, (2) promotion of HIVinfected PBMC trafficking across the BBB by astrocytes and microglia (Trojan-horse effect), (3) neurotoxin elaboration induced by viral factors and inflammatory mediators, (4) CD4-independent astrocyte invasion by HIV, (4) neuronal death mediated through viral-induced chemokine receptor expression, and microglial/macrophagedependent and microglial-independent apoptotic death, and (5) inhibition of neural stem cell proliferation by viral gp120. Both direct and indirect mechanisms are involved in neuronal injury and death. Early initiation of HAART during infancy prevents the development of HIV encephalopathy, lowering the prevalence of progressive encephalopathy to < 2%. HAART reverses existing neurological abnormalities, although not completely, resulting in residual motor and cognitive sequelae (arrested PE) and consequent scholastic impairments. HAART also reduces the frequency of OIs and malignancy. In conclusion, HIV-associated nervous system disease remains highly prevalent in settings where treatment is sub-optimal. Implementation of HAART during infancy and additional prophylaxis against OIs such as

Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study

Abstract Background Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory childhood myopathy of uncertain aetiology. The demographic and clinical presentation of JDM may differ by race and geographic regions. Few studies have described the characteristics of JDM patients from Africa. Methods We conducted a retrospective observational study to determine clinical characteristics and outcomes of patients satisfying the Bohan and Peter criteria for probable JDM seen between 2004 and 2013 in three hospitals in Cape Town, South Africa. Results Twenty five cases were identified: 16 female and 9 male; thirteen (52 %) were of indigenous African, eleven (44 %) mixed and one (4 %) European ancestry. The median ages at disease onset and diagnosis were 6.75 (range 2.0–9.7) and 7.9 (range 3.4–9.75) years respectively. Eleven patients had calcinosis while the mortality was 2/25 (8 %). Only 40 % of the patients had clinically inactive disease by PRINTO criteria (modified) at last review. There was no statistically significant difference in racial distribution (p-value = 1), age at disease onset (p-value = 0.87) and disease duration prior to treatment initiation (p-value = 0.75) between patients who had clinically active and inactive disease. Conclusion The demographic characteristics of children with JDM were similar to that from most other regions of the world with female predominance and similar age at onset. Majority of the patients remained with clinically active disease, which put them at risk of further disease complications. Long term follow up and use of appropriate treatment guidelines may be indicated in management of JDM patients for optimum treatment outcomes

Presentation and Outcome of Tuberculous Meningitis among Children: Experiences from a Tertiary Children’s Hospital

Background: Diagnosis of tuberculous meningitis (TBM) is complicated and outcome is poor especially in resource limited settings. Early diagnosis and prompt treatment are vital in effective treatment. We set out to describe experiences in the management and immediate outcome of TBM a tertiary-level children’s hospital in a high HIV and tuberculosis co-infection setting. Methods: This retrospective study included children who were diagnosed with TBM in the year 2009. A pre-coded questionnaire was used to extract data on presentation, diagnostics, treatment and outcome at the time of hospital discharge. Data was analyzed using STATA statistical package (StataCorp, Version 11). Results: Of the 40 children diagnosed with TBM, 6 (15%) had definitive TBM, 17 (42.5%) had probable TBM and 17 (42.5%) had possible TBM. The cerebrospinal fluid (CSF) chemistry and cells were abnormal in 39/40 (98%). Mantoux test was reactive in 16/29 (55%) and 17/30 (57%) had Chest X-rays suggestive of tuberculosis. Only 3/21 (14%) had positive sputum tuberculosis culture and 89% (32/36) had neuro-imaging abnormalities. Outcome at discharge was; 8% died, 49% improved with neurological sequelae and 43% improved without sequelae. Having TBM stage 3 at admission was associated with mortality (p=0.001). Conclusions: Most children had early diagnosis of TBM and mortality was lower than previous studies. We recommend a larger prospective study to further understand the outcome of TBM.Keywords: Tuberculous meningitis, children, presentation, outcome, AfricaAfrican Health sciences Vol 14 No. 1 March 201

Parental Understanding of Tuberous Sclerosis Complex

Tuberous sclerosis complex is a genetic disorder with multisystem involvement that poses significant challenges to the affected child and family. Caregiver knowledge in the South African population has not previously been reported. A prospective study of the parents of 21 children with tuberous sclerosis complex was undertaken. Median parental age was 38 (interquartile range 34.5-45) years. Parents were randomly allocated to receive written information about the condition, or to receive verbal counselling already established in clinic. A significant difference (P ¼ .001) was observed in the change in the mean knowledge scores for the parent group that received written information (34.2 at baseline, 51.7 at the second visit. This impact was higher in parents with an education level of at least grade 8 (P ¼ .003). Parental understanding of tuberous sclerosis complex can be improved by provision of written information and should be routinely available in a readily understandable format