Impulse control disorders and other non-motor symptoms in Sri Lankan patients with Parkinson's disease.

The impact of non-motor symptoms is often overlooked in favour of the motor symptoms when managing Parkinson's disease resulting in suboptimal patient outcomes. This study aimed to characterise the non-motor symptoms of Parkinson's disease in a cohort of Sri Lankan patients with a special focus on the impulsive control disorders and other compulsive behaviours (ICDs-CB) that had not been previously studied in this population. All patients with idiopathic Parkinson's disease followed up at the National Hospital of Colombo, Sri Lanka were included. The presence or absence of non-motor symptoms and their perceived impact was recorded with an interviewer administered questionnaire. Symptoms of anxiety and depression were assessed with Hamilton Anxiety and Depression scales. Presence of ICDs-CB was assessed with the questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease. Of 192 patients 97% (186) reported at least 2 non-motor symptoms. About 83% (160/192) screened positive for anxiety, 40% (76/192) for depression, and 17% (32/192) for an ICDs-CB. A lower Barthel index, history of past psychiatric disorders and family history of alcohol abuse were independent predictors of ICDs-DB. Managing both motor and non-motor symptoms are important to preserve the quality of life of patients with Parkinson's disease. They should be screened for symptoms for anxiety and depression regularly during follow up and educated about the possibility of ICDs-CB soon after diagnosis.

Surgical Outcome of Pharmaco Refractory Epilepsy in the National Epilepsy Center of Sri Lanka.

BACKGROUND: The National Epilepsy Center (NEC) in Sri Lanka was established in 2017. Seizure outcome, effects on quality of life (QOL) and surgical complications among nonpediatric patients who underwent epilepsy surgery from October 2017 to February 2023 are described. METHODS: Nineteen patients (≥14 years) underwent epilepsy surgery at the NEC. We used Engel classification and Quality of Life in Epilepsy 31 (QOLIE-31) questionnaire to assess seizure outcome and QOL respectively. Surgical complications were categorized into neurological and complications related to surgery. RESULTS: Nine female and 10 male patients underwent surgery (mean age 27.5 years (range 14-44 years). The mean follow-up duration was 10.5 months (range 6-55 months). Twelve patients underwent temporal lobe resections. At 6-months follow-up, 83.3% (10/12) had favorable seizure outcomes with Engel class I/II. At 1-year follow-up 6/8 patients (75.0%) and at 2-year follow-up, 5/7 patients (71.4%) had a favorable outcome. Seven patients had extra-temporal lobe surgeries and one defaulted. Seizure freedom was observed in 6/6 at 6 months, 3/3 at 1-year, and 2/2 at 2-year follow-up. Five patients (26.3%) experienced minor post-operative surgical site infection. Two (11.1%) had persistent quadrantanopia. Meaningful improvement in QOL (change in QOLIE-31 score ≥11.8) was observed irrespective of seizure outcome or type of surgery (P < 0.001). CONCLUSIONS: Epilepsy surgery is effective in developing countries. Seizure outcomes in our patients are comparable to those worldwide. Clinically important QOL improvement was observed in our series. This is the first published data on epilepsy surgery outcomes in nonpediatric patients from Sri Lanka.

Neurological disorders associated with COVID-19 in Sri Lanka.

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.

Estimation of body weight using anthropometric parameters in Sri Lankan hospitalized adult patients.

Body weight is an important clinical parameter for accurate dosing of drugs with a narrow therapeutic window, However, it is difficult to measure the body weight of a patient if they cannot stand on a scale. There are several anthropometrics-based equations to estimate the body weight, but most of these are derived from white Caucasian populations and are not validated for South Asians. This study aimed to validate existing anthropometrics-based weight estimation equations and develop a new equation for the same purpose for Sri Lankan adults. This prospective study was conducted at the National Hospital of Sri Lanka over a 6-month period, split into a development and a validation phase. During the development phase, estimated body weight of patients by doctors and nurses and patients themselves were noted and compared against their actual body weight. In addition, 13 anthropometric measurements were taken, which were used to validate 12 anthropometrics-based equations to estimate body weight described in literature previously. Two new gender specific regression models to estimate the body weight in the local population was also derived and validated. A total of 502 (males = 249) and 217 (males = 108) patients were recruited for the development and validation phases respectively. Both doctors and patients had comparable accuracy in predicting body weight (p>0.05). All anthropometric based equations were significantly correlated with actual body weight (correlation coefficients: 0.741-0.869), and the new equations derived from the local data performed similarly to the best performing equation identified from the literature during validation phase. However, even the best of these equations could not outperform patient/physician estimates. When the patient weight cannot be measured, an estimate by the patient or the doctor may be the best substitute.

Gene therapy for selected neuromuscular and trinucleotide repeat disorders - An insight to subsume South Asia for multicenter clinical trials.

Background: In this article, the authors discuss how they utilized the genetic mutation data in Sri Lankan Duchenne muscular dystrophy (DMD), Spinal muscular atrophy (SMA), Spinocerebellar ataxia (SCA) and Huntington's disease (HD) patients and compare the available literature from South Asian countries to identifying potential candidates for available gene therapy for DMD, SMA, SCA and HD patients. Methods: Rare disease patients (n = 623) with the characteristic clinical findings suspected of HD, SCA, SMA and Muscular Dystrophy were genetically confirmed using Multiplex Ligation Dependent Probe Amplification (MLPA), and single plex PCR. A survey was conducted in the "Wiley database on Gene Therapy Trials Worldwide" to identify DMD, SMA, SCA, and HD gene therapy clinical trials performed worldwide up to April 2021. In order to identify candidates for gene therapy in other neighboring countries we compared our findings with available literature from India and Pakistan which has utilized the same molecular diagnostic protocol to our study. Results: From the overall cohort of 623 rare disease patients with the characteristic clinical findings suspected of HD, SCA, SMA and Muscular Dystrophy, n = 343 (55%) [Muscular Dystrophy- 65%; (DMD-139, Becker Muscular Dystrophy -BMD-11), SCA type 1-3-53% (SCA1-61,SCA2- 23, SCA3- 39), HD- 52% (45) and SMA- 34% (22)] patients were positive for molecular diagnostics by MLPA and single plex PCR. A total of 147 patients in Sri Lanka amenable to available gene therapy; [DMD-83, SMA-15 and HD-49] were identified. A comparison of Sri Lankan finding with available literature from India and Pakistan identified a total of 1257 patients [DMD-1076, SMA- 57, and HD-124] from these three South Asian Countries as amenable for existing gene therapy trials. DMD, SMA, and HD gene therapy clinical trials (113 studies) performed worldwide up to April 2021 were concentrated mostly (99%) in High Income Countries (HIC) and Upper Middle-Income Countries (UMIC). However, studies on the potential use of anti-sense oligonucleotides (ASO) for treatment of SCAs have yet to reach clinical trials. Conclusion: Most genetic therapies for neurodegenerative and neuromuscular disorders have been evaluated for efficacy primarily in Western populations. No multicenter gene therapy clinical trial sites for DMD, SMA and HD in the South Asian region, leading to lack of knowledge on the safety and efficacy of such personalized therapies in other populations, including South Asians. By fostering collaboration between researchers, clinicians, patient advocacy groups, government and industry in gene therapy initiatives for the inherited-diseases community in the developing world would link the Global North and Global South and breathe life into the motto "Together we can make a difference".

Adaptation and Validation of a Sinhala version of the Radbound Oral Motor Inventory (ROMP) for Parkinson's disease.

Background: The Radboud Oral Motor Inventory for Parkinson's disease (ROMP) is a patient-rated assessment measuring patients' perceptions of speech, swallowing, and saliva control among patients with idiopathic Parkinson's disease (IPD). Objective: The present study was carried out to adapt and validate the Sinhala version of the ROMP questionnaire in a Sinhala-speaking patient cohort diagnosed with IPD. Materials and Methods: The study population consisted of patients diagnosed with IPD attending a tertiary care neurology clinic at the National Hospital of Sri Lanka. ROMP was translated from English to Sinhala, and an expert committee verified its content. Construct validity was assessed by correlating the Sinhala ROMP scores with the subscales in speech, salivation, and swallowing of the Unified Parkinson's Disease Rating Scale and with five-point Likert-type scale to assess dysarthria, dysphagia, and drooling by a speech and language therapist. Test-retest reproducibility was assessed by repeating the questionnaire in 2 weeks. Results: A cohort of 21 patients was evaluated (male to female ratio = 2.5:1, mean age was 58.8 [±8.3] years). The Spearman's correlations between ROMP and the Likert-type scale assessment, that is, speech r = 0.85 (P < 0.01), swallowing r = 0.86 (P < 0.01), and drooling r = 0.88 (P < 0.01), and subscales of the UPDRS were statistically significant, that is, speech r = 0.75 (P < 0.01), swallowing r = 0.96 (P < 0.01), and salivation r = 0.94 (P < 0.01). Reproducibility of the three domains and total intraclass correlation coefficients indicated a high level of agreement in test-retest reproducibility (range: 0.98-0.99). The three subdomains of the instrument also had excellent internal consistency (total Cronbach's α = 0.99). Conclusion: The Sinhala version of ROMP has proved to be a good assessment tool for dysphagia, dysarthria, and drooling in the early stage of IPD patients.

Post-Stroke Depression: Prevalence, Associated Factors, and Relationship to Disability in a Tertiary Care Center in Sri Lanka.

Background and Objectives The prevalence of stroke in urban Sri Lanka is estimated at 10.4 per 1000 and is expected to rise. Post-stroke depression (PSD) is an independent predictor of poor long-term outcomes. It leads to suboptimal rehabilitation, decreased quality of life, and increased mortality and is under-recognized. The main objectives of this study were to estimate the prevalence of depression in stroke, assess factors associated with PSD, and assess the relationship of PSD to disability. Materials and Methods A descriptive cross-sectional study was conducted at the Neurology and Medical Ward, National Hospital of Sri Lanka. Non-probability, consecutive sampling was used to collect data from patients with ischemic stroke admitted from January 2019 to January 2020. Patients with significant pre-existing depression, cognitive impairment, and language deficits were excluded. A structured, pre-tested interviewer-administered questionnaire was used to assess the prevalence and associated factors of PSD. Beck's Depression Inventory (BDI) was administered 3 months following the stroke to screen for depression. Modified Rankin Score (MRS) was used to assess disability on admission, discharge, and at 3 months. Results Eighty-one stroke patients were screened. The mean age was 66.6 years (±standard deviation [SD]: 12.5). Male:female ratio was 1.2:1. Depression at 3 months of follow-up was observed in 35.8% (95% confidence interval [CI]: 25.4-47.2%) of participants. Following bivariate analysis, large vessel stroke ( p < 0.001), cortical stroke ( p < 0.001), frontal lobe lesions ( p < 0.001), history of past stroke ( p = 0.014), and sexual dysfunction ( p = 0.026) were associated with increased risk of PSD. The odds of a person with severe disability developing PSD was 7.9 times more than a person with a less severe disability at discharge from hospital and at 3 months of follow-up (odds ratio [OR] =7.9; 95% CI: 2.7-23.3, p = 0.000). Conclusions PSD occurs in one-third of strokes, keeping with previous studies. The risk of having PSD is higher among patients with severe disabilities. The difference in risk factors identified compared with previous studies can be attributable to differences in methodology. Identifying risk factors for post-stroke depression is essential to mitigate the poor outcome.

Outcomes of stroke patients undergoing thrombolysis in Sri Lanka; an observational prospective study from a low-middle income country.

BACKGROUND: Stroke related deaths are relatively higher in low- and middle-income countries where only a fraction of eligible patients undergo thrombolysis. There is also limited evidence on post-thrombolysis outcomes of patients from Asian countries in these income bands. METHODS: This is a single center prospective observational study of a patient cohort with acute ischaemic stroke, undergoing thrombolysis with alteplase (low and standard dose), over a 24-month period in 2019/2020. Modified Rankin scale (mRS) for dependency at 3 months (primary outcome), duration of hospital stay, incidence of symptomatic intracranial haemorrhages and all-cause mortality at 3 months (secondary outcomes) were recorded. Demographic, clinical and treatment related factors associated with these outcomes were explored. RESULTS: Eighty-nine patients (males - 61, 69%, mean age: 60 years ±12.18) were recruited. Time from symptom onset to reperfusion was 174 min ± 56.50. Fifty-one patients were independent according to mRS, 11 (12.4%) patients died, and 11 (12.5%) developed symptomatic intracranial haemorrhages by 3 months. Functional independence at 3 months was independently associated with National Institutes of Health Stroke Scale (NIHSS) on admission (p < 0.05). Thrombolysis with low dose alteplase did not lead to better or worse outcomes compared to standard dose. CONCLUSIONS: On admission NIHSS is predictive of functional independence at 3 months post-thrombolysis. Low dose alteplase may be as efficacious as standard dose alteplase with associated cost savings, but this needs to be confirmed by a prospective clinical trial for the Sri Lankan population.

Slow-Channel Congenital Myasthenic Syndrome due to a Novel Mutation in the Acetylcholine Receptor Alpha Subunit in a South Asian: A Case Report.

Congenital myasthenic syndromes (CMS) result from genetic mutations that cause aberrations in structure and/or function of proteins involved in neuromuscular transmission. The slow-channel CMS (SCCMS) is an autosomal dominant postsynaptic defect caused by mutations in genes encoding alpha, beta, delta, or epsilon subunits of the acetylcholine receptor resulting in a functional defect which is an increase of the opening time of the receptor. We report a case of SCCMS due to a heterozygous mutation in the M2 domain of the AChR alpha subunit - CHRNA1:ENST00000348749.6:exon7:c.806T>G:p.Val269Gly and corresponding kinetic defect. A substitution of valine with phenylalanine in the same position has been previously described. This is the first reported case of a new CHRNA1 variant in a patient with SCCMS from South Asia. We also highlight the phenotype that would favour a genetic basis over an autoimmune one, in an adult presenting with fatigable weakness.

Contrast-induced encephalopathy: a complication of coronary angiography.

Contrast-induced encephalopathy is a rare idiosyncratic reaction to contrast material. A 56-year-old woman with hypertension developed a hemiparesis with confusion and disorientation 3 hours after routine coronary angiography. The procedure had been prolonged, and during it she had received 130 mL of iopromide contrast. A metabolic screen was negative, and cerebral angiography and MR scan of brain were normal. She recovered completely by day 5. Contrast-induced encephalopathy should be considered in patients developing focal neurological deficits following coronary angiography. Patients requiring investigations to exclude acute stroke in this setting should not receive additional intravenous or intra-arterial contrast, although MR with gadolinium appears safe. Better awareness of this complication should avoid potentially harmful interventions such as thrombolysis.

Subacute sclerosing panencephalitis: a rare cause of acute cortical visual impairment in an adult.

Adult onset subacute sclerosing panencephalitis (SSPE) after the third decade of life is rare and the manifestations can mimic disorders such as dysmyelinating and metabolic disorders. This case report presents a patient with acute binocular visual impairment in his fifth decade as the first manifestation of SSPE. This is preventable with immunisation against measles. A prior history of measles infection may not be forthcoming in adult onset SSPE. This should be kept in mind as a differential diagnosis when a patient from endemic area presents with acute visual loss even in the absence of classic features. Periodic generalised discharges on the EEG without myoclonus may be seen in this condition rarely as in this case.

What We Fail to See in Neuro-Genetic Diseases: A Bird's Eye View from the Developing World.

BACKGROUND: Progressive neurological genetic diseases are not rare. They cause psychosocial damages to its victims. This article focuses on common psychosocial issues faced by those from the developing world. METHODS: A multicentre observational survey of 246 patients from teaching hospitals in Sri Lanka. Participants were clinically and genetically confirmed by neurologists and the Interdisciplinary Centre for Innovation in Biotechnology and Neuroscience (ICIBN) respectively from 2014 to 2018. Convenience sample with random geographical distribution. Factors were equally weighted. ANOVA, Student's t-test and chi-square analysis were used. Statistical Software R Statistics-version 3.5 and one-sample t-test with CI = 95% was used. This study meets the ethical guidelines of the local institutional review boards which are in compliance with the Helsinki Declaration. RESULTS: Sample included 184 males and 62 females of 3-76 years with either Duchenne muscular dystrophy (n=121), spinocerebellar ataxia (n = 87) or Huntington disease (n = 38). Mean income of the affected is lower than the standard average monthly income (P ≤ .001). Consultation visits depend on the monthly income (CI 20421.074-34709.361; P ≤ .001). CONCLUSION: Poverty is inversely proportionate to the patients' living conditions. As developing countries are financially challenged, it is a societal challenge to rebuild our values to enhance their living status.

Phencyclidine: A Rare Cause of Saccadic Intrusions.

Saccadic intrusions such as opsoclonus and ocular flutter are often due to a paraneoplastic or a parainfectious condition. Toxins/drugs may rarely cause them. Herein, we report a rare case of ocular flutter/opsoclonus due to phencyclidine (PCP) toxicity. Our patient is a 21-year-old male who presented with a 3-day history of headache, generalized ill health, and aggressive behavior. He was admitted with reduced level of consciousness following generalized seizures. He had features of sympathetic overactivity with ocular flutter and opsoclonus. Urine toxicology was positive for PCP. Despite supportive care, he succumbed to complications of rhabdomyolysis. Several drugs including cocaine, phenytoin, lithium, and amitriptyline are known to cause ocular flutter/opsoclonus rarely. It is poorly described with PCP. This case highlights PCP as a rare cause of toxin-induced saccadic intrusions and attempts to postulate its pathogenesis. Moreover, our report is the first case of PCP intoxication in Sri Lanka and one of the few documented reports in the South Asian region. Therefore, it represents a significant worrisome alarm about the spread of this substance in this region.

Tongue fasciculations with denervation pattern in osmotic demyelination syndrome: a case report of diagnostic dilemma.

BACKGROUND: The pathogenesis of osmotic demyelination syndrome is not completely understood and usually occurs with severe and prolonged hyponatremia, particularly with rapid correction. It can occur even in normonatremic patients, especially who have risk factors like alcoholism, malnutrition and liver disease. Bilateral tongue fasciculations with denervation pattern in electromyogram is a manifestation of damage to the hypoglossal nucleus or hypoglossal nerves. Tongue fasciculations were reported rarely in some cases of osmotic demyelination syndrome, but the exact mechanism is not explained. CASE PRESENTATION: A 32-year-old Sri Lankan male, with a history of daily alcohol consumption and binge drinking, presented with progressive difficulty in walking, dysphagia, dysarthria and drooling of saliva and alteration of consciousness. On examination he was akinetic and rigid resembling Parkinsonism with a positive Babinski sign. Clinical features were diagnostic of osmotic demyelination syndrome and MRI showed abnormal signal intensity within the central pons and basal ganglia. He also had tongue fasciculations. The electromyogram showed denervation pattern in the tongue with normal findings in the limbs. Medulla and bilateral hypoglossal nerves were normal in MRI. CONCLUSION: We were unable to explain the exact mechanism for the denervation of the tongue, which resulted in fasciculations in this chronic alcoholic patient who developed osmotic demyelination syndrome. The hypoglossal nuclei are located in the dorsal medulla and radiologically undetected myelinolysis of the medulla is a possibility. Hypoglossal nerve damage caused by methanol or other toxic substances that can contaminate regular ethyl alcohol is another possibility, as it is known to cause neurological and radiological features similar to osmotic demyelination syndrome with long-term exposure. So these toxic substances might play a role in chronic alcoholic patients with central pontine myelinolysis.

Case report of hyperglycemic nonketotic chorea with rapid radiological resolution.

BACKGROUND: Hemichorea is a rare manifestation of nonketotic hyperglycemia that usually affects elderly Asian women with poor glycemic control. Non-contrast computerized Tomography and T1- weighted Magnetic Resonance Imaging shows characteristic hyperintense basal ganglia lesions. CASE PRESENTATION: A Fifty-seven year old Sri Lankan female presented with a two-day history of right upper limb chorea. She had been diagnosed with diabetes mellitus one year ago, but was not on any treatment and did not have any micro vascular or macro vascular complications. Random blood sugar was 420 mg/dl and full blood count, liver function tests, renal function tests, inflammatory markers, thyroid function tests, Urine protein / creatinine ratio, electrocardiogram and 2D Echo were normal. Arterial blood gas did not show acidosis and ketone bodies were not detected in urine. Non-contrast computerized Tomography brain on day 1 showed left side hyperdense lentiform and caudate nuclei and MRI on day 3 showed slightly high signal intensity of left side basal ganglia on T1- weighted images and low signal intensity on T2-weighted and Fluid-attenuated inversion recovery images. She was started on insulin and a low dose of clonazepam and glycemic control was achieved on day 3. Two days later, the chorea completely disappeared. CT brain was repeated 4 days and 10 days following glycemic control, which showed rapid resolution of CT changes. Clonazepam was stopped in 2 weeks and chorea did not recur. CONCLUSION: This is a rare manifestation of diabetes in Sri lanka and diagnosing this rare entity will direct clinicians to achieve optimum glycemic control as the treatment which will lead to rapid clinical response without any other medications. In this case report we high light that with the clinical improvement, repeating a CT scan even after a very short period like 2 weeks will show rapid radiological resolution. This repeat imaging can also be useful to confirm the diagnosis, which will minimize unnecessary investigations and treatments. Further cases of hyperglycemic nonketotic chorea with brain imaging performed within short intervals is needed to evaluate the nature of rapid radiological changes, which will be useful to understand the pathology of this condition.

Complete ophthalmoplegia, complete ptosis and dilated pupil due to internal carotid artery dissection: as the first manifestation of Takayasu arteritis.

BACKGROUND: Takayasu arteritis is a rare, chronic large vessel vasculitis involving the aorta and its primary branches. As the disease progresses, the active inflammation of large vessels leads to dilation, narrowing and occlusion of the arteries. Arterial dissection is due to separation of the layers of the arterial wall resulting in a false lumen, where blood seeps into the vessel wall. Neurological sequelae of intracranial arterial dissection results from cerebral ischemia due to thromboembolism and hypo perfusion. Internal carotid artery dissection in Takayasu arteritis is very rare and complete ophthalmoplegia due to internal carotid artery dissection is also rare. This is the first case report of Takayasu arteritis presenting as complete ophthalmoplegia due to internal carotid artery dissection. CASE PRESENTATION: A 38-year-old Sri Lankan female presented with sudden onset severe headache, fixed dilated pupil, complete ptosis and ophthalmoplegia on the right side. On imaging, dissection and dilatation was evident in the right internal carotid artery from the origin up to the cavernous segment. She also had stenosis and aneurysmal dilatation of right subclavian artery. Takayasu arteritis was diagnosed subsequently. She was started on aspirin and high dose steroids. CONCLUSIONS: Internal carotid artery dissection within the cavernous sinus can lead to third, fourth and sixth nerve palsy due to compression, stretching and ischemia from occlusion of the nutritional arteries. This case report illustrates that internal carotid artery dissection should be a differential diagnosis in palsies of the third, fourth, or sixth cranial nerves, especially when associated with headache. In cases of internal carotid artery dissection, vasculitis such as Takayasu arteritis should also be considered.

Clinical and serological study of myasthenia gravis using both radioimmunoprecipitation and cell-based assays in a South Asian population.

BACKGROUND: Identification of autoantibodies has defined distinct clinico-immuno-pathological subtypes of myasthenia gravis (MG) such as AChR-antibody-positive-MG and MuSK-antibody-positive-MG. The use of more sensitive assays such as the cell-based assay (CBA) is expected to reduce the proportion of seronegative-MG. We studied the seroprevalence of AChR and MuSK antibodies using both radioimmunoprecipitation (RIA) and CBA amongst MG patients in Sri Lanka and related their antibody status to their clinical subtypes and severity. METHODS: 113 patients with MG attending Neurology units in the district of Colombo were studied. Clinical data were obtained using an interviewer-administered questionnaire and medical records. The severity of MG was assessed according to MGFA clinical grading. RIA and CBA were used to detect serum AChR and MuSK antibodies. Patients with other neurological diseases were recruited as controls. RESULTS: We detected either AChRAb (85%) or MuSKAb (6.2%) in 91.2% of MG patients. Complementing the RIA with the CBA improved the diagnostic power of detecting AChRAbs by 21% and MuSKAbs by 77%. The clinical characteristics and the occurrence of thymic pathology were similar to other populations except for a male preponderance (1.5:1). The AChRAb titer appeared to parallel the clinical severity. Seven of 11 (63.6%) patients with AChRAb-negative generalized MG had MuSK-MG. CONCLUSIONS: Clinical characteristics of MG in Sri Lanka are similar to other populations. Complementing the RIA with CBA increases the diagnostic power of detecting pathogenic autoantibodies.

Challenging epilepsy with antiepileptic pharmacotherapy in a tertiary teaching hospital in Sri Lanka.

The goal of antiepileptic drug (AED) therapy is to achieve a seizure-free state and eliminate the medical and psychosocial risks of recurrent seizures. Burden of epilepsy on the economy of a country may be largely due to the expenditure on AEDs. The adverse effects may influence the compliance to AEDs and effective control of epilepsy. We determined the pattern of AED use, the degree of epileptic control achieved and the adverse effects experienced by the epileptics in a Tertiary Teaching Hospital in Sri Lanka. Carbamazepine was found to be the most frequently used AED. Monotherapy was used on 70.8% of subjects. 86.27% of the study sample had achieved effective control of epilepsy with a 50% or more reduction in seizure frequency. Of them 72.64% were on monotherapy and they were either on carbamazepine, sodium valproate, phenytoin sodium or phenobarbitone. None of the new AEDs were prescribed to these patients. 50.9% on monotherapy and 51.5% on polytherapy reported adverse effects. Somnolence followed by headache was found to be the most frequently reported adverse effects by those on monotherapy and polytherapy both. This study shows that most epileptics can be effectively managed with the conventional AEDs with clinical monitoring.