Conventional and unconventional therapies in typical and atypical chronic inflammatory demyelinating polyneuropathy with different clinical course of progression

Hung, Stefanie Kar Yan; Hiew, Fu Liong; Shanthi, Vissa; Puvanarajah, Santhi Datuk

doi:10.1111/jns.12282

Cited by 5 publications

(3 citation statements)

References 23 publications

(29 reference statements)

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“…The same treatment protocol was applied to MG patients admitted to our centre with myasthenic crisis. For patients with CIDP, treatments were primarily with corticosteroids and intravenous Ig 17. The use of TPE was limited due to the chronicity of the disease.…”

Section: Discussionmentioning

confidence: 99%

Therapeutic plasma exchange (TPE) for semi-critical neurology presentations in a non-acute neurology set-up: clinical practice and challenges

Wong

Hiew

2020

BMJ Neurol Open

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IntroductionTherapeutic 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 methodsWe 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.ResultsA 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.ConclusionPerforming 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.

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Section: Discussionmentioning

confidence: 99%

Therapeutic plasma exchange (TPE) for semi-critical neurology presentations in a non-acute neurology set-up: clinical practice and challenges

Wong

Hiew

2020

BMJ Neurol Open

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“…The same treatment protocol applied to MG patients admitted to our center with myasthenic crisis. For patients with CIDP in our center, treatments were primarily with corticosteroids and IVIg . The use of TPE was limited due to the chronicity of the disease.…”

Section: Discussionmentioning

confidence: 99%

The establishment of in‐house neurology driven therapeutic plasma exchange infrastructure in a resource‐limited public hospital in Malaysia: Adopting and integrating evidenced‐based health care technology through time

Shanthi

Hiew

2019

J of Clinical Apheresis

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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.

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“…The findings of our study are similar to other longitudinal studies in CIDP. 1–4 However, none of the patients were on maintenance IVIG or plasma exchange treatment. Also, very few patients were given cyclophosphamide and rituximab.…”

mentioning

confidence: 99%

Determinants of Outcome in Chronic Inflammatory Demyelinating Polyneuropathy: A Longitudinal Study

Siddegowada

Singh²,

Prasad³

et al. 2021

Journal of Clinical Neuromuscular Disease

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We report a 52-year-old woman who came to the Blessing Neurology Clinic for further evaluation of numbness and tingling of her feet that started 9 years ago and has recently gotten worse to the point she could not drive because she could not feel her feet. Her examination results showed reduced vibration, proprioception, and pinprick up to her midcalf and wrists. She underwent NCS EMG which showed severe sensorimotor axonal polyneuropathy. Her laboratory results showed normal HbA1c, b12, TSH, and folate; ANA 1:640; and negative ENA; P-ANCA and C-ANCA were negative; however, atypical was positive 1:80 (reference range ,1: 20). After discussion with the patient, she had 2 cycles of IVIG and her symptoms and examination results have gotten better; now, she is able to drive. DISCUSSIONAtypical ANCA has been reported before with ocular inflammatory disease, autoimmune hepatitis, ulcerative colitis, and primary sclerosing cholangitis; however, it was never reported before that atypical P-ANCA is associated with autoimmune neuropathy. In the above-mentioned case, the patient has severe sensorimotor axonal polyneuropathy with high atypical P-ANCA. This case report may indicate considering autoimmune neuropathy associated with atypical P-ANCA, and further studies and research are needed to show the association of these autoantibodies with neuropathy.

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Conventional and unconventional therapies in typical and atypical chronic inflammatory demyelinating polyneuropathy with different clinical course of progression

Cited by 5 publications

References 23 publications

Therapeutic plasma exchange (TPE) for semi-critical neurology presentations in a non-acute neurology set-up: clinical practice and challenges

Therapeutic plasma exchange (TPE) for semi-critical neurology presentations in a non-acute neurology set-up: clinical practice and challenges

The establishment of in‐house neurology driven therapeutic plasma exchange infrastructure in a resource‐limited public hospital in Malaysia: Adopting and integrating evidenced‐based health care technology through time

Determinants of Outcome in Chronic Inflammatory Demyelinating Polyneuropathy: A Longitudinal Study

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