ABSTRACT
Objective: To determine the validity and reliability of the Chinese parent proxy and child self-report health related quality of life measure for children with epilepsy (CHEQOL-25) in Malaysia. Methods: Face and content validity of the Chinese parent proxy and child self-report CHEQOL-25 was verified by an expert panel, and piloted in five children with epilepsy (CWE). The Chinese CHEQOL-25 was then administered to 40 parent proxies and their CWE (aged 8-18 years), from two tertiary hospitals, at baseline and 2 weeks later. Results: Forty parents and their CWE were recruited. Cronbach’s alpha for each subscale ranged from 0.56-0.83. At test-retest, the interclass correlation for all items ranged from 0.68-0.97. Items 8 and 25 were removed as their corrected item-total correlation values were <0.3. Epilepsy severity, the number of anti-epileptic drugs taken daily, number of close friends and number of time spent with friends were found to be associated with the parent proxy CHEQOL-25 score. Duration of epilepsy, child’s cognitive ability, number of close friends and number of time spent with friends were associated with child self-report CHEQOL-25. The parent proxy and the child selfreport showed high to fair agreement on the “interpersonal/social” [Intraclass correlation coefficient (ICC)=0.670, p<0.001] and “epilepsy secrecy” subscale (ICC=0.417, p=0.048). Conclusions: Our small study found that the Chinese CHEQOL-25 was a valid and reliable questionnaire to assess the quality of life of children with epilepsy from the parent prospective and child self-report when items 8 and 25 were removed.
Subject(s)
EpilepsyABSTRACT
Background & Objective: Posterior reversible encephalopathy syndrome (PRES) is associated with immunosuppressive agents used in children with haemato-oncological diseases. There are no reports to date from the South Asia and South East Asia region. We report a Malaysian tertiary centre case series of children with haemato-oncological disease who developed PRES. Methods: Retrospective study of children seen with haemato-oncology diseases seen at the University Malaya Medical Centre Kuala Lumpur who developed PRES from 2011 – 2013. Clinical details were obtained from medical records and brain neuroimaging was reviewed. Results: Five patients met the inclusion criteria. All 5 patients had significant hypertension acutely or subacutely prior to neurology presentation. Four presented with acute seizures and the remainder 1 presented with encephalopathy.Three patients were on chemotherapy, 1 had renal impairment and 1 had prior immunosupression for bone marrow transplantation. A full recovery was seen in 4 patients and 1 patient had mild residual quadriplegia. Conclusion: Our case series expands the clinico-radiological spectrum of PRES in children with underlying haemato-oncological disorders. It is the first to show that prior cyclosporin intake as long as 2 months is a potential risk factor for PRES. Clinicians need to be vigilant for development of PRES and closely monitor the blood pressure in these children who are receiving or recently had immunosuppressive drugs and present with acute neurological symptoms.
Subject(s)
Posterior Leukoencephalopathy Syndrome , Brain DiseasesABSTRACT
Children with epilepsy are at increased risk of behavioral problems and psychiatric disorders compared with the general population and those with other chronic illnesses. Depression and anxiety disorders are the most common and require further attention because they carry the risk of reduced quality of life and have far reaching consequences in later life. The wide range of prevalence quoted in the literature refl ects methodologic differences. Risk factors are often multifactorial and include illness severity, coexisting morbidity, and psychosocial factors. Diagnosis remains a challenge, as the presentation is heterogenous and age related. This is complicated by epilepsy issues and associated comorbidity that hamper the use of standardized assessment tools. Multimodal treatment involving psychotherapy and antidepressant therapy currently represents the best approach. The mental health needs of these children are largely unmet; increased awareness and better research are required to determine best practices.
ABSTRACT
There is growing evidence that antiepileptic drugs used in children that are meant to control seizures can actually cause seizure exacerbate instead. To minimize this problem, clinicians should be familiar with the epilepsy syndromes and the appropriate choice of antiepileptic drugs, avoid certain antiepileptic drugs which consistently aggravate specifi c seizure types and maintain a high index of suspicion of those at high risk as seizure exacerbation can present in diverse forms.
ABSTRACT
Dystrophinopathies commonly present as Duchenne or Becker muscular dystrophy but rare, unusual phenotypes have also been described. We have identifi ed three Malaysian boys with an unusual form of dystrophinopathy, presenting with exercise-induced cramps and myoglobinuria, but with no apparent muscle weakness. Immunohistochemistry for dystrophin and genetic analysis confi rmed the diagnosis. The frequency of this phenotype is unknown but there have been several case reports. Consistent with these reports, we also found that two of our patients had deletions in the rod domain of dystrophin, which has been suggested to be associated with this unusual manifestation