Top of Basilar Artery Occlusion Thromboembolism in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Treated with Intravenous Thrombolysis and Mechanical Thrombectomy.

Introduction This case report illustrates the complexities of arrhythmogenic right ventricular cardiomyopathy (ARVC) and its thromboembolic complications resulting in top-of-basilar artery syndrome. We discuss the case of a 37-year-old male with ARVC who presented with acute onset of dizziness, imbalance, and vomiting, leading to the diagnosis of a top-of-basilar artery occlusion which was successfully treated. Case presentation This case highlights the diagnostic and acute treatment challenges in basilar artery occlusion (BAO) due to its non-specific symptoms and emphasizes the critical role of CT angiography in detecting occlusive thrombi for timely intervention.

Ocular Myasthenia gravis: determining the predictive factors of secondary generalisation.

Introduction: Myasthenia gravis (MG) can be classified according to clinical features into ocular MG (OMG) and generalised MG (GMG). However, OMG carries the risk of conversion to GMG. In this study, we aimed to determine the predictive factors for the secondary generalisation of OMG patients.

Multistep modeling applied to a Malaysian ALS registry.

Objective: To apply the multistep model of pathogenesis in amyotrophic lateral sclerosis (ALS) to data from a multiethnic Malaysian registry.

Methods: Clinical data, including age at symptom onset, was collected from 289 patients who presented to our multidisciplinary clinic from 2016 until 2024. A least squares linear regression model was constructed from the logarithm of approximated incidence and the logarithm of age.

Clinical Characteristics and Outcomes of Generalized Myasthenia Gravis in Malaysia: A Single-Center Experience.

Background And Purpose: Myasthenia gravis (MG) is clinically heterogeneous and can be classified into subgroups according to the clinical presentation, antibody status, age at onset, and thymic abnormalities. This study aimed to determine the clinical characteristics and outcomes of generalized MG (GMG) patients based on these subgroups.

Methods: Medical records of MG patients from 1976 to 2023 were reviewed retrospectively.

Botulinum toxin A injection for post-stroke upper limb spasticity and rehabilitation practices from centers across Asian countries.

Purpose: Describe real-life practice and outcomes in the management of post-stroke upper limb spasticity with botulinum toxin A (BoNT-A) in Asian settings.

Methods: Subgroup analysis of a prospective, observational study (NCT01020500) of adult patients (≥18 years) with post-stroke upper limb spasticity presenting for routine spasticity management, including treatment with BoNT-A. The primary outcome was goal attainment as assessed using goal-attainment scaling (GAS).

Evaluating disease status in idiopathic inflammatory myopathies with quantitative muscle ultrasound.

Introduction/aims: Muscle strength, functional status, and muscle enzymes are conventionally used to evaluate disease status in idiopathic inflammatory myopathies (IIM). This study aims to investigate the role of quantitative muscle ultrasound in evaluating disease status in IIM patients.

Methods: Patients with IIM, excluding inclusion body myositis, were recruited along with age- and sex-matched healthy controls (HC).

Quantitative muscle ultrasound as a disease biomarker in hereditary transthyretin amyloidosis with polyneuropathy.

Introduction: There is an increasing need for a reproducible and sensitive outcome measure in patients with hereditary transthyretin amyloidosis (ATTRv) with polyneuropathy (PN) due to the emergence of disease modifying therapies. In the current study, we aimed to investigate the role of quantitative muscle ultrasound (QMUS) as a disease biomarker in ATTRv-PN.

Methods: Twenty genetically confirmed ATTRv amyloidosis patients (nine symptomatic, 11 pre-symptomatic) were enrolled prospectively between January to March 2023.

Nerve Ultrasound Score in Chronic Inflammatory Demyelinating Polyneuropathy.

: Studies have suggested that, by applying certain nerve ultrasound scores, demyelinating and axonal polyneuropathies can be differentiated. In the current study, we investigated the utility of ultrasound pattern sub-score A (UPSA) and intra- and internerve cross-sectional area (CSA) variability in the diagnostic evaluation of demyelinating neuropathies. : Nerve ultrasound was performed in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and acute inflammatory demyelinating polyneuropathy (AIDP) and compared to patients with axonal neuropathies.

Autoantibody profile in a Malaysian cohort of chronic inflammatory demyelinating polyneuropathy.

We report on our cohort of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) who fulfilled the 2010 diagnostic criteria of CIDP. Patients were consecutively recruited and their demographics, clinical features and serological analysis of autoantibodies against neurofascin (NF)-155, NF-186, contactin-1 (CNTN1) and contactin-associated protein 1 were obtained. A total of 26 patients for which there was serologic testing were included: 22 typical CIDP, 3 distal CIDP and 1 multifocal CIDP.

Mutation analysis of SOD1, C9orf72, TARDBP and FUS genes in ethnically-diverse Malaysian patients with amyotrophic lateral sclerosis (ALS).

Recent studies have identified SOD1, FUS, TARDBP and C9orf72 as major ALS-related genes in both European and Asian populations. However, significant differences exist in the mutation frequencies of these genes between various ancestral backgrounds. This study aims to identify the frequency of mutations in the common causative ALS genes in a multi-ethnic Malaysian cohort.

A model incorporating ultrasound to predict the probability of fast disease progression in amyotrophic lateral sclerosis.

Objective: We aimed to develop a model to predict amyotrophic lateral sclerosis (ALS) disease progression based on clinical and neuromuscular ultrasound (NMUS) parameters.

Methods: ALS patients were prospectively recruited. Muscle fasciculation (≥2 over 30-seconds, examined in biceps brachii-brachialis (BB), brachioradialis, tibialis anterior and vastus medialis) and nerve cross-sectional area (CSA) (median, ulnar, tibial, fibular nerve) were evaluated through NMUS.

A survey on patients' disease perception and the impact of the COVID-19 pandemic on persons living with amyotrophic lateral sclerosis in Malaysia.

To investigate the patients' perception of their disease, its management and the impact of the COVID-19 pandemic on persons living with amyotrophic lateral sclerosis (ALS) in Malaysia. An online survey comprising 42 questions was conducted on ALS patients during the peak of the COVID-19 pandemic. Responses were received from 37/60 (62%) participants with ALS directly or through their caregivers.

Hereditary transthyretin amyloidosis in multi-ethnic Malaysians.

We report the clinical and genetic characteristics of hereditary transthyretin amyloidosis in the multi-ethnic Malaysian population. Subjects with genetically confirmed transthyretin amyloidosis seen between 2001 till August 2020 were included. There were 30 patients and 14 asymptomatic carriers, of which 26 (59.

Determining the Utility of the Guillain-Barré Syndrome Classification Criteria.

Background And Purpose: Several variants of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) exist, but their frequencies vary in different populations and do not always meet the inclusion criteria of the existing diagnostic criteria. However, the GBS classification criteria by Wakerley and colleagues recognize and define the clinical characteristics of each variant. We applied these criteria to a GBS and MFS cohort with the aim of determining their utility.

The utility of ALS staging systems in a multi-ethnic patient cohort.

To compare two ALS staging systems, King's clinical staging and Milano-Torino (MiToS) functional staging, using prospective data from a multi-ethnic cohort of ALS patients. The stages of disease were determined prospectively based on existing definitions. The two systems were compared for timing of stages using box plots, correspondence using chi-square tests and association using Spearman's rank correlation.

Large fiber peripheral neuropathy in systemic sclerosis: A prospective study using clinical and electrophysiological definition.

Aim: The reported prevalence of peripheral neuropathy in systemic sclerosis (SSc) is variable between 0.01% to 28%, probably due to differences in sample size, study design and population. Our aim is to determine the prevalence of large fiber peripheral neuropathy in SSc and to identify any contributing factors.

Neuropathic Pain after Adolescent Idiopathic Scoliosis Correction Surgery.

Study Design: Prospective study.

Purpose: To investigate the prevalence and the associated risk factors of chronic neuropathic pain symptoms using painDETECT questionnaire in adolescent idiopathic scoliosis (AIS) patients who underwent posterior spinal fusion (PSF) surgery.

Overview Of Literature: Post-lumbar surgery syndrome is a disease entity that describes neuropathic pain following spinal surgery.

Natural history and clinical features of ALS in Malaysia.

Studies from multiethnic populations are rarely reported but do indicate differences in phenotypic presentation and survival in amyotrophic lateral sclerosis (ALS). In this study, we aimed to investigate the natural history of a cohort of ALS patients from a multiethnic population. : Data from ALS patients presenting to our multidisciplinary ALS clinic were prospectively collected from January 2015 to June 2020 as part of an ongoing hospital-based patient registry.

Prolonged Exercise Test in Patients With History of Thyrotoxicosis.

Purpose: Thyrotoxic periodic paralysis is characterized by recurrent episodes of reversible, severe proximal muscle weakness associated with hypokalemia and hyperthyroidism. Prolonged exercise test is an easy, noninvasive method of demonstrating abnormal muscle membrane excitability in periodic paralyses. Although abnormal in thyrotoxic periodic paralysis patients, the effects thyroid hormone levels in non-thyrotoxic periodic paralysis thyrotoxicosis patients have not been well studied.

Diagnosis of Guillain-Barré syndrome and validation of the Brighton criteria in Malaysia.

We aimed to evaluate the key diagnostic features of Guillain-Barré syndrome (GBS) in Malaysian patients and validate the Brighton criteria. This was a retrospective study of patients presenting with GBS and Miller Fisher syndrome (MFS) between 2010 and 2019. The sensitivity of the Brighton criteria was evaluated.