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.
Posterior Leukoencephalopathy Syndrome ; Brain Diseases

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.
Epilepsy

Childhood chronic inflammatory demyelinating polyneuropathy (CIDP) can be misdiagnosed for the more common genetic neuropathies such as Charcot-Marie-Tooth (CMT) disease. We present a case of childhood-onset demyelinating polyneuropathy who was initially diagnosed as CMT before a revised diagnosis of CIDP was made. A 14-year-old boy with bilateral pes cavus presented with progressive history of ataxic gait, generalized areflexia and proprioceptive sensory loss. Nerve conduction studies showed demyelinating features including markedly slow motor conduction velocities and prolonged distal motor latencies resembling CMT1. Despite the absence of a family history of genetic neuropathies, a diagnosis of CMT1 was considered most likely. The patient presented two years later with an acute onset of worsening instability and muscle weakness. A detailed history revealed functional improvement following the last presentation along with two separate episodes of exacerbations suggesting a relapsingremitting form of neuropathy. Cerebrospinal fluid analysis showed cytoalbuminergic dissociation. Nerve ultrasound demonstrated enlarged peripheral nerves, particularly in the proximal and non-entrapment sites. Genetic testing was negative for known mutations in common CMT genes. A course of intravenous immunoglobulin resulted in clinically significant improvement. In conclusion, our patient highlights the diagnostic challenges in childhood-onset demyelinating neuropathies and the importance of not missing a potentially treatable immune-mediated neuropathy.