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1.
J Clin Apher ; 38(4): 437-446, 2023 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-36896493

RESUMEN

INTRODUCTION: Therapeutic plasma exchange (TPE) for neuroimmunological disorders has played an important role in the Southeast Asian region. This study investigates the challenges of performing TPE within the region. METHOD: A questionnaire-based survey was conducted and launched to 15 South East Asian Therapeutic Plasma Exchange Consortium (SEATPEC) members from seven countries in January 2021. It included demographics, TPE techniques, indications, challenges, timing, outcome measurement, and access to laboratory testing in each local center. RESULTS: A total of 15 neurologists from 12 participating centers were included. They usually perform five sessions of TPE (100.0%), with 1 to 1.5 plasma volume (93.3%), and exchanges via the central catheter (100.0%). Acute relapses of neuromyelitis optica spectrum disorder and myasthenia gravis are the most common indications. They used a combination of normal saline and 5% albumin (60.0%) as replacement fluid. Most (66.7%) used TPE as an add-on treatment in steroid-refractory cases or as first-line treatment for severe attacks. They suggested assessing the TPE efficacy of TPE by the interval to the next attack, post-TPE relapse rates, and TPE-related complications. The major challenges within our region are expense, reimbursibility, and access to TPE. CONCLUSION: Although countrywise differences exist, all share similarities regarding methods, indications, timing, obstacles, and challenges of TPE for neuroimmunological conditions. Regional collaboration will be essential to identify strategies to reduce these barriers to access to TPE in the future.


Asunto(s)
Enfermedades Autoinmunes del Sistema Nervioso , Intercambio Plasmático , Humanos , Miastenia Gravis/terapia , Intercambio Plasmático/métodos , Plasmaféresis , Estudios Retrospectivos , Pueblos del Sudeste Asiático , Enfermedades Autoinmunes del Sistema Nervioso/terapia
2.
J Clin Apher ; 36(6): 849-863, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34694652

RESUMEN

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.


Asunto(s)
COVID-19 , Intercambio Plasmático , Asia Sudoriental/epidemiología , COVID-19/complicaciones , COVID-19/epidemiología , COVID-19/terapia , Consenso , Humanos , Enfermedades del Sistema Nervioso/complicaciones , Enfermedades del Sistema Nervioso/terapia , Neurólogos , Pandemias , Intercambio Plasmático/métodos , Intercambio Plasmático/estadística & datos numéricos , SARS-CoV-2 , Encuestas y Cuestionarios
3.
J Clin Apher ; 34(4): 434-444, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-30829434

RESUMEN

There has been an increase in the use of therapeutic plasma exchange (TPE) in immune-mediated neurological disorders in recent years. However, accessibility and availability of TPE remains low and costly, especially for a country with limited healthcare funding like Malaysia. With expanding clinical indications in neurological disorders, and increasingly expensive conventional immunomodulatory treatment such as intravenous immunoglobulin and monoclonal antibodies, TPE remains an effective part of first or second-line treatment. In this article, we detailed the historical aspects of the use of TPE in neurological disorders in Malaysia over the last four decades and discussed the challenges behind the establishment of the first in-house neurology-driven TPE service in the country. Local TPE database from a national neurology centre in Kuala Lumpur over the past 20 years was analyzed. We observed a remarkable three folds increase in the use of TPE at our center over the past 10 years (total 131 TPE treatments) compared to a decade prior, with expanding clinical indications predominantly for central nervous system demyelinating disorders. Besides using membrane filtration method, centrifugal technique was adopted, providing new opportunities for other clinical beneficiaries such as a neurologist driven "in-house TPE unit". However, there were real world challenges, especially having to provide services with limited funding, human resources, and space. In addition, much has to be done to improve accessibility, availability, and sustainability of TPE services at our center and nationwide. Nevertheless, even with limited resources and support, it is possible with concerted efforts to work within the confines of these limitations to establish a safe, successful, and sustainable TPE service.


Asunto(s)
Tecnología Biomédica/métodos , Hospitales Públicos/métodos , Neurología/métodos , Intercambio Plasmático/métodos , Anticuerpos Monoclonales/uso terapéutico , Enfermedades Autoinmunes del Sistema Nervioso/terapia , Tecnología Biomédica/tendencias , Enfermedades del Sistema Nervioso Central/terapia , Hospitales Públicos/economía , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Intercambio Plasmático/economía , Intercambio Plasmático/tendencias
4.
J Clin Apher ; 33(5): 559-568, 2018 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-29626354

RESUMEN

In December 2017, 79 delegates attended the 2nd regional plasmapheresis conference and workshop for Southeast Asia (SEA) on the immunomodulatory role of plasma exchange in central and peripheral nervous system disorders in Kuala Lumpur, Malaysia. This meeting featured 6 plenary lectures, interactive sessions dedicated for experience sharing, case presentations, and a practical session for paramedics. Clinical experts and researchers from 7 SEA countries and India shared experience and challenges in treating autoimmune neurological disorders. While the spectrum of diseases and neurology practice remained largely similar, there was great disparities in accessibility of therapeutic plasma exchange (TPE) within SEA countries and between urban or rural settings. Costs, human resources, and healthcare policies are common challenges in providing sustainable TPE services. Novel techniques and innovative ideas in performing TPE were explored. A working consortium comprising of key opinion leaders was proposed to improve standards of TPE and enhance future research.


Asunto(s)
Congresos como Asunto , Intercambio Plasmático/métodos , Plasmaféresis/métodos , Asia Sudoriental , Enfermedades del Sistema Nervioso Central/inmunología , Enfermedades del Sistema Nervioso Central/terapia , Enfermedades Desmielinizantes , Humanos , Inmunomodulación , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/terapia
5.
Muscle Nerve ; 55(5): 748-751, 2017 05.
Artículo en Inglés | MEDLINE | ID: mdl-27750406

RESUMEN

INTRODUCTION: The most efficient method of performing electrophysiology for Guillain-Barré syndrome (GBS) is unknown. METHODS: We retrospectively analyzed electrophysiological data of 97 consecutive GBS patients from Birmingham, UK (2001-2012), studied ≤ 3 weeks post-onset. RESULTS: The sensitivity of electrophysiology for each GBS subtype was dependent on the upper and lower limb nerves tested. In acute inflammatory demyelinating polyneuropathy (AIDP), abnormalities were predominant in the arms, whereas leg abnormalities predominated in axonal GBS. In AIDP, the most common abnormal parameters were distal motor latency and conduction block, and the most frequently affected nerve was the median. In axonal GBS, reduced motor amplitudes and conduction block were the most common parameters, and the most frequently abnormal nerve was the tibial. CONCLUSIONS: Electrodiagnostic sensitivity in GBS is dependent on nerves tested and parameters considered. Each subtype preferentially involves specific nerves and parameters. These findings may help per-procedure interpretation, improve electrodiagnostic sensitivity, and reduce patient discomfort. Muscle Nerve 55: 748-751, 2017.


Asunto(s)
Electrodiagnóstico/métodos , Síndrome de Guillain-Barré/diagnóstico , Conducción Nerviosa/fisiología , Adulto , Anciano , Femenino , Síndrome de Guillain-Barré/fisiopatología , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos
6.
J Peripher Nerv Syst ; 22(1): 27-33, 2017 03.
Artículo en Inglés | MEDLINE | ID: mdl-27868289

RESUMEN

Our objective was to assess determinants of quality of life (QoL) in anti-myelin associated glycoprotein antibody (MAG) neuropathy. The SF-36 questionnaire was assessed in 55 patients, from Marseille, Angers (France) and Birmingham (UK). Routine clinical evaluations included Medical Research Council (MRC) sum score, inflammatory neuropathy cause and treatment (INCAT) sensory score, inflammatory Rasch-built overall disability score (I-RODS), ataxia score, Jamar grip dynamometry, timed 10-m walk, neuropathic pain symptom inventory (NPSI) score, and fatigue severity score (FSS). Physical component summary (PCS) and mental component summary (MCS) of the SF36 questionnaire were significantly lower than in reported normal subjects of both countries (p < 0.001). All SF-36 domains correlated with I-RODS, except MCS for which significance was, however, approached (p = 0.056). PCS correlated with MRC sum score, ataxia score, timed 10-m walk, tremor, Jamar grip dynamometry, NPSI pain score, FSS and level of social support. MCS correlated exclusively with FSS and level of social support. In multivariate regression, PCS was associated independently with I-RODS (p < 0.001) and NPSI pain score (p = 0.011), whereas MCS was associated independently with FSS (p = 0.022). QoL is accurately predicted in anti-MAG neuropathy by the I-RODS and FSS, lending support to their use in clinical and research settings. Effective measures to improve QoL should include tremor and neuropathic pain treatment, fatigue management, and improved social support.


Asunto(s)
Anticuerpos/sangre , Glicoproteína Asociada a Mielina/inmunología , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/psicología , Calidad de Vida/psicología , Anciano , Anciano de 80 o más Años , Estudios Transversales , Evaluación de la Discapacidad , Europa (Continente)/epidemiología , Femenino , Humanos , Modelos Lineales , Masculino , Persona de Mediana Edad , Enfermedades del Sistema Nervioso Periférico/epidemiología , Encuestas y Cuestionarios
7.
Mult Scler Relat Disord ; 85: 105555, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38547547

RESUMEN

BACKGROUND: Despite the global availability of multiple sclerosis (MS) treatments, accessing and financing them in Southeast Asia (SEA) remains a challenge. This descriptive survey-based study aimed to describe the current state of MS treatment access and local access dynamics within this region. METHODS: The survey questionnaire, comprising of 15 closed-ended and five open-ended questions, was developed by three neurologists with expertise in MS and routine MS patient management, or had training in neuroimmunology. Questionnaire development was guided by the recent Atlas of MS and in alignment with the Access to Treatment framework, focusing on MS diagnosis and treatment issues in SEA. Fifteen neurologists experienced in managing MS across the region were identified as key informants for this study. RESULTS: All fifteen neurologists participated in the survey via email and videoconferencing between January 2020 and February 2023, which included the following countries: Brunei, Cambodia, Indonesia, Malaysia, Myanmar, Lao PDR, Philippines, Singapore, Thailand, Timor-Leste, and Vietnam. All had at least five years of experience in managing MS patients and six had previously completed a neuroimmunology fellowship programme. SEA countries showed disparities in healthcare financing, availability of neurologists, MS treatments, and investigative tools. Access to MS disease-modifying treatments (DMTs) is hindered by high cost, lack of MS specialists, and weak advocacy efforts. On-label DMTs are not listed as essential medicines regionally except for interferon beta1a and teriflunomide in Malaysia. On-label monoclonals are available only in Malaysia, Singapore, and Thailand. Generic on-label DMTs are unavailable due to lack of distributorship and expertise in using them. Off-label DMTs (azathioprine, methotrexate, and rituximab) predominate in most SEA countries. Other challenges include limited access to investigations, education, and knowledge about DMTs among general neurologists, and absence of registries and MS societies. Patient champions, communities, and MS organisations have limited influence on local governments and pharmaceutical companies. Despite its increasing prevalence, there is a lack of concerted priority setting due to MS being perceived as a rare, non-communicable disease. CONCLUSION: This study highlights the distinct dynamics, challenges, and research gaps within this region, and provides suggestions to improve MS diagnosis, education, and medicine access.


Asunto(s)
Accesibilidad a los Servicios de Salud , Esclerosis Múltiple , Neurólogos , Humanos , Asia Sudoriental , Esclerosis Múltiple/tratamiento farmacológico , Esclerosis Múltiple/terapia , Accesibilidad a los Servicios de Salud/estadística & datos numéricos , Neurólogos/estadística & datos numéricos , Encuestas y Cuestionarios , Factores Inmunológicos/uso terapéutico , Agentes Inmunomoduladores/uso terapéutico
8.
Artículo en Inglés | MEDLINE | ID: mdl-34728497

RESUMEN

BACKGROUND AND OBJECTIVES: To study the clinical and laboratory features of antineurofascin-155 (NF155)-positive autoimmune nodopathy (AN). METHODS: Patients with anti-NF155 antibodies detected on routine immunologic testing were included. Clinical characteristics, treatment response, and functional scales (modified Rankin Scale [mRS] and Inflammatory Rasch-built Overall Disability Scale [I-RODS]) were retrospectively collected at baseline and at the follow-up. Autoantibody and neurofilament light (NfL) chain levels were analyzed at baseline and at the follow-up. RESULTS: Forty NF155+ patients with AN were included. Mean age at onset was 42.4 years. Patients presented with a progressive (75%), sensory motor (87.5%), and symmetric distal-predominant weakness in upper (97.2%) and lower extremities (94.5%), with tremor and ataxia (75%). Patients received a median of 3 (2-4) different treatments in 46 months of median follow-up. Response to IV immunoglobulin (86.8%) or steroids (72.2%) was poor in most patients, whereas 77.3% responded to rituximab. HLA-DRB1*15 was detected in 91.3% of patients. IgG4 anti-NF155 antibodies were predominant in all patients; anti-NF155 titers correlated with mRS within the same patient (r = 0.41, p = 0.004). Serum NfL (sNfL) levels were higher in anti-NF155+ AN than in healthy controls (36.47 vs 7.56 pg/mL, p < 0.001) and correlated with anti-NF155 titers (r = 0.43, p = 0.001), with I-RODS at baseline (r = -0.88, p < 0.001) and with maximum I-RODS achieved (r = -0.58, p = 0.01). Anti-NF155 titers and sNfL levels decreased in all rituximab-treated patients. DISCUSSION: Anti-NF155 AN presents a distinct clinical profile and good response to rituximab. Autoantibody titers and sNfL are useful to monitor disease status in these patients. The use of untagged-NF155 plasmids minimizes the detection of false anti-NF155+ cases. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that anti-NF155 antibodies associate with a specific phenotype and response to rituximab.


Asunto(s)
Autoanticuerpos/sangre , Enfermedades Autoinmunes del Sistema Nervioso , Moléculas de Adhesión Celular/inmunología , Factores Inmunológicos/farmacología , Factores de Crecimiento Nervioso/inmunología , Nódulos de Ranvier/inmunología , Rituximab/farmacología , Adulto , Anciano , Enfermedades Autoinmunes del Sistema Nervioso/sangre , Enfermedades Autoinmunes del Sistema Nervioso/tratamiento farmacológico , Enfermedades Autoinmunes del Sistema Nervioso/inmunología , Enfermedades Autoinmunes del Sistema Nervioso/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Adulto Joven
9.
Ther Apher Dial ; 25(4): 513-532, 2021 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-33029928

RESUMEN

Evidence on the benefits of intermittent therapeutic plasma exchange (TPE) as maintenance therapy in neuromyelitis optica spectrum disorder (NMOSD) is limited. This study explores the possible effectiveness of sequential intermittent therapeutic plasma exchange (SITPE), a novel TPE protocol in the management of adult NMOSD patients. Through retrospective review of medical records in Kuala Lumpur Hospital, Malaysia, NMOSD patients who underwent SITPE, namely, an induction phase of monthly cycle of TPE (1 cycle = five exchange sessions) for three cycles with or without a subsequent maintenance phase of three-monthly cycle of TPE for three cycles, were included in this controlled historical cohort study. We explored their serial improvements in Expanded Disability Status Scale (EDSS), limb power, visual acuity, and annualized relapse rate following SITPE initiation. Statistical significance was set at P < .05. Fifteen adults (mean age: 35.4 years, mean disease duration: 9.5 years, 73% female, 87% AQP4-IgG positive) with corticosteroid-refractory attacks were included. Upon SITPE initiation, significant improvements in EDSS and limb motor power for up to 12 months, in addition to significant improvements in visual acuity for up to 6 months, were recorded. Significant reduction in annualized relapse rates for up to 2 years was documented. These improvements were not significantly influenced by age groups, gender, or presence of cord atrophy. Notably, adverse events of SITPE were infrequent and manageable. Sequential intermittent therapeutic plasma exchange as induction and maintenance therapy may improve the disease outcomes and prevent relapses in adult NMOSD patients with severe, corticosteroid-refractory attacks.


Asunto(s)
Neuromielitis Óptica/terapia , Intercambio Plasmático/métodos , Corticoesteroides/uso terapéutico , Adulto , Evaluación de la Discapacidad , Femenino , Humanos , Malasia , Masculino , Persona de Mediana Edad , Estudios Retrospectivos
10.
eNeurologicalSci ; 22: 100321, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-33553704

RESUMEN

Amyotrophic lateral sclerosis (ALS) is characterized by progressive onset motor deficits with heterogenous presentations ranging from dysarthria to foot drop. Approximately 20% of the patients present with focal bulbar symptoms, in which some may remain restricted to bulbar region (isolated bulbar palsy), and the remaining eventually spreads to involve other body regions (classical ALS). Without accompanying upper and lower motor neurons signs elsewhere, differential diagnoses for isolated bulbar symptoms are extensive, include ALS variants as well as potentially treatable mimics. Therefore, it is important to take heed on every possible aetiology that may disrupt the hypoglossal nucleus, nerve, or lingual muscle itself. Herein, we illustrated a rare presentation of Group A basilar invagination, which mimicked bulbar-onset ALS.

11.
J Cent Nerv Syst Dis ; 13: 11795735211039913, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34899003

RESUMEN

Optic nerve demyelination is one of the clinical features of combined central and peripheral demyelination (CCPD), an entity with heterogenous immunopathogenesis and clinical characteristics, overlapping between multiple sclerosis (MS) and chronic inflammatory demyelinating polyneuropathy (CIDP). Of interest, earlier studies among patients with CIDP prior to discovery of antibodies against paranodal protein neurofascin 155 (anti-NF 155) also reported optic nerve dysfunction. We aimed to evaluate optic nerve demyelination among anti-NF 155 CIDP patients. We studied 2 patients with anti-NF 155 CIDP using visual-evoked potentials (VEP) and optical coherence tomography (OCT). Both patients had distal acquired demyelinating symmetric (DADS) subtype CIDP. Other common features were prominent sensory ataxia, hand tremors, significantly elevated cerebral spinal fluid protein, high titre anti-NF 155 antibodies and poor response to corticosteroid and intravenous immunoglobulin (IVIg). No central nervous system neuroradiological abnormality detected. Both had normal visual acuity and colour vision, but one had subclinical right relative afferent pupillary defect (RAPD). VEP of both showed bilateral prolonged P100 latencies. OCT for patient with RAPD demonstrated moderate to severe retinal nerve fibre layer (RNFL) thinning. Identification of optic nerve demyelination among subclinical CIDP with anti-NF 155 antibodies expanded the spectrum of demyelination within the subset of CCPD.

12.
Gates Open Res ; 5: 178, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-35299829

RESUMEN

Background: There are >70 million forcibly displaced people worldwide, including refugees, internally displaced persons, and asylum seekers. While the health needs of forcibly displaced people have been characterized in the literature, more still needs to be done globally to translate this knowledge into effective policies and actions, particularly in neurology. Methods: In 2020, a global network of published experts on neurological disease and refugees was convened. Nine physician experts from nine countries (2 low, 1 lower-middle income, 5 upper-middle, 1 high income) with experience treating displaced people originating from 18 countries participated in three survey and two discussion rounds in accordance with the Delphi method. Results: A consensus list of priority interventions for treating neurological conditions in displaced people was created, agnostic to cost considerations, with the ten highest ranking tests or treatments ranked as: computerized tomography scans, magnetic resonance imaging scans, levetiracetam, acetylsalicylic acid, carbamazepine, paracetamol, sodium valproate, basic blood tests, steroids and anti-tuberculous medication. The most important contextual considerations (100% consensus) were all economic and political, including the economic status of the displaced person's country of origin, the host country, and the stage in the asylum seeking process. The annual cost to purchase the ten priority neurological interventions for the entire displaced population was estimated to be 220 million USD for medications and 4.2 billion USD for imaging and tests. Conclusions: A need for neuroimaging and anti-seizure medications for forcibly displaced people was emphasized. These recommendations could guide future research and investment in neurological care for forcibly displaced people.

13.
J Cent Nerv Syst Dis ; 13: 11795735211057314, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-35173510

RESUMEN

Therapeutic plasma exchange (TPE) is an effective and affordable treatment option in most parts of Southeast Asia (SEA). In 2018, the SEA TPE Consortium (SEATPEC) was established, consisting of regional neurologists working to improve outcome of various autoimmune neurological diseases. We proposed an immunotherapeutic guideline prioritizing TPE for this region. We reviewed disease burden, evidence-based treatment options, and major guidelines for common autoimmune neurological disorders seen in SEA. A modified treatment algorithm based on consensus agreement by key-opinion leaders was proposed. Autoimmune antibody diagnostic testing through collaboration with accredited laboratories was established. Choice of first-line immunotherapies (IVIg/corticosteroid/TPE) is based on available evidence, clinicians' experience, contraindications, local availability, and affordability. TPE could be chosen as first-line therapy for GBS, CIDP, MG (acute/short term), IgG, A paraproteinemic neuropathy, and NMDAR encephalitis. Treatment is stopped for acute monophasic conditions such as GBS and ADEM following satisfactory outcome. For chronic immune disorders, a therapy taper or long-term maintenance therapy is recommended depending on the defined clinical state. TPE as second-line treatment is indicated for IVIg or corticosteroids refractory cases of ADEM, NMOSD (acute), MG, and NMDAR/LGI1/CASPR2/Hashimoto's encephalitis. With better diagnosis, treatment initiation with TPE is a sustainable and effective immunotherapy for autoimmune neurological diseases in SEA.

15.
BMJ Neurol Open ; 2(1): e000020, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-33681775

RESUMEN

INTRODUCTION: Therapeutic plasma exchange (TPE) for semi-critical neurological manifestations can be managed in non-acute setting instead of critical care unit. In 2014, we established a non-acute neurology TPE unit for semi-critical haemodynamically stable patients. In this study, we aimed to evaluate the technical and safety parameters from the first 3 years of service. MATERIALS AND METHODS: We analysed prospectively collected TPE data for patients treated with centrifugation TPE at our non-acute neurology TPE unit in Kuala Lumpur Hospital between May 2015 and June 2018. RESULTS: A total of 245 TPE procedures were performed in 55 patients for nine neurological indications, predominantly the central nervous system (79%). Twenty four per cent (n=13) had category I and 73% (n=40) had category II indication (American Society for Apheresis (ASFA) 2019). Others (4%) were not in ASFA indications. Neuromyelitis optica spectrum disorders accounted for half (51%) of the total patients. Twenty-three (41.8%) patients experienced adverse events, with hypotensive episodes being the the most common (n=12/55, 21.8%). Five (9.1%) patients had catheter-related blood stream infection, correlating with higher exchange plasma volume (p=0.023). Symptomatic hypocalcaemia was less common (n=5/55, 9.1%) and allergic reaction to human albumin was rare (n=1/55, 1.8%). Four technical errors detected. Three involved centrifugation sets manufacturing defects and one involved error in centrifugation set installation. Seven (2.9%) procedures were terminated: 5 for adverse effects and 2 for technical errors. CONCLUSION: Performing TPE among semi-critical patients with neurology manifestations in basic non-acute set-up proved safe, with predictable complications. This set-up reduced the reliance on critical care services for TPE procedures.

16.
Clin Neurophysiol Pract ; 5: 112-117, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32613149

RESUMEN

OBJECTIVE: We detailed the electrophysiological patterns of peripheral nerve temporal dispersion across spectrum of POEMS syndrome and Castleman disease (CD). METHODS: Compound muscle action potentials (CMAP) duration of 3 patients with POEMS syndrome and 2 with hyaline vascular type CD without clonal plasma cell dyscrasia were retrospectively analysed. RESULTS: Median and ulnar nerves distal CMAP duration were prolonged in all patients irrespective of plasma cell dyscrasia or M protein. All lower limbs distal CMAP responses were absent. Greatest distal CMAP duration prolongation was observed in median nerves for POEMS syndrome (17.0 ms, 158% upper limit normal) and in ulnar nerves for CD (9.8 ms, 47% upper limit normal). Distal/proximal CMAP duration ratio of <0.7 were seen in 33% of median and ulnar nerves studied among POEMS syndrome. Among nerves with ratio >0.7, all had distal CMAP duration prolongation (Range 7%-158% of upper limit normal). CONCLUSIONS: Abnormal distal CMAP dispersion is not uncommon in POEMS syndrome and CD without clonal plasma cell dyscrasia or M protein. POEMS syndrome has greater distal CMAP duration in median and ulnar nerves, particularly in median nerve that can reach up to 150% of upper limit normal, compared to <50% in CD. SIGNIFICANCE: Detailed electrophysiological analysis of distal CMAP duration may help in distinguishing POEMS syndrome and CD.

18.
Ann Indian Acad Neurol ; 22(1): 102-103, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30692769

RESUMEN

Multiple co-infections can predispose a patient to autoimmune encephalitis. Out of thirty cases of N-methyl-D-aspartate receptor (NMDAR) encephalitis seen at a single tertiary referral center, only two cases of co-infection with NMDAR encephalitis were identified. One of these cases was highly interesting due to the presence of more than one co-infections along with the presence of cortical dysfunction, seizures, and orofacial dyskinesias at the onset in a male in the absence of tumors, which was refractory to initial treatment.

19.
J Clin Neurosci ; 69: 114-119, 2019 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-31416736

RESUMEN

We determined the clinical progression, disability and outcome of 11 Multifocal Motor Neuropathy (MMN) patients from Malaysia. Mean patient age was 46.8 (SD 13.3), with mean disease duration of 108.0 months (SD 80.2). All reported unilateral limb weakness at onset. At diagnosis, after mean 49.9 months (SD 73.5) delay, 7 (63.6%) had more than 2 limbs involvement. Nine (90%) of 10 patients received induction IVIg dose of 2.0 gm/kg responded, demonstrated improvement in MRCSS of > 2 points or mRS score of > 1 point. We observed 38.5% drop in IVIg dose to mean 1.12 gm/kg/month after 12 months of treatment, and a further 34.8% drop upon 24th month treatment to mean dose of 0.73 gm/kg/month. This was in parallel with initial improvement in MRCSS and mRS, observed among 88.9% and 77.8% of the patients, and later further improvement (33.3%) or stabilization (66.7%) of mRS score toward 2nd year. During the same period, 50% of patients reported deterioration in ONLS, 33.3% in grip strength and 16.7% in MMN-RODS. Beyond 36th month, average annual IVIg dose increased at 0.12 gm/kg/year (SD 0.09) or 11.2%, up to the 84th months. Despite that, progressive deterioration was observed in term of number of limbs involvement, definite motor conduction blocks on electrophysiology study, and both clinical as well as functional scores. Although IVIg dose reduction for maintenance treatment in MMN is recommended, careful clinical assessment is required to prevent under-treatment. Use of reliable and responsive modern outcome measures is important to quantify clinically relevant change to guide therapy.


Asunto(s)
Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Enfermedad de la Neurona Motora/tratamiento farmacológico , Adulto , Evaluación de la Discapacidad , Progresión de la Enfermedad , Femenino , Humanos , Malasia , Masculino , Persona de Mediana Edad , Resultado del Tratamiento
20.
Ther Apher Dial ; 23(3): 289-297, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-30927331

RESUMEN

In conjunction with the third regional Southeast Asian (SEA) therapeutic plasma exchange (TPE) conference in Kuala Lumpur, Malaysia, 25 clinicians and researchers from SEA and South Asian countries attended the inaugural strategy meeting for the establishment of a regional TPE consortium for neurological disorders. The primary objective was to establish regional collaboration to improve delivery of TPE services in SEA. A pre-meeting survey was conducted to gather insights on disease spectrum, contextual practice challenges, and the need for a regional TPE consensus. Challenges identified include limited healthcare funding in support of diagnostic workup, TPE therapy, as well as development of clinical infrastructure and expertise capacity building. There was favorable interest in developing a working plan contextualized to this region. Strategies to overcome challenges were discussed. This included the need for a comprehensive referral system and network of regional TPE centers suited to local needs, supported by innovative TPE delivery programs.


Asunto(s)
Congresos como Asunto , Enfermedades del Sistema Nervioso/terapia , Intercambio Plasmático/métodos , Asia Sudoriental , Consenso , Humanos , Malasia , Enfermedades del Sistema Nervioso/diagnóstico
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