MuSK-Myasthenia Gravis Unmasked by Hydroxychloroquine.

Introduction: Muscle-specific tyrosine kinase (MuSK) antibody positive myasthenia gravis (MuSK-MG) is a rare clinical disorder, and diagnosing it can be challenging. Most of the patients present with predominant facial, oculo-bulbar, and neck muscle weakness along with respiratory muscle involvement. Such a presentation can be mistaken for bulbar onset motor neuron disease or as one of the rare oculopharyngeal myopathies. Case Report. We present a young female patient, who reported to us with neck muscle weakness, ocular and bulbar muscle paralysis, and breathing difficulty. She had been healthy till she was prescribed hydroxychloroquine (HCQ) tablets (400 mg per day) for a malar rash. By the end of the second week after commencing the HCQ therapy, she developed the muscle weakness. Her symptoms began to regress after stopping HCQ and starting steroids, pyridostigmine, and, subsequently, azathioprine. She was negative for anticholinesterase receptor antibodies (AChR-Ab) but was positive for MuSK antibodies (MuSK-Ab). Conclusion: This report proves that MuSK-MG can also be unmasked by HCQ administration. Awareness of drug-induced/-unmasked MG is important, as failure to do so may result in a severe morbidity and a fatal outcome. The offending drug has to be promptly discontinued, and appropriate treatment should be instituted.

Impact of Covid-19 on the therapeutic plasma exchange service within the South East Asian region: Consensus recommendations and global perspectives.

INTRODUCTION: Therapeutic plasma exchange (TPE) for neuroimmunological disorders has played an increasingly important role within the Southeast Asian (SEA) region. The South East Asian Therapeutic Plasma exchange Consortium (SEATPEC) was formed in 2018 to promote education and research on TPE within the region. The advent of the Covid-19 pandemic has produced challenges for the development and expansion of this service. METHODOLOGY: A qualitative and semi-quantitative questionnaire-based survey was conducted by SEATPEC member countries from January to June 2020 (Phase 1) and then from July 2020 to January 2021 in (Phase 2) to assess the impact of Covid-19 on regional TPE. OBJECTIVES: The study's main objectives were to explore the challenges experienced and adaptations/adjustments taken by SEATPEC countries in order to continue safe and efficient TPE during the Covid-19 pandemic. RESULTS: The pandemic was found to disrupt the delivery of TPE services in all SEATPEC countries. Contributing factors were multifactorial due to overstretched medical services, staff shortages, quarantines and redeployments, fear of acquiring Covid-19, movement restriction orders, and patient's psychological fear of attending hospitals/testing for Covid-19. All SEATPEC countries practiced careful stratification of cases for TPE (electives vs emergencies, Covid-19 vs non-Covid-19 cases). SEATPEC countries had to modify TPE treatment protocols to include careful preprocedure screening of patient's for Covid-19, use of personal protective equipment (PPE) and post-TPE sanitization of machines and TPE suites. CONCLUSION: Based on the responses of the survey, SEATPEC countries produced a consensus statement with five recommendations for safe and effective TPE within the region.

Ataxic gait.

Ataxia as the main complaint in a young patient prompts to diagnose neurodegenerative diseases. Not all ataxias are irreversible. We present a video of a patient in the clinic with ataxia diagnosed as subacute combined degeneration of the cord.

Human tuberculosis brain promotes neuronal apoptosis but not in astrocytes with high expression of vascular endothelial growth factor.

The present study aimed to investigate the involvement of the angiogenic marker vascular endothelia growth factor (VEGF) and apoptotic markers of Bcl-2 and Bax in the neurons and astrocytes in the brain infected by Mycobacterium tuberculosis. The immunohistochemistry staining was performed to analyze the expression of the VEGF, Bcl-2 and Bax in the astrocytes and neurons. The expression of VEGF was high in neurons and astrocytes in both the infected brain and control tissues with no difference of angiogenic activity (p = 0.40). Higher Bcl-2 expression was seen in astrocytes of infected brain tissues compared to the control tissues (p = 0.004) promoted a higher anti-apoptotic activity in astrocytes. The neurons expressed strong Bax expression in the infected brain tissues compared to the control tissues (p < 0.001), which indicated more apoptosis in neurons. Thus, neuronal death and survival of infected astrocytes together with high expression of VEGF might be associated with formation of brain tuberculosis. In conclusion, neurons could be more vulnerable than astrocytes in human tuberculosis brain with high expression of VEGF.

Epilepsy Surgery in Hospital Universiti Sains Malaysia: Our Experiences since 2004.

Epilepsy surgery has been performed by a few centres in Malaysia, including Hospital Universiti Sains Malaysia (HUSM). To date, a total of 15 patients have undergone epilepsy surgery in HUSM. The epilepsy surgery included anterior temporal lobectomy (ATL) with amygdalohippocampectomy (AH) and Vagal nerve stimulation (VNS). The surgical outcomes of the patients were assessed using the International League Against Epilepsy (ILAE) outcome scale. The ILAE scores for patients who underwent ATL with AH were comparatively better than those who underwent VNS. One of the patient who underwent ATL with AH and frontal lesionectomy was found to have psychosis during follow up. Epilepsy surgery has proven to be an important treatment for medically resistant epilepsy. Thus it is important to raise public awareness regarding epilepsy and its treatment.

Updates on Knowledge, Attitude and Preventive Practices on Tuberculosis among Healthcare Workers.

Ranking as the most communicable disease killer worldwide, tuberculosis, has accounted with a total of 9.6 million new tuberculosis cases with 1.5 million tuberculosis-related deaths reported globally in 2014. Tuberculosis has remain as an occupational hazard for healthcare workers since 1920s and due to several tuberculosis outbreaks in healthcare settings in the early 1990s, the concern about the transmission to both patients and healthcare workers has been raised. Healthcare workers have two to three folds greater the risk of active tuberculosis than the general population. Several studies on knowledge, attitude and practices on tuberculosis among healthcare workers worldwide have revealed that majority of the participated healthcare workers had good knowledge on tuberculosis. Most of the healthcare workers from South India and South Africa also reported to have positive attitude whereas a study in Thailand reported that most of the healthcare providers have negative attitude towards tuberculosis patients. Nevertheless, majority of the healthcare workers have low level of practice on tuberculosis prevention. An improved communication between healthcare workers and the patients as well as their families is the key to better therapeutic outcomes with good knowledge, attitude and preventive practice towards tuberculosis.

Validity and reliability of the Malay version of the Structured Migraine Interview (SMI) Questionnaire.

BACKGROUND: The Structured Migraine Interview (SMI) is a valid and reliable instrument for migraine diagnosis. However, a Malay version of the SMI is not available to be applied to the local Malaysian population. This study was designed to access the validity and reliability of a new Malay version of the SMI questionnaire. METHODS: Patients with headache attending the Neurology Clinic, Hospital Universiti Sains Malaysia, Kelantan, Malaysia, were screened against the inclusion/exclusion criteria before recruitment. A standard translation procedure was used to translate and adapt the questionnaire into the Malay language. The translated version was tested for face, content and construct validities. Subsequently, validity and reliability studies were conducted (1(st) compilation), followed by retesting seven days later (2(nd) compilation). RESULTS: A total of 157 patients between 15 and 60 years of age were enrolled in this study. The kappa value was 0.70 (p < 0.001) with high sensitivity (0.97) and specificity (0.63). The misclassification rate was 0.15, with a positive predictive value of 0.82 and a negative predictive value of 0.92. The positive likelihood ratio was 2.62, while the negative likelihood ratio was 0.05. The Cronbach's alpha was 0.93 (1(st) compilation) and 0.90 (2(nd) compilation), respectively. The Spearman's correlation coefficient ranged from 0.86 (Question 4) to 0.95 (Question 1). The overall concordance for item 1 was very high (97%), followed by item 4 (83%), item 2 (71%) and finally item 3 (64%). CONCLUSION: The Malay version of the SMI questionnaire is comparable to the English version in terms of validity and reliability. It was highly reliable with good internal consistency and can be used for the diagnosis of migraine in clinical settings in Malaysia.

Prevalence of aspirin resistance in diabetic patients and its associated factors.

BACKGROUND: Aspirin resistance has posed a major dilemma in the prevention of cardiovascular disease and stroke. There have been many factors that have been associated with aspirin resistance. Among these factors, the inflammatory processes of diabetes and glycaemic control have been significantly associated with aspirin resistance. Our study evaluated the prevalence of aspirin resistance and its associated factors. METHODS: This was a cross-sectional, interventional study, which was implemented from October to November 2012 at the Hospital Universiti Sains Malaysia (HUSM). Sixty-nine patients with diabetes who were taking aspirin were enrolled. The glycosylated haemoglobin (HbA1c) and C-reactive protein (CRP) levels were measured in these patients. The thromboelastography (TEG) level was measured using a TEG machine by a trained technician employing standard methods. The variables obtained were analysed for prevalence of aspirin resistance, HbA1c, CRP, and TEG level. The Chi-square test (and Fisher exact test where applicable) were used to evaluate the associations between aspirin resistance with glycaemic control (HbA1c) and inflammatory markers (CRP). RESULTS: The prevalence of aspirin resistance was 17.4% (95%; CI 9.3, 28.4). Glycaemic control (HbA1c) and inflammatory markers (CRP) were not associated with aspirin resistance. Aspirin resistance was prevalent in our study population and was comparable to other studies. The mean HbA1c in the aspirin-resistant group was 8.9%, whereas the mean HbA1c in the aspirin-sensitive group was 8.6%. CONCLUSION: There was no significant difference in HbA1c between the two groups. There was no significant association between CRP levels and aspirin resistance.

Validity and reliability of the Bahasa Melayu version of the Migraine Disability Assessment questionnaire.

BACKGROUND: The study was designed to determine the validity and reliability of the Bahasa Melayu version (MIDAS-M) of the Migraine Disability Assessment (MIDAS) questionnaire. METHODS: Patients having migraine for more than six months attending the Neurology Clinic, Hospital Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia, were recruited. Standard forward and back translation procedures were used to translate and adapt the MIDAS questionnaire to produce the Bahasa Melayu version. The translated Malay version was tested for face and content validity. Validity and reliability testing were further conducted with 100 migraine patients (1st administration) followed by a retesting session 21 days later (2nd administration). RESULTS: A total of 100 patients between 15 and 60 years of age were recruited. The majority of the patients were single (66%) and students (46%). Cronbach's alpha values were 0.84 (1st administration) and 0.80 (2nd administration). The test-retest reliability for the total MIDAS score was 0.73, indicating that the MIDAS-M questionnaire is stable; for the five disability questions, the test-retest values ranged from 0.77 to 0.87. CONCLUSION: The MIDAS-M questionnaire is comparable with the original English version in terms of validity and reliability and may be used for the assessment of migraine in clinical settings.

An Unusual Association between Unilateral Intracranial Vessels Occlusion with Iron Deficiency Anaemia and Alpha-Thalassemia Trait: A Case Report.

We report a 33-year-old Malay woman presented with acute left dense hemiparesis and an NIHSS score of 11/15. Computed tomography (CT) scan brain showed a massive right middle cerebral artery (MCA) territory infarct. The right internal carotid artery (ICA) and right proximal MCA were shown occluded from digital substraction angiography (DSA). Carotid dissection, carotid canal anomaly, and intercavernous communication were systematically ruled out. She had no risk factors for atherosclerosis. The connective tissue screening and thrombophilic markers were negative. However, she was anaemic on admission and subsequent investigations revealed that she had alpha-thalassemia and iron deficiency anaemia. The right ICA remained occluded from a repeat CT cerebral angiogram after one year, but otherwise she was neurologically stable. This case illustrates an unusual association between intracranial vessel occlusion with iron deficiency anaemia and alpha-thalassemia trait.

Intracerebral haematomas after deep brain stimulation surgery in a patient with Tourette syndrome and low factor XIIIA activity.

A 24-year-old male patient with refractory Tourette syndrome was treated with deep brain stimulation (DBS) and developed subsequent bilateral subcortical haematomas. Additional blood tests revealed abnormalities of plasma factor XIIIA and tryptophan levels, which may be associated with Tourette syndrome. Neurosurgeons who perform DBS surgery on patients with Tourette syndrome must be aware of possible disastrous complications resulting from factor XIIIA disorders of blood haemostasis. Routine screening for this condition is not typically performed prior to surgery in these patients.

Coexistence of CACNA1A, ATP1A2, and KCNN3 gene mutation in migraine patients with human platelet polymorphism.

OBJECTIVE: To look for any possible coexistence of CACNA1A, ATP1A2, and KCNN3 gene mutations in migraine patients who had human platelet HPA-1a/1b polymorphism, which is also known as PlA1/A2 polymorphism. METHODS: The study was carried out at the Neurology Clinic, Hospital University Sains Malaysia, Kelantan, Malaysia between April 2004 and March 2005. The DNA from 4 patients who had migraine with the HPA1a/1b polymorphism were analyzed by polymerase chain reaction using the allele specific oligonucleotide technique to detect the presence of CACNA1A, ATP1A2, and KCNN3 genotypes. RESULTS: We found that the CACNA1A gene mutation alone was present in only one patient who presented with classical migraine with aura. The gene mutations on ATP1A2 and KCNN3 were seen in none of our 4 cases with migraine. CONCLUSION: There is no coexistence between the platelet HPA-1a/1b polymorphism and the ATP1A2 and KCNN3 gene mutations, though one classical migraine patient with HPA-1a/1b polymorphism had the CACNA1A gene mutation. Larger studies are warranted to confirm these findings.