Browsing by Author "Wanigasinghe, J."

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6542 Clinical profiles of children less than 5 years presenting with or high risk of cerebral palsy in the Western Province of Sri Lanka
(BMJ, 2024) Sumanasena, S.; Heiyanthuduwage, T.M.; Fernando, R.; Sheedy, H.S.; Jagoda, J.; Wijesekara, S.; Wanigasinghe, J.; Muttiah, N.; Rathnayake, P.; Kitnasamy, G.; Khandaker, G.
OBJECTIVES Cerebral palsy (CP) is the commonest physical disability in children globally.1 It is a clinical diagnosis based on clinical and neurological findings. International clinical practice guidelines recommend early diagnosis and CP specific interventions to invest in neural plasticity and achieve optimal functional levels.2 In the past diagnosis was confirmed at 12–24 months but now it is advanced to confirm or identify as high risk for CP before the age of six months.3 4 Sri Lanka is one of the few Asian countries that initiated a CP register and National Guidelines on management of CP.5 The objective of this paper is to describe the clinical profiles of children less than 5 years presenting to Western Province hospitals in Sri Lanka based on the data from the Sri Lanka Cerebral Palsy Register (SLCPR).METHODS A cross sectional hospital-based study was conducted in the Western Province from September 2018 – October 2021 in three teaching hospitals to collect a minimum data set for the Sri Lanka SLCPR. Data of children less than 60 months was extracted with a confirmed clinical diagnosis of CP or identified formally as ‘high risk’ of CP.Information on sociodemographic, pre/peri/neonatal, and post neonatal risk factors, and associated impairments were collected using hospital records and clinic notes. Clinical motor type, topography, and associated impairments were evaluated.RESULTS Data of 431 children were extracted, 254 (58.9%) were males. Mean age at diagnosis was 28.73 months (median 27, SD 14.98). Most children (n= 422, 97.9%) acquired CP in the pre/peri/neonatal period. The mean birth weight was 2304.4 g (median 37, SD 825.58g) and the mean POA was 35.82 months (median 37, SD 4.88). Main risk factors identified were prematurity (n=190, 44.1%), hypoxic ischaemic encephalopathy (HIE) (n= 234, 54.3%), jaundice (n=31, 7.2%) and sepsis (n= 13, 3.0%). While 183 children (42.5%) showed evidence of definitive spastic motor type, 184 (42.7%) showed predominant dyskinesia.CONCLUSION The age at diagnosis of this population from Sri Lanka is significantly lower than from other LMICs. HIE and prematurity, both preventable conditions remain the highest risk factors. Longitudinal follow up will ascertain the final motor outcomes as a higher proportion of children showed dyskinesia. The SLCPR is an important resource which will support new research towards investigating opportunities for prevention and service planning for children.
A Case of Kleine-Levin syndrome
(Sri Lanka Medical Association, 2011) Wanigasinghe, J.; Mettananda, S.G.; Kodikaraarachchi, D. S.
No Abstract Available.
Developmental profile at initial presentation in children with infantile spasms.
(Mac Keith Press, 2019) Sumanasena, S. P.; Wanigasinghe, J.; Arambepola, C.; Sri Ranganathan, S.; Muhandiram, E.
AIM: To describe the baseline developmental profile and influence of clinical and demographic factors on the developmental skills of infants diagnosed with infantile spasms. METHOD: Ninety-five infants (55 males, 40 females) newly diagnosed with infantile spasms were recruited for a cross-sectional, longitudinal study. All infants underwent Bayley Scales of Infant and Toddler Development assessments in the cognitive, receptive communication, expressive communication, and fine and gross motor developmental domains; they also underwent visual, auditory, and social behaviour assessments. Infants were categorized as 'early' (<6mo) or 'late' (≥6mo) presenters; if presented within 28 days, this was considered as 'early presentation', whereas a delay greater than 28 days was considered as a 'delay in presentation'. Antenatal, perinatal, and postnatal risk factors were identified. RESULTS: Over 90% of infants showed impairment in all domains, with the majority having severe delay; 99% showed cognitive impairment. Delayed presentation was significantly associated with receptive communication delay (odds ratio [OR]=5.35; 95% confidence interval [CI]=1.05-27.32). Onset at 6 months or less influenced auditory (OR=2.8; 95% CI=1.16-6.8) and visual (OR=3.03; 95% CI=1.22-7.57) behaviours. Neonatal infections impacted both receptive (OR=1.12; 95% CI=1.04-1.2) and expressive communication (OR=1.08; 95% CI=1.02-1.14) delay. Neonatal seizures significantly influenced visual, auditory, and social impairments. Expressive communication and gross motor development shared common perinatal risk factors. INTERPRETATION: Adverse developmental status at presentation, associated with delayed presentation and neonatal risk factors should alert clinicians to the surveillance of at-risk infants and seek out timely interventions. WHAT THIS PAPER ADDS: Ninety per cent of infants showed impaired cognitive, communication, and motor skills at presentation. Visual, auditory, and social behaviour impairments were significantly associated with perinatal risks. Visual, auditory, and social behaviour impairments were significantly associated with neonatal seizures.
Effect of intramuscular ACTH versus oral prednisolone on the developmental trajectories of children with West syndrome over 24 months: A randomised control study
(Elsevier Science-Saunders, 2021) Sumanasena, S.P.; Wanigasinghe, J.; Arambepola, C.; Sri Ranganathan, S.; Muhandiram, E.
OBJECTIVE: To assess the developmental progression and compare the developmental attainments of children treated with two hormonal therapies for infantile spasms (IS) over two years (seizure and EEG outcomes of this RCT published previously). METHODS: Newly diagnosed infants with IS were randomised to receive adrenocorticotrophin (ACTH) or prednisolone for 14 days. All underwent Bayley III Infant and Toddler Assessments in cognitive (Cog), receptive (RC) and expressive (EC) communication, fine (FM) and gross (GM) motor developmental subsets at baseline (T0), one-year (T1) and two-years (T2). RESULTS: 95 infants randomised to prednisolone (n = 48) and ACTH (n = 47) groups were eligible for developmental assessments. Mean age at initial assessment was 8.75 months (SD = 6.37, range 1.46-34.4 months). 48 children presented for all three assessments. Mean composite scores of each developmental domain improved across the three time points; but the progression was significant only in relation to motor development (p = 0.04). When comparing the treatment outcomes at 2-years, mean composite scores of children treated with ACTH were significantly lower in motor domain (p = 0.023). As for developmental delay, the ACTH group (n = 32) showed significant delay in expressive communication (adjusted OR 5.46, 95% CI: 1.1, 28.57; p = 0.04) and fine motor (adjusted OR 9.4, 95% CI: 1.1, 83.3; p = 0.04) at T2 compared to the prednisolone (n = 30) in a regression analysis. CONCLUSION: The number of children with delay at the 2 year follow up were significantly higher in two domains in the ACTH group compared to the prednisolone group. Overall results do not show a significant advantage of ACTH over prednisolone for developmental outcomes at two years, but further comparative studies over longer periods are required for more definitive conclusions. KEYWORDS: Developmental trajectories; Infantile spasms; Treatment with hormonal therapies.
Epidermal naevus syndrome: 2 cases with a rare triad
(Sri Lanka College of Paediatricians, 2013) Wanigasinghe, J.; Rodrigo, R.; Dissanayake, P.
No Abstract Available
Gillespie syndrome in a South Asian child: a case report with confirmation of a heterozygous mutation of the ITPR1 gene and review of the clinical and molecular features
(BioMed Central, 2018) de Silva, D.; Williamson, K.A.; Dayasiri, K.C.; Suraweera, N.; Quinters, V.; Abeysekara, H.; Wanigasinghe, J.; de Silva, D.; de Silva, H.
BACKGROUND: Gillespie syndrome is a rare, congenital, neurological disorder characterized by the association of partial bilateral aniridia, non-progressive cerebellar ataxia and intellectual disability. Homozygous and heterozygous pathogenic variants of the ITPR1 gene encoding an inositol 1, 4, 5- triphosphate- responsive calcium channel have been identified in 13 patients recently. There have been 22 cases reported in the literature by 2016, mostly from the western hemisphere with none reported from Sri Lanka. CASE PRESENTATION: A 10-year-old girl born to healthy non-consanguineous parents with delayed development is described. She started walking unaided by 9 years with a significantly unsteady gait and her speech was similarly delayed. Physical examination revealed multiple cerebellar signs. Slit lamp examination of eyes revealed bilateral partial aniridia. Magnetic resonance imaging of brain at the age of 10 years revealed cerebellar (mainly vermian) hypoplasia. Genetic testing confirmed the clinical suspicion and demonstrated a heterozygous pathogenic variant c.7786_7788delAAG p.(Lys2596del) in the ITPR1 gene. CONCLUSION: The report of this child with molecular confirmation of Gillespie syndrome highlights the need for careful evaluation of ophthalmological and neurological features in patients that enables correct clinical diagnosis. The availability of genetic testing enables more accurate counseling of the parents and patients regarding recurrence risks to other family members.
Hypothermia for moderate or severe neonatal encephalopathy in low-income and middle-income countries (HELIX): a randomised controlled trial in India, Sri Lanka, and Bangladesh
(Elsevier Ltd., 2021) Thayyil, S.; Pant, S.; Montaldo, P.; Shukla, D.; Oliveira, V.; Ivain, P.; Bassett, P.; Swamy, R.; Mendoza, J.; Moreno-Morales, M.; Lally, P.J.; Benakappa, N.; Bandiya, P.; Shivarudhrappa, I.; Somanna, J.; Kantharajanna, U.B.; Rajvanshi, A.; Krishnappa, S.; Joby, P.K.; Jayaraman, K.; Chandramohan, R.; Kamalarathnam, C.N.; Sebastian, M.; Tamilselvam, I.A.; Rajendran, U.D.; Soundrarajan, R.; Kumar, V.; Sudarsanan, H.; Vadakepat, P.; Gopalan, K.; Sundaram, M.; Seeralar, A.; Vinayagam, P.; Sajjid, M.; Baburaj, M.; Murugan, K.D.; Sathyanathan, B.P.; Kumaran, E.S.; Mondkar, J.; Manerkar, S.; Joshi, A.R.; Dewang, K.; Bhisikar, S.M.; Kalamdani, P.; Bichkar, V.; Patra, S.; Jiwnani, K.; Shahidullah, M.; Moni, S.C.; Jahan, I.; Mannan, M.A.; Dey, S.K.; Nahar, M.N.; Islam, M.N.; Shabuj, K.H.; Rodrigo, R.; Sumanasena, S.; Abayabandara-Herath, T.; Chathurangika, G.K.; Wanigasinghe, J.; Sujatha, R.; Saraswathy, S.; Rahul, A.; Radha, S.J.; Sarojam, M.K.; Krishnan, V.; Nair, M.K.; Devadas, S.; Chandriah, S.; Venkateswaran, H.; Burgod, C.; Chandrasekaran, M.; Atreja, G.; Muraleedharan, P.; Herberg, J.A.; Chong, W.K.K.; Sebire, N.J.; Pressler, R.; Ramji, S.; Shankaran, S.; HELIX consortium
BACKGROUND: Although therapeutic hypothermia reduces death or disability after neonatal encephalopathy in high-income countries, its safety and efficacy in low-income and middle-income countries is unclear. We aimed to examine whether therapeutic hypothermia alongside optimal supportive intensive care reduces death or moderate or severe disability after neonatal encephalopathy in south Asia. METHODS: We did a multicountry open-label, randomised controlled trial in seven tertiary neonatal intensive care units in India, Sri Lanka, and Bangladesh. We enrolled infants born at or after 36 weeks of gestation with moderate or severe neonatal encephalopathy and a need for continued resuscitation at 5 min of age or an Apgar score of less than 6 at 5 min of age (for babies born in a hospital), or both, or an absence of crying by 5 min of age (for babies born at home). Using a web-based randomisation system, we allocated infants into a group receiving whole body hypothermia (33·5°C) for 72 h using a servo-controlled cooling device, or to usual care (control group), within 6 h of birth. All recruiting sites had facilities for invasive ventilation, cardiovascular support, and access to 3 Tesla MRI scanners and spectroscopy. Masking of the intervention was not possible, but those involved in the magnetic resonance biomarker analysis and neurodevelopmental outcome assessments were masked to the allocation. The primary outcome was a combined endpoint of death or moderate or severe disability at 18-22 months, assessed by the Bayley Scales of Infant and Toddler Development (third edition) and a detailed neurological examination. Analysis was by intention to treat. This trial is registered with ClinicalTrials.gov, NCT02387385. FINDINGS: We screened 2296 infants between Aug 15, 2015, and Feb 15, 2019, of whom 576 infants were eligible for inclusion. After exclusions, we recruited 408 eligible infants and we assigned 202 to the hypothermia group and 206 to the control group. Primary outcome data were available for 195 (97%) of the 202 infants in the hypothermia group and 199 (97%) of the 206 control group infants. 98 (50%) infants in the hypothermia group and 94 (47%) infants in the control group died or had a moderate or severe disability (risk ratio 1·06; 95% CI 0·87-1·30; p=0·55). 84 infants (42%) in the hypothermia group and 63 (31%; p=0·022) infants in the control group died, of whom 72 (36%) and 49 (24%; p=0·0087) died during neonatal hospitalisation. Five serious adverse events were reported: three in the hypothermia group (one hospital readmission relating to pneumonia, one septic arthritis, and one suspected venous thrombosis), and two in the control group (one related to desaturations during MRI and other because of endotracheal tube displacement during transport for MRI). No adverse events were considered causally related to the study intervention. INTERPRETATION: Therapeutic hypothermia did not reduce the combined outcome of death or disability at 18 months after neonatal encephalopathy in low-income and middle-income countries, but significantly increased death alone. Therapeutic hypothermia should not be offered as treatment for neonatal encephalopathy in low-income and middle-income countries, even when tertiary neonatal intensive care facilities are available. FUNDING: National Institute for Health Research, Garfield Weston Foundation, and Bill & Melinda Gates Foundation. TRANSLATIONS: For the Hindi, Malayalam, Telugu, Kannada, Singhalese, Tamil, Marathi and Bangla translations of the abstract see Supplementary Materials section.
Multiple subcutaneous folds in oculocerebrorenal syndrome of Lowe
(Sri Lanka Medical Association, 2013) Wanigasinghe, J.; Mettananda, S.; de Silva, K.S.H.; Gunethilake, S.
No Abstract Available
Neurological disorders associated with COVID-19 in Sri Lanka
(BioMed Central,, 2023) Chang, T.; Wijeyekoon, R.; Keshavaraj, A.; Ranawaka, U.; Senanayake, S.; Ratnayake, P.; Senanayake, B.; Caldera, M.C.; Pathirana, G.; Sirisena, D.; Wanigasinghe, J.; Gunatilake, S.; ASN COVID-19 Study Group
BACKGROUND: Neurological manifestations of SARS-CoV-2 infection have been reported from many countries around the world, including the South Asian region. This surveillance study aimed to describe the spectrum of neurological disorders associated with COVID-19 in Sri Lanka. METHODS: COVID-19 patients manifesting neurological disorders one week prior and up to six weeks after infection were recruited from all the neurology centres of the government hospitals in Sri Lanka from May 2021 – May 2022. Data was collected using a structured data form that was electronically transmitted to a central repository. All patients were evaluated and managed by a neurologist. Data were analysed using simple descriptive analysis to characterise demographic and disease related variables, and simple comparisons and logistic regression were performed to analyse outcomes and their associations. RESULTS: One hundred and eighty-four patients with neurological manifestations associated with COVID-19 were recruited from all nine provinces in Sri Lanka. Ischaemic stroke (31%) was the commonest neurological manifestation followed by encephalopathy (13.6%), Guillain–Barre syndrome (GBS) (9.2%) and encephalitis (7.6%). Ischaemic stroke, encephalitis and encephalopathy presented within 6 days of onset of COVID-19 symptoms, whereas GBS and myelitis presented up to 10 days post onset while epilepsy and Bell palsy presented up to 20 – 40 days post onset. Haemorrhagic stroke presented either just prior to or at onset, or 10 – 25 days post onset of COVID-19 symptomatic infection. An increased frequency of children presenting with encephalitis and encephalopathy was observed during the Omicron variant predominant period. A poor outcome (no recovery or death) was associated with supplemental oxygen requirement during admission (Odds Ratio: 12.94; p=0.046). CONCLUSIONS: The spectrum and frequencies of COVID-19 associated neurological disorders in Sri Lanka were similar to that reported from other countries, with strokes and encephalopathy being the commonest. Requiring supplemental oxygen during hospitalisation was associated with a poor outcome.
Ohtahara syndrome due to bilateral perisylvian polymicrogyria
(Sri Lanka College of Paediatricians, 2014) Wanigasinghe, J.; Gamaathige, N.I.S.; Sumanasena, S.; Weerasekera, M.
No abstract available
Protocol for the Sri Lankan Cerebral Palsy Register pilot study
(BMJ Publishing Group Ltd., 2020) Heiyanthuduwage, T.M.; Sumanasena, S.P.; Kitnasamy, G.; Sheedy, H. S.; Khandaker, G.; Fernando, R.; Wijesekara, S.; Jagoda, J.; Ratnayake, P.; Wanigasinghe, J.; Mclntyre, S.; Goldsmith, S.; Waight, E.; Badawi, N.; Muhit, M.; Muttiah, N.
INTRODUCTION: Cerebral palsy (CP) describes a heterogeneous group of motor disorders resulting from disturbance in the developing brain. CP occurs in approximately 2.1 per 1000 live births in high-income countries, but in low- and middle-income countries (LMICs) the prevalence and severity of CP may be greater and aetiological risk factors different. In Sri Lanka, a LMIC, there have been no epidemiological studies of CP to date. Systematically collected data are required to identify opportunities for primary and secondary prevention, to plan and establish services to support children and adults with CP and their families and to act as a sampling frame for new research. Here we describe a pilot study protocol for a CP register in Sri Lanka. METHODS AND ANALYSIS: The aim of this study is to establish a CP register in Sri Lanka. We will use different surveillance methodologies in two provinces of Sri Lanka: hospital and community surveillance in the Western Province and community surveillance in the Eastern Province. A common record form will collect demographic, clinical and service data for children with CP <18 years living in these two provinces. Data will be transferred to a secure online data repository and used to describe the epidemiology of CP in these regions. We will describe the strengths and challenges of the surveillance mechanisms and estimate the resources required for ongoing hospital and community based surveillance in the Western and Eastern provinces and to include additional provinces across the country. ETHICS AND DISSEMINATION: This study has ethical clearance from The University of Kelaniya, National Health Research Council, the Institutional Ethics Review Committee of the Lady Ridgeway Hospital, Colombo South Teaching Hospital and the Director of the North Colombo Teaching Hospital. Results from this research will be disseminated through local and international conferences and through publications in peer-reviewed journals. KEYWORDS: developmental neurology & neurodisability; perinatology; public health; rehabilitation medicine.
Randomized, single-blind, parallel clinical trial on efficacy of oral prednisolone versus intramuscular corticotropin on immediate and continued spasm control in West syndrome
(Elsevier Science Publishing, 2015) Wanigasinghe, J.; Arambepola, C.; Sri Ranganathan, S.; Sumanasena, S.; Attanapola, G.
OBJECTIVE: A single-center, single-blind, parallel-group, randomized clinical trial was performed to test the null hypothesis that adrenocorticotropic hormone is not superior to high-dose prednisolone for treatment of newly diagnosed West syndrome. METHODS: Newly diagnosed infants with West syndrome were randomized to receive 14 days of oral prednisolone (40-60 mg/day) or a synthetically prepared intramuscular long-acting adrenocorticotropic hormone (40-60 IU/every other day [0.5-0.75 mg]) according to the United Kingdom Infantile Spasm Study protocol. They were blindly evaluated for infantile spasm remission by day 14, electroclinical remission (spasm cessation + resolution of hypsarrhythmia on a 30-minute electroencephalograph) by day 14 and continued spasm freedom for 28 days. RESULTS: Ninety-seven patients were enrolled in the study, with 48 of them receiving prednisolone and 49 receiving ACTH. There was no significant difference in the baseline characteristics or risk factors for the two treatment groups. By day 14, cessation of infantile spasms occurred in 28/48 (58.3%) infants on prednisolone compared with only 18/49 (36.7%) infants given adrenocorticotropic hormone (P = 0.03) and electroclinical remission in 21 on prednisolone compared with nine on adrenocorticotropic hormone (P = 0.007). Sustained spasm control for 28 consecutive days following electroclinical remission occurred in 15 on prednisolone compared with six on adrenocorticotropic hormone (P = 0.008). The total number of days required for spasm cessation was significantly less in those treated with prednisolone (3.85 days ± 2.4) compared with adrenocorticotropic hormone (8.65 days ± 3.7) (P = 0.001). Among patients who did not achieve remission, there was a non-significant trend toward greater quantitative reduction of spasms with prednisolone than with adrenocorticotropic hormone (P = 0.079). CONCLUSION: Synthetic adrenocorticotropic hormone of 40-60 IU/every other day did not yield superior rates of electroencephalographic or clinical remission when compared with prednisolone of 40-60 mg/day. Significantly, more patients achieved electroclinical remission when treated with prednisolone than with adrenocorticotropic hormone. Copyright © 2015 Elsevier Inc. All rights reserved.
Randomized, single-blind, parallel clinical trial on efficacy of oral prednisolone versus intramuscular corticotropin: A 12-month assessment of spasm control in west syndrome
(Elsevier-Professional Publications, 2017) Wanigasinghe, J.; Arambepola, C.; Ranganathan, S.S.; Sumanasena, S.
OBJECTIVE: We earlier completed a single-blind, parallel-group, randomized clinical trial to test the null hypothesis that adrenocorticotropic hormone (ACTH) is not superior to high-dose prednisolone for short-term control of West syndrome. We now present long-term follow-up data for spasm control for individuals who completed this earlier trial. METHODS: Infants with untreated West syndrome were randomized to receive 14 days of prednisolone (40 to 60 mg/day) or intramuscularlong-acting ACTH (40 to 60 IU every other day). They were evaluated at three, six, and 12 months to evaluate long-term spasm control. RESULTS: The total number of infants treated was 97 (48 prednisolone; 49 ACTH). All completed the treatment course. Eighty-five, 82, and 76 children were available for follow-up at three, six, and 12 months. Number lost to follow-up at each interval was not statistically different. Likelihood of spasm freedom at three months was significantly higher for prednisolone (64.6%) than for ACTH (38.8%) (P = 0.01; odds ratio = 2.9; 95% confidence interval = 1.3 to 6.6). At six months (P = 0.19) and twelve months (P = 0.13), the control of spasms was not statistically different, although a trend in favor of prednisolone was documented at both these time points (58.3% versus 44.9% for ACTH at six months and 56.2% versus 40.8% with ACTH at 12 months). After initial remission by day 14 (n = 46), the likelihood of a relapse within the next 12 months was not statistically different between the two treatment groups (P = 0.1). CONCLUSIONS: Control of spasms at three months was significantly better if initially treated with prednisolone. Control of spasms at six and 12 months was not significantly different despite a trend favoring prednisolone. Risk of relapse following initial remission was similar in the two groups.