Chee Geap Tay

Vincristine-induced peripheral neuropathy in survivors of childhood acute lymphoblastic leukaemia

Vincristine, an essential component of childhood acute lymphoblastic leukaemia (ALL) therapeutic protocols, is associated with dose‐dependent neurotoxicity, but its long‐term morbidity in treated children has not been clearly elucidated. The aim of this study is to determine the prevalence of vincristine‐induced peripheral neuropathy (VIPN) among Malaysian childhood ALL survivors and its impact on motor function and quality of life.

Longitudinal extensive transverse myelitis with cervical epidural haematoma following dengue virus infection

BACKGROUND Longitudinal extensive transverse myelitis associated with dengue infection is rare with no reported paediatric cases. METHODS We report a 12-year-old girl who presented with flaccid quadriplegia 8 days after onset of acute dengue fever. MRI spine showed T2 hyperintensity associated with epidural hematoma at C3-C6 level of the spinal cord. Transcranial magnetic brain stimulation revealed absent motor evoked potentials bilaterally. We also summarise and compare the reported cases of transverse myelitis associated with dengue infection. RESULTS Immunomodulatory treatment was given which included pulse methylprednisolone, intravenous immunoglobulin and plasmapharesis. Six months post-admission, there was a good (near-complete) clinical recovery with the repeat MRI showing mild residual hyperintensity at C4 level and complete resolution of epidural haematoma. CONCLUSION This is the first reported paediatric case of longitudinal extensive transverse myelitis following dengue infection. It is also the first to illustrate that in patients with concomitant epidural haematoma a good outcome is possible despite not having surgical decompression. Clinicians should be aware of parainfectious dengue-related longitudinal extensive transverse myelitis in children and consider prompt immunomodulatory treatment.

Succinic Semialdehyde Dehydrogenase Deficiency in a Chinese Boy A Novel ALDH5A1 Mutation With Severe Phenotype

Succinic semialdehyde dehydrogenase deficiency is a rare autosomal recessive disorder affecting catabolism of the neurotransmitter gamma-aminobutyric acid (GABA), with a wide range of clinical phenotype. We report a Malaysian Chinese boy with a severe early onset phenotype due to a previously unreported mutation. Urine organic acid chromatogram revealed elevated 4-hydroxybutyric acid. Magnetic resonance imaging (MRI) of the brain demonstrated cerebral atrophy with atypical putaminal involvement. Molecular genetic analysis showed a novel homozygous 3-bp deletion at the ALDH5A1 gene c.1501_1503del (p.Glu501del). Both parents were confirmed to be heterozygotes for the p.Glu501del mutation. The clinical course was complicated by the development of subdural hemorrhage probably as a result of rocking the child to sleep for erratic sleep-wake cycles. This case illustrates the need to recognize that trivial or unintentional shaking of such children, especially in the presence of cerebral atrophy, can lead to subdural hemorrhage.

Post-dengue encephalopathy and Parkinsonism.

Parkinsonism as a neurologic manifestation of dengue infection is rare with only 1 reported case in an adult patient. We report a case of a 6-year-old child with self-limiting post-dengue encephalopathy and Parkinsonism. This is the first reported pediatric case of post-dengue Parkinsonism and expands the neurologic manifestations associated with dengue infection in children. Clinicians should consider the possibility of post-dengue Parkinsonism in children with a history of pyrexia from endemic areas of dengue.

Sulthiame-induced drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome

BACKGROUND Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and potentially life-threatening acute drug-induced hypersensitivity reaction. Antiepileptic drugs (AEDs) predominantly aromatic AEDs are commonly reported in DRESS. To date there are no reports of sulthiame AED causing DRESS syndrome. METHOD We report a 10-year-old girl of Indian descent with AED resistant epilepsy on maintenance sodium valproate and clonazepam. Sulthiame AED was initiated to try to improve her seizure control. Five weeks after commencing sulthiame, she developed fever with a diffuse erythematous morbilliform maculopapular rash, elevated transaminases and atypical lymphocytes. At day 3 of illness, she deteriorated with worsening elevation of liver transaminases, thrombocytopenia, progression of rash, hepatosplenomegaly, pneumonitis and markedly elevated inflammatory markers. Immunomodulatory treatment of pulse methylprednisolone was given from day 7 which was associated with improvement inflammatory markers and complete resolution of rash from day 30 of illness. RESULTS The diagnosis of sulthiame-induced DRESS syndrome was made based on clinical, laboratory and skin histology findings. She was HLA-B heterozygous for HLA-B∗15:123 and 15:240 and HLA-A homozygous for HLA-A∗11:01:09. Both these HLA-A and HLA-B typing has not been reported before in cutaneous drug reactions. CONCLUSION This is the first reported case of sulthiame-induced DRESS syndrome. Our case expands the list of possible susceptible HLA alleles associated with cutaneous drug reactions. It also raises the awareness of possible DRESS syndrome among patients commenced on sulthiame who will require immediate discontinuation of sulthiame and consideration of prompt treatment of corticosteroids.

Cross-cultural adaptation and validation of the Malay language version of the TZO-AZL Preschool Children Quality of Life questionnaire: A health-related quality of life instrument for preschool children

The aim of this study is to evaluate the psychometric properties of the translated Malay language version of TZO-AZL Preschool Children Quality of Life (TAPQOL) questionnaire in preschool children. Preterm children and term children aged between two and five years were enrolled into the study. The Malay language version of TAPQOL and a set of questions regarding the child’s health status were answered by the caregivers. The internal consistency, Spearman’s correlation coefficients and principal component analysis (PCA) with Varimax rotation and Mann–Whitney U test for group comparison were employed to evaluate the psychometric properties of this instrument. A total of 258 children (120 preterm children and 138 term children) were recruited to this study with a response rate of 94%. All (sub)domains except one had Cronbach’s α coefficients of more than .7. The Spearman’s correlation coefficients between 12 subdomains were generally low. PCA supported the structural unidimensionality of the items in the instrument. Preterm children had lower quality of life scores than that of term children. Malay version of TAPQOL has multidimensional construct. It is a reliable and valid instrument for preschool children, with almost similar psychometric properties to the original version.

Transient Parkinsonism Following Mycoplasma pneumoniae Infection With Normal Brain Magnetic Resonance Imaging (MRI)

Parkinsonism caused by infection is uncommon in children. We report 2 previously healthy children with acute self-limiting parkinsonism following Mycoplasma pneumoniae infection, with normal brain magnetic resonance imaging (MRI). Our case report expands the phenotype of parkinsonism associated with M pneumoniae infection. We recommend that children with acute parkinsonism preceded by a period of febrile illness, even with a normal brain MRI, should be investigated for M pneumoniae infection.

A probable case of poliomyelitis imported to Malaysia

We report a previously well 10-month-old Somalian girl who acquired asymmetric lower limb weakness in July 2013 in Mogadishu, Banadir, before arriving in Malaysia at 12 months of age. In May 2013, there was a wild poliomyelitis outbreak in that area, as reported by the World Health Organization. Laboratory investigation, including cerebrospinal fluid, was unremarkable, and electrophysiological studies showed active axonal denervation in the left lower limb. The whole spine T2-weighted MRI revealed non-enhancing hyperintensities of the bilateral anterior horn cells, predominantly on the left side at T11-12. The viral isolations from two stool specimens at her presentation to our centre, 2 months after the onset of illness and 2 weeks apart, were negative. Despite lacking the acute virological evidence of poliomyelitis, in view of the girls clinical, electrophysiological and classical spinal neuroradiological features, together with her temporal relationship with a World Health Organization reported wild poliomyelitis outbreak, we believe these findings are consistent with a diagnosis of imported poliomyelitis. A review at 30 months of age showed persistent left lower limb monoplegia with little recovery. Our patient reiterates the importance of maintaining awareness of wild polio importation, and keeping abreast of the latest news of global poliomyelitis outbreaks when treating patients with flaccid paralysis, even if they arrive from non-endemic poliomyelitis areas.

Central precocious puberty secondary to hypothalamic hamartoma

Central precocious puberty (CPP) presenting at a very young age is likely to have an underlying pathology. One of the pathologies is hypothalamic hamartoma (HH), a non-neoplastic tumour-like lesion located at the floor of the third ventricle, near the tuber cinereum. Two young children with CPP due to HH without gelastic seizures or mental retardation were successfully managed and described in this report.