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Fulltext Nine Hereditary Movement Disorders First Described in Asia: Their History and Evolution

Jagota P, Ugawa Y, Aldaajani Z, Ibrahim NM, Ishiura H, Nomura Y, et al.

J Mov Disord, 2023 Sep;16(3):231-247.
PMID: 37309109 DOI: 10.14802/jmd.23065

Clinical case studies and reporting are important to the discovery of new disorders and the advancement of medical sciences. Both clinicians and basic scientists play equally important roles leading to treatment discoveries for both cures and symptoms. In the field of movement disorders, exceptional observation of patients from clinicians is imperative, not just for phenomenology but also for the variable occurrences of these disorders, along with other signs and symptoms, throughout the day and the disease course. The Movement Disorders in Asia Task Force (TF) was formed to help enhance and promote collaboration and research on movement disorders within the region. As a start, the TF has reviewed the original studies of the movement disorders that were preliminarily described in the region. These include nine disorders that were first described in Asia: Segawa disease, PARK-Parkin, X-linked dystonia-parkinsonism, dentatorubral-pallidoluysian atrophy, Woodhouse-Sakati syndrome, benign adult familial myoclonic epilepsy, Kufor-Rakeb disease, tremulous dystonia associated with mutation of the calmodulin-binding transcription activator 2 gene, and paroxysmal kinesigenic dyskinesia. We hope that the information provided will honor the original researchers and help us learn and understand how earlier neurologists and basic scientists together discovered new disorders and made advances in the field, which impact us all to this day.
Fulltext Historical and More Common Nongenetic Movement Disorders From Asia

Ibrahim NM, Jagota P, Pal PK, Bhidayasiri R, Lim SY, Ugawa Y, et al.

J Mov Disord, 2023 Sep;16(3):248-260.
PMID: 37291830 DOI: 10.14802/jmd.22224

Nongenetic movement disorders are common throughout the world. The movement disorders encountered may vary depending on the prevalence of certain disorders across various geographical regions. In this paper, we review historical and more common nongenetic movement disorders in Asia. The underlying causes of these movement disorders are diverse and include, among others, nutritional deficiencies, toxic and metabolic causes, and cultural Latah syndrome, contributed by geographical, economic, and cultural differences across Asia. The industrial revolution in Japan and Korea has led to diseases related to environmental toxin poisoning, such as Minamata disease and β-fluoroethyl acetate-associated cerebellar degeneration, respectively, while religious dietary restriction in the Indian subcontinent has led to infantile tremor syndrome related to vitamin B12 deficiency. In this review, we identify the salient features and key contributing factors in the development of these disorders.
Movement disorders in Indochina: Resource challenges and future solutions

Bhidayasiri R, Sringean J, Van Le T, Lim TT, Navuth C, Phoumindr A, et al.

J Neural Transm (Vienna), 2023 Jul;130(7):875-889.
PMID: 37306791 DOI: 10.1007/s00702-023-02662-1

Movement disorders are a major cause of disability worldwide and their increasing prevalence predicts a substantial future burden of care. Impactful patient care requires availability of, and accessibility to, effective medications, knowledge, and disease awareness among both medical professionals and patients, driven by skilled personnel to harness and manage resources. The highest burden of movement disorders is in low-to-middle income countries where resources are often limited and infrastructure is insufficient to meet growing demands. This article focuses on the specific challenges faced in the management and delivery of care for movement disorders in Indochina, the mainland region of Southeast Asia comprising the neighboring countries of Cambodia, Laos, Malaysia, Myanmar, Thailand, and Vietnam. The first Indochina Movement Disorders Conference was held in August 2022 in Ho Chi Minh City, Vietnam, to provide a platform to better understand the situation in the region. Future management of movement disorders in Indochina will require progressive adaptation of existing practices to reflect modern approaches to care delivery. Digital technologies offer an opportunity to strengthen these processes and address the challenges identified in the region. Ultimately, a long-term collaborative approach by regional healthcare providers is key.
Fulltext Fecal Calprotectin in Parkinson's Disease and Multiple System Atrophy

Hor JW, Lim SY, Khor ES, Chong KK, Song SL, Ibrahim NM, et al.

J Mov Disord, 2021 Dec 24.
PMID: 34937162 DOI: 10.14802/jmd.21085

Objective: Converging evidence suggests that intestinal inflammation is involved in the pathogenesis of neurodegenerative diseases. Previous studies on fecal calprotectin in Parkinson's disease (PD) were limited by small sample sizes, and literature regarding intestinal inflammation in multiple system atrophy (MSA) is very scarce. We investigated the levels of fecal calprotectin, a marker of intestinal inflammation, in PD and MSA.
Methods: We recruited 169 subjects (71 PD, 38 MSA, and 60 age-similar nonneurological controls). Clinico-demographic data were collected. PD and MSA were subtyped and the severity assessed using the MDS-UPDRS and UMSARS, respectively. Fecal calprotectin and blood immune markers were analyzed.
Results: Compared to controls (median: 35.7 [IQR: 114.2] μg/g), fecal calprotectin was significantly elevated in PD (median: 95.6 [IQR: 162.1] μg/g, p = 0.003) and even higher in MSA (median: 129.5 [IQR: 373.8] μg/g, p = 0.002). A significant interaction effect with age was observed; between-group differences were significant only in older subjects (i.e., ≥ 61 years) and became more apparent with increasing age. A total of 28.9% of MSA and 18.3% of PD patients had highly abnormal fecal calprotectin levels (≥ 250 μg/g); however, this difference was only significant for MSA compared to controls. Fecal calprotectin correlated moderately with selected blood immune markers in PD, but not with clinical features of PD or MSA.
Conclusions: Elevated fecal calprotectin suggests a role for intestinal inflammation in PD and MSA. A more complete understanding of gut immune alterations could open up new avenues of research and treatment for these debilitating diseases.
Fulltext A KMT2B Frameshift Variant Causing Focal Dystonia Restricted to the Oromandibular Region After Long-Term Follow-up

Closas AMFD, Lohmann K, Tan AH, Ibrahim NM, Lim JL, Tay YW, et al.

J Mov Disord, 2023 Jan;16(1):91-94.
PMID: 36537064 DOI: 10.14802/jmd.22109

KMT2B-linked dystonia (DYT-KMT2B) is a childhood-onset dystonia syndrome typically beginning in the lower limbs and progressing caudocranially to affect the upper limbs with eventual prominent craniocervical involvement. Despite its recent recognition, it now appears to be one of the more common monogenic causes of dystonia syndromes. Here, we present an atypical case of DYT-KMT2B with oromandibular dystonia as the presenting feature, which remained restricted to this region three decades after symptom onset. This appears to be the first reported case of DYT-KMT2B from Southeast Asia and provides further supporting evidence for the pathogenic impact of the KMT2B c.6210_6213delTGAG variant.
Thyroid hormones: Possible roles in epilepsy pathology

Tamijani SM, Karimi B, Amini E, Golpich M, Dargahi L, Ali RA, et al.

Seizure, 2015 Sep;31:155-64.
PMID: 26362394 DOI: 10.1016/j.seizure.2015.07.021

Thyroid hormones (THs) L-thyroxine and L-triiodothyronine, primarily known as metabolism regulators, are tyrosine-derived hormones produced by the thyroid gland. They play an essential role in normal central nervous system development and physiological function. By binding to nuclear receptors and modulating gene expression, THs influence neuronal migration, differentiation, myelination, synaptogenesis and neurogenesis in developing and adult brains. Any uncorrected THs supply deficiency in early life may result in irreversible neurological and motor deficits. The development and function of GABAergic neurons as well as glutamatergic transmission are also affected by THs. Though the underlying molecular mechanisms still remain unknown, the effects of THs on inhibitory and excitatory neurons may affect brain seizure activity. The enduring predisposition of the brain to generate epileptic seizures leads to a complex chronic brain disorder known as epilepsy. Pathologically, epilepsy may be accompanied by mitochondrial dysfunction, oxidative stress and eventually dysregulation of excitatory glutamatergic and inhibitory GABAergic neurotransmission. Based on the latest evidence on the association between THs and epilepsy, we hypothesize that THs abnormalities may contribute to the pathogenesis of epilepsy. We also review gender differences and the presumed underlying mechanisms through which TH abnormalities may affect epilepsy here.
Glycogen synthase kinase-3 beta (GSK-3β) signaling: Implications for Parkinson's disease

Golpich M, Amini E, Hemmati F, Ibrahim NM, Rahmani B, Mohamed Z, et al.

Pharmacol Res, 2015 Jul;97:16-26.
PMID: 25829335 DOI: 10.1016/j.phrs.2015.03.010

Glycogen synthase kinase 3 (GSK-3) dysregulation plays an important role in the pathogenesis of numerous disorders, affecting the central nervous system (CNS) encompassing both neuroinflammation and neurodegenerative diseases. Several lines of evidence have illustrated a key role of the GSK-3 and its cellular and molecular signaling cascades in the control of neuroinflammation. Glycogen synthase kinase 3 beta (GSK-3β), one of the GSK-3 isomers, plays a major role in neuronal apoptosis and its inhibition decreases expression of alpha-Synuclein (α-Synuclein), which make this kinase an attractive therapeutic target for neurodegenerative disorders. Parkinson's disease (PD) is a chronic neurodegenerative movement disorder characterized by the progressive and massive loss of dopaminergic neurons by neuronal apoptosis in the substantia nigra pars compacta and depletion of dopamine in the striatum, which lead to pathological and clinical abnormalities. Thus, understanding the role of GSK-3β in PD will enhance our knowledge of the basic mechanisms underlying the pathogenesis of this disorder and facilitate the identification of new therapeutic avenues. In recent years, GSK-3β has been shown to play essential roles in modulating a variety of cellular functions, which have prompted efforts to develop GSK-3β inhibitors as therapeutics. In this review, we summarize GSK-3 signaling pathways and its association with neuroinflammation. Moreover, we highlight the interaction between GSK-3β and several cellular processes involved in the pathogenesis of PD, including the accumulation of α-Synuclein aggregates, oxidative stress and mitochondrial dysfunction. Finally, we discuss about GSK-3β inhibitors as a potential therapeutic strategy in PD.
Fulltext Geometric morphometric analysis of malocclusion on lateral cephalograms in Malaysian population

Woon CK, Jamal NAA, Noor MNIM, Abdullah SM, Ibrahim NM, Norman NH, et al.

Anat Cell Biol, 2020 Sep 30;53(3):378.
PMID: 32782237 DOI: 10.5115/acb.19.118e
Stroke Riskometer Application (SRA™) influence on lifestyle changes of home bound familial Malaysian stroke caregivers: a randomised controlled trial in a primary care based longer term stroke care facility

Hussin R, Aziz AFA, Ali MF, Monoto EMM, Arvinder-Singh HS, Alabed AAA, et al.

BMC Prim Care, 2023 Sep 08;24(1):181.
PMID: 37684626 DOI: 10.1186/s12875-023-02138-x

BACKGROUND: In countries where access to Specialist stroke care services are limited, primary care physicians often manage stroke patients and the caregiving family members. This study aimed to evaluate the impact of Stroke Riskometer Application (SRA™) on promoting healthier lifestyles among familial stroke caregivers for primary prevention.
METHODS: A parallel, open-label, 2-arm prospective, pilot randomised controlled trial was conducted at a long-term stroke service at a university based primary care clinic. All stroke caregivers aged ≥ 18 years, proficient in English or Malay and smartphone operation were invited. From 147 eligible caregivers, 76 participants were randomised to either SRA™ intervention or conventional care group (CCG) after receiving standard health counselling. The intervention group had additional SRA™ installed on their smartphones, which enabled self-monitoring of modifiable and non-modifiable stroke risk factors. The Stroke Riskometer app (SRATM) and Life's Simple 7 (LS7) questionnaires assessed stroke risk and lifestyle practices. Changes in clinical profile, lifestyle practices and calculated stroke risk were analysed at baseline and 3 months. The trial was registered in the Australia-New Zealand Clinical Trial Registry, ACTRN12618002050235.
RESULTS: The demographic and clinical characteristics of the intervention and control group study participants were comparable. Better improvement in LS7 scores were noted in the SRA™ arm compared to CCG at 3 months: Median difference (95% CI) = 0.88 (1.68-0.08), p = 0.03. However, both groups did not show significant changes in median stroke risk and relative risk scores at 5-, 10-years (Stroke risk 5-years: Median difference (95% CI) = 0.53 (0.15-1.21), p = 0.13, 10-years: Median difference (95% CI) = 0.81 (0.53-2.15), p = 0.23; Relative risk 5-years: Median difference (95% CI) = 0.84 (0.29-1.97), p = 0.14, Relative risk 10-years: Median difference (95% CI) = 0.58 (0.36-1.52), p = 0.23).
CONCLUSION: SRA™ is a useful tool for familial stroke caregivers to make lifestyle changes, although it did not reduce personal or relative stroke risk after 3 months usage.
TRIAL REGISTRATION: No: ACTRN12618002050235 (Registration Date: 21st December 2018).
Fulltext Factors Associated With Length of Stay for Patients With Stroke in Malaysia

Ismail A, Reffien MAM, Ibrahim NM, Sobri HNM, Abidin NDIZ, Rusli SASS, et al.

Glob J Qual Saf Healthc, 2020 Nov;3(4):134-138.
PMID: 37260578 DOI: 10.36401/JQSH-20-10

INTRODUCTION: Globally, stroke continues to become a significant public health issue contributing to one of the significant causes of morbidity and mortality. The study aimed to describe the characteristics of patients with stroke who were admitted to a teaching hospital in Malaysia and to determine the factors associated with length of stay (LOS).
METHODS: This is a single-center, cross-sectional study using in-patient data maintained by the Case-Mix Unit of a teaching hospital in Malaysia from 2016 to 2017. The study included all patients with International Classification of Disease (ICD) code 164 (stroke, not specified as hemorrhage or infarct). The significance of association was determined using nonparametric tests in the form of the Mann-Whitney U test and the Kruskal-Wallis test.
RESULTS: A total of 162 stroke patients from 2016 to 2017 from Case-Mix database were included in the study. The age ranged from 31 to 97 years old. The minimum and maximum LOS for patients with stroke ranged from 1 to 17 days. The severity of illness was found to be significantly associated with longer LOS (p < 0.001); however, age, sex, and presence of co-morbidities did not show any significant association.
CONCLUSION: Despite its limitations, this study is an essential first step to examine the characteristics of patients with stroke and to determine the factors associated with LOS.
Fulltext Multi-strain probiotics (Hexbio) containing MCP BCMC strains improved constipation and gut motility in Parkinson's disease: A randomised controlled trial

Ibrahim A, Ali RAR, Manaf MRA, Ahmad N, Tajurruddin FW, Qin WZ, et al.

PLoS One, 2020;15(12):e0244680.
PMID: 33382780 DOI: 10.1371/journal.pone.0244680

OBJECTIVE: We determined the effectiveness of a multi-strain probiotic (Hexbio®) containing microbial cell preparation MCP®BCMC® on constipation symptoms and gut motility in PD patients with constipation.
METHODS: PD patients with constipation (ROME III criteria) were randomized to receive a multi-strain probiotic (Lactobacillus sp and Bifidobacterium sp at 30 X 109 CFU) with fructo-oligosaccaride or placebo (fermented milk) twice daily for 8 weeks. Primary outcomes were changes in the presence of constipation symptoms using 9 items of Garrigues Questionnaire (GQ), which included an item on bowel opening frequency. Secondary outcomes were gut transit time (GTT), quality of life (PDQ39-SI), motor (MDS-UPDRS) and non-motor symptoms (NMSS).
RESULTS: Of 55 recruited, 48 patients completed the study: 22 received probiotic and 26 received placebo. At 8 weeks, there was a significantly higher mean weekly BOF in the probiotic group compared to placebo [SD 4.18 (1.44) vs SD 2.81(1.06); (mean difference 1.37, 95% CI 0.68, 2.07, uncorrected p<0.001)]. Patients in the probiotic group reported five times higher odds (odds ratio = 5.48, 95% CI 1.57, 19.12, uncorrected p = 0.008) for having higher BOF (< 3 to 3-5 to >5 times/week) compared to the placebo group. The GTT in the probiotic group [77.32 (SD55.35) hours] reduced significantly compared to placebo [113.54 (SD 61.54) hours]; mean difference -36.22, 95% CI -68.90, -3.54, uncorrected p = 0.030). The mean change in GTT was 58.04 (SD59.04) hour vs 20.73 (SD60.48) hours respectively (mean difference 37.32, 95% CI 4.00, 70.63, uncorrected p = 0.028). No between-groups differences were observed in the NMSS, PDQ39-SI, MDS-UPDRS II and MDS-UPDRS III scores. Four patients in the probiotics group experienced mild reversible side effects.
CONCLUSION: This study showed that consumption of a multi-strain probiotic (Hexbio®) over 8 weeks improved bowel opening frequency and whole gut transit time in PD patients with constipation.
Optimal set of EEG features for emotional state classification and trajectory visualization in Parkinson's disease

Yuvaraj R, Murugappan M, Ibrahim NM, Sundaraj K, Omar MI, Mohamad K, et al.

Int J Psychophysiol, 2014 Dec;94(3):482-95.
PMID: 25109433 DOI: 10.1016/j.ijpsycho.2014.07.014

In addition to classic motor signs and symptoms, individuals with Parkinson's disease (PD) are characterized by emotional deficits. Ongoing brain activity can be recorded by electroencephalograph (EEG) to discover the links between emotional states and brain activity. This study utilized machine-learning algorithms to categorize emotional states in PD patients compared with healthy controls (HC) using EEG. Twenty non-demented PD patients and 20 healthy age-, gender-, and education level-matched controls viewed happiness, sadness, fear, anger, surprise, and disgust emotional stimuli while fourteen-channel EEG was being recorded. Multimodal stimulus (combination of audio and visual) was used to evoke the emotions. To classify the EEG-based emotional states and visualize the changes of emotional states over time, this paper compares four kinds of EEG features for emotional state classification and proposes an approach to track the trajectory of emotion changes with manifold learning. From the experimental results using our EEG data set, we found that (a) bispectrum feature is superior to other three kinds of features, namely power spectrum, wavelet packet and nonlinear dynamical analysis; (b) higher frequency bands (alpha, beta and gamma) play a more important role in emotion activities than lower frequency bands (delta and theta) in both groups and; (c) the trajectory of emotion changes can be visualized by reducing subject-independent features with manifold learning. This provides a promising way of implementing visualization of patient's emotional state in real time and leads to a practical system for noninvasive assessment of the emotional impairments associated with neurological disorders.
Inter-hemispheric EEG coherence analysis in Parkinson's disease: assessing brain activity during emotion processing

Yuvaraj R, Murugappan M, Ibrahim NM, Sundaraj K, Omar MI, Mohamad K, et al.

J Neural Transm (Vienna), 2015 Feb;122(2):237-52.
PMID: 24894699 DOI: 10.1007/s00702-014-1249-4

Parkinson's disease (PD) is not only characterized by its prominent motor symptoms but also associated with disturbances in cognitive and emotional functioning. The objective of the present study was to investigate the influence of emotion processing on inter-hemispheric electroencephalography (EEG) coherence in PD. Multimodal emotional stimuli (happiness, sadness, fear, anger, surprise, and disgust) were presented to 20 PD patients and 30 age-, education level-, and gender-matched healthy controls (HC) while EEG was recorded. Inter-hemispheric coherence was computed from seven homologous EEG electrode pairs (AF3-AF4, F7-F8, F3-F4, FC5-FC6, T7-T8, P7-P8, and O1-O2) for delta, theta, alpha, beta, and gamma frequency bands. In addition, subjective ratings were obtained for a representative of emotional stimuli. Interhemispherically, PD patients showed significantly lower coherence in theta, alpha, beta, and gamma frequency bands than HC during emotion processing. No significant changes were found in the delta frequency band coherence. We also found that PD patients were more impaired in recognizing negative emotions (sadness, fear, anger, and disgust) than relatively positive emotions (happiness and surprise). Behaviorally, PD patients did not show impairment in emotion recognition as measured by subjective ratings. These findings suggest that PD patients may have an impairment of inter-hemispheric functional connectivity (i.e., a decline in cortical connectivity) during emotion processing. This study may increase the awareness of EEG emotional response studies in clinical practice to uncover potential neurophysiologic abnormalities.
Emotion classification in Parkinson's disease by higher-order spectra and power spectrum features using EEG signals: a comparative study

Yuvaraj R, Murugappan M, Ibrahim NM, Omar MI, Sundaraj K, Mohamad K, et al.

J Integr Neurosci, 2014 Mar;13(1):89-120.
PMID: 24738541 DOI: 10.1142/S021963521450006X

Deficits in the ability to process emotions characterize several neuropsychiatric disorders and are traits of Parkinson's disease (PD), and there is need for a method of quantifying emotion, which is currently performed by clinical diagnosis. Electroencephalogram (EEG) signals, being an activity of central nervous system (CNS), can reflect the underlying true emotional state of a person. This study applied machine-learning algorithms to categorize EEG emotional states in PD patients that would classify six basic emotions (happiness and sadness, fear, anger, surprise and disgust) in comparison with healthy controls (HC). Emotional EEG data were recorded from 20 PD patients and 20 healthy age-, education level- and sex-matched controls using multimodal (audio-visual) stimuli. The use of nonlinear features motivated by the higher-order spectra (HOS) has been reported to be a promising approach to classify the emotional states. In this work, we made the comparative study of the performance of k-nearest neighbor (kNN) and support vector machine (SVM) classifiers using the features derived from HOS and from the power spectrum. Analysis of variance (ANOVA) showed that power spectrum and HOS based features were statistically significant among the six emotional states (p < 0.0001). Classification results shows that using the selected HOS based features instead of power spectrum based features provided comparatively better accuracy for all the six classes with an overall accuracy of 70.10% ± 2.83% and 77.29% ± 1.73% for PD patients and HC in beta (13-30 Hz) band using SVM classifier. Besides, PD patients achieved less accuracy in the processing of negative emotions (sadness, fear, anger and disgust) than in processing of positive emotions (happiness, surprise) compared with HC. These results demonstrate the effectiveness of applying machine learning techniques to the classification of emotional states in PD patients in a user independent manner using EEG signals. The accuracy of the system can be improved by investigating the other HOS based features. This study might lead to a practical system for noninvasive assessment of the emotional impairments associated with neurological disorders.
Emotion processing in Parkinson's disease: an EEG spectral power study

Yuvaraj R, Murugappan M, Omar MI, Ibrahim NM, Sundaraj K, Mohamad K, et al.

Int J Neurosci, 2014 Jul;124(7):491-502.
PMID: 24168328 DOI: 10.3109/00207454.2013.860527

Although an emotional deficit is a common finding in Parkinson's disease (PD), its neurobiological mechanism on emotion recognition is still unknown. This study examined the emotion processing deficits in PD patients using electroencephalogram (EEG) signals in response to multimodal stimuli.
Validation study of the Mini-Mental State Examination in a Malay-speaking elderly population in Malaysia

Ibrahim NM, Shohaimi S, Chong HT, Rahman AH, Razali R, Esther E, et al.

Dement Geriatr Cogn Disord, 2009;27(3):247-53.
PMID: 19246909 DOI: 10.1159/000203888

BACKGROUND/AIMS: In view of the differing sensitivity and specificity of the Mini-Mental State Examination (MMSE) in the non-English-speaking populations, we conducted the first validation study of the Malay version (M-MMSE) in Malaysia among 300 subjects (from the community and outpatient clinics).
METHODS: Three versions were used: M-MMSE-7 (serial 7), M-MMSE-3 (serial 3) and M-MMSE-S (spell 'dunia' backwards). Dementia was assessed using the criteria of the Diagnostic and Statistical Manual of Mental Disorders IV. The optimal cutoff scores were obtained from the receiver operating characteristics curves.
RESULTS: Seventy-three patients (24.3%) had dementia and 227 (75.7%) were controls. Three hundred patients completed the M-MMSE-7, 160 the M-MMSE-3 and 145 the M-MMSE-S. All 3 versions were valid and reliable in the diagnosis of dementia. The optimal cutoff scores varied with each version and gender. In the control group, significant gender differences were observed in the patients with the lowest educational status. Increasing educational levels significantly improved the M-MMSE performance in both genders.
CONCLUSION: All 3 versions of the M-MMSE are valid and reliable as a screening tool for dementia in the Malaysian population, but at different cutoff scores. In those with the lowest educational background, gender-adjusted cutoff scores should be applied.
Study site: Community, neurology and dementia outpatient clinics, Perubatan Universiti Kebangsaan Malaysia (PPUKM), University Malaya Medical Centre (UMMC)
Fulltext A case of Hashimoto encephalopathy in a Malay woman with Graves disease

Ngiu CS, Ibrahim NM, Yahya WN, Tan HJ, Mustafa N, Basri H, et al.

BMJ Case Rep, 2009;2009.
PMID: 21709844 DOI: 10.1136/bcr.01.2009.1501

Hashimoto encephalopathy (HE) is a poorly recognised steroid-responsive encephalopathy, with prominent neuropsychiatric features. Diagnosis is often difficult due to its heterogeneous clinical presentation, especially since the thyroid status or anti-thyroid antibody titres may not be related to the disease state. Here, the case of a 23-year-old Malay woman with Graves disease who presented with progressive encephalopathy diagnosed as HE is presented. She responded dramatically to high dose intravenous and then oral corticosteroid. A month after the initiation of treatment, she regained full independency.
Fulltext Anti-GABAB Receptor Encephalitis Presenting with Atypical Corticobasal Syndrome in a Patient with Parkinson's Disease

Abdullah NS, Jan TH, Remli R, Mukari SAM, Ibrahim NM

J Mov Disord, 2020 Sep;13(3):235-237.
PMID: 32894902 DOI: 10.14802/jmd.20011
Knowledge and practice of burn first aid among parents of under-age children

Halil MF, Ibrahim NM, Ahmad ZNBS, Che Hasan MK

Enferm Clin, 2021 04;31 Suppl 2:S100-S104.
PMID: 33849139 DOI: 10.1016/j.enfcli.2020.09.008

The objective of this study is to assess the level of knowledge and practice of burn first aid management among parents. A cross-sectional study was conducted among parents of under-age children in the Taman Desa Darul Naim area of Pasir Tumbuh, in Kelantan, Malaysia, using an adapted survey from Davies et al. Out of 80 respondents, 46.3% showed minimal knowledge of burn first aid, 47.5% had moderate knowledge, and 6.3% extensive knowledge. There was a significant relationship between the demographic data and the knowledge of burn first aid for gender (p=0.02), but no significant relationship between the level of knowledge and level of education (p=0.29) or age (p=0.09). The findings indicate that the level of knowledge of burn first aid is limited. Education for parents of under-age children, including demonstrations of burn first aid, is needed to promote safe action and prevent further injury in the community.
Fulltext Clinical and genetic profile in index patients with spinocerebellar ataxia type 3 in Indonesia: case report

Aminah S, Huda F, Gamayani U, Pusparini I, Mochyadin MFA, Sribudiani Y, et al.

Heliyon, 2021 Jul;7(7):e07519.
PMID: 34345727 DOI: 10.1016/j.heliyon.2021.e07519

Spinocerebellar ataxia (SCA) is an autosomal dominant hereditary disease with progressive course, and no causal therapy. Diagnostics are still challenging, due to facility and protocols, and so as in Indonesia. As a national referral center, Dr. Hasan Sadikin Central General Hospital has received a lot of patients from all over Indonesia, particularly from Western Java. Study related to SCA (including clinical and genetic profile) is still limited in Indonesia. We identified index patients from three families with ataxia, hence intend to determine their clinical and genetic pattern. The hereditary pattern is autosomal dominant. Scale for the assessment and rating of ataxia (SARA) shows mild and moderate ataxia. Inventory of non-ataxia signs (INAS) scores of the patients were 3, 5 and 6. Montreal cognitive assessment-Indonesian version (MOCA-INA) shows only one patient has mild cognitive impairment, despite young age. Barthel index shows 1 subject has moderate dependency. Mutation in Ataxin3 polyQ repeats shows pathologically long CAG repeats, 72,10; 72,10; and 72,23 respectively in mutant and wild type allele. We diagnosed the index patients with spinocerebellar ataxia type 3. This study is the first case series study in Indonesia. The hereditary pattern is clearly shown as an autosomal dominant ataxia. The clinical and genetic profile was varied, and the symptom is progressive and deteriorates overtime, including wide based gait, speech problem, motor and sensor complaint, and cognitive decline complaint. Despite the same polyQ stretch length, the onset and clinical characteristics of patients are diverse.

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