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Fulltext Biomarkers of 'acute-onset' chronic inflammatory demyelinating polyneuropathy

Miura Y, Shahrizaila N, Yuki N

Brain, 2015 Mar;138(Pt 3):e335.
PMID: 25183714 DOI: 10.1093/brain/awu252
Matched MeSH terms: Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnosis*
Fulltext A Severe Form of M - protein Negative Distal Acquired Demyelinating Symmetric Neuropathy

Ong TL, Goh KJ, Shahrizaila N, Wong KT, Tan CY

Neurol India, 2019 12 21;67(6):1532-1535.
PMID: 31857554 DOI: 10.4103/0028-3886.273621

Distal acquired demyelinating symmetric neuropathy (DADS) is a variant of chronic inflammatory demyelinating polyneuropathy (CIDP) characterized by symmetrical, distal, sensory or sensorimotor involvement. DADS with M-protein (DADS-M) is less responsive to immunotherapy compared to those without M-protein (DADS-I). We report a case of DADS-I with severe clinical presentation viz. early hand involvement with marked wasting, inexcitable peripheral nerves on neurophysiology and poor response to immunotherapy. Despite the unusual presentation, ancillary tests including cerebrospinal fluid analysis, nerve biopsy and nerve ultrasound were supportive of an inflammatory demyelinating polyneuropathy. This case demonstrated the heterogeneity of the disorder and expands the clinical spectrum of DADS neuropathy.

Matched MeSH terms: Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/diagnosis*; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/drug therapy; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/immunology; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/physiopathology
Fulltext AMSAN variant of Guillain Barre syndrome progressing to chronic inflammatory demyelinating polyneuropathy in a patient with Marfan's syndrome and pulmonary tuberculosis

Soehardy Z, Yuhanisa A, Thein SS, Rohana AG, Fauzi AR, Norlinah MI, et al.

Med J Malaysia, 2005 Dec;60(5):655-6.
PMID: 16515122

We report a 40-year-old man who has Marfan's syndrome and was recently diagnosed to have pulmonary tuberculosis when he presented with chronic cough. He was admitted with bilateral lower limb weakness which was ascending in nature. He eventually required ventilation. It was initially thought to be isoniazid-neuropathy. However, stopping the drug did not improve the condition and the patient developed bilateral lower motor neuron 7th cranial nerve palsy. Nerve conduction, MRI and CSF studies were done to confirm a first case report of AMSAN variant progressing to CIDP in a patient with Marfan's syndrome and pulmonary tuberculosis.

Matched MeSH terms: Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/etiology*
Are ERM (ezrin/radixin/moesin) proteins targets for autoantibodies in demyelinating neuropathies?

Miyaji K, Shahrizaila N, Umapathi T, Chan YC, Hirata K, Yuki N

Hum Immunol, 2014 Nov;75(11):1089-91.
PMID: 25286001 DOI: 10.1016/j.humimm.2014.09.010

Ezrin, radixin and moesin, which are strongly expressed in the Schwann cell microvilli, are putative targets for autoantibodies in acute or chronic inflammatory demyelinating polyneuropathy (AIDP or CIDP). An association between anti-moesin IgG antibodies and cytomegalovirus-related AIDP has been postulated. None of 41 AIDP patients, including 8 cytomegalovirus-related AIDP patients, and 23 CIDP had IgG or IgM antibodies to ezrin, radixin and moesin; whereas, one patient with cytomegalovirus-related AIDP had anti-ezrin IgM antibodies. Ezrin, radixin and moesin are unlikely targets for autoantibodies in AIDP and CIDP, and the association of anti-moesin antibodies with cytomegalovirus-related AIDP was not confirmed.

Matched MeSH terms: Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/blood; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/genetics*; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/immunology; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/pathology
Outcome in chronic inflammatory demyelinating polyneuropathy from a Malaysian centre over sixteen years

Hiew FL, Ong JJ, Viswanathan S, Puvanarajah S

J Clin Neurosci, 2018 Apr;50:203-207.
PMID: 29398193 DOI: 10.1016/j.jocn.2018.01.018

Long-term outcome in Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is very limited, especially from Asian countries. We aimed to determine the outcome of our cohort of CIDP patients and to define the relevant clinical, electrophysiological and laboratory determinants of disease activity, progression and treatment response. We retrospectively reviewed records of 23 CIDP patients attending our Neurology service at Kuala Lumpur Hospital, Malaysia between January 2000 and December 2016. We analysed data on neurological deficits, electrophysiological and laboratory parameters to determine diagnostic characteristics, correlation with disease activity and clinical outcomes following treatment. Included were 15 (65%) males and 8 (35%) females with a mean age of 42.7 years (SD 14.4). Mean duration of follow-up visit was 66 months (range 6-134 months). The cohort consists of 19 classical (sensory-motor) CIDP and 4 MADSAM. Large majority of patients (66%) had either stable active disease (CDAS 3, 44%) or were in remission (CDAS class 2, 22%) following treatment with standard immunotherapies (Intravenous Immunoglobulins, steroids or immunosuppressants). The proportion of CIDP patients in each CDAS class was comparable to published cohorts from North America and Europe. Medical Research Council (MRC) sum score was the only clinical score that differed across CDAS classes (p = .010) with significant inverse correlation (Spearman's rho -0.664, p = .001). In conclusion, treatment outcomes of our CIDP cohort was comparable to those of published series. Further studies with larger cohort of patients from other parts of Asia are important to determine the long-term outcome of this heterogenous disease in this region.

Matched MeSH terms: Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/drug therapy*; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/epidemiology*
Successful treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) in systemic lupus erythematosus (SLE) with oral cyclophosphamide

Jasmin R, Sockalingam S, Shahrizaila N, Cheah TE, Zain AA, Goh KJ

Lupus, 2012 Sep;21(10):1119-23.
PMID: 22433918 DOI: 10.1177/0961203312440346

Peripheral neuropathy is a known manifestation of systemic lupus erythematosus. However, the association of primary autoimmune inflammatory neuropathies such as chronic inflammatory demyelinating polyneuropathy (CIDP) with SLE is uncommon. We report a 26-year-old man who simultaneously presented with severe CIDP and photosensitive rash, but was unresponsive to intravenous immunoglobulin infusion and continued to progress. He was found to have underlying SLE and improved with combined corticosteroid and immunosuppressive therapy with oral cyclophosphamide. CIDP with underlying SLE may be more resistant to conventional therapy with IVIG, requiring the addition of other immunosuppressive agents.

Matched MeSH terms: Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/complications*; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/drug therapy*; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/physiopathology
Diagnostic yield of nerve biopsy in the evaluation of peripheral neuropathies

Lee LY, Tan CY, Wong KT, Goh KJ, Shahrizaila N

J Clin Neurosci, 2023 Jan;107:40-47.
PMID: 36502780 DOI: 10.1016/j.jocn.2022.11.017

BACKGROUND: With progress made in neurogenetics and neuroinflammation, the indications and value of nerve biopsies in the diagnostic evaluation of peripheral neuropathies are less clear. In this study, we aimed to evaluate the diagnostic yield of nerve biopsies in patients with peripheral neuropathies.
METHODS: We performed a retrospective review of nerve biopsy reports from April 1998 to June 2021 of patients with peripheral neuropathies presenting to the Department of Pathology, University of Malaya Medical Centre, Kuala Lumpur, Malaysia. The diagnostic value of the biopsies was determined based on the criteria by Midroni and Bilbao as follows: contributive (essential and helpful), non-contributive and inadequate.
RESULTS: A total of 107 nerve biopsies were analysed. Sixty-four (60 %) were males and the mean age was 52 years, ranging from 13 to 86 years. Ninety-four (88 %) were sural nerve biopsies; and only one patient (1 %) each had superficial peroneal and superficial radial nerve biopsy. The indications for the procedure were vasculitis (34 %), peripheral neuropathy of unknown aetiology (34 %), amyloidosis (14 %) and chronic inflammatory demyelinating polyneuropathy (10 %). In 68 (63 %) biopsies, the diagnostic value was contributive. Of these, 28 (26 %) were essential and 40 (37 %) were helpful. In contrast, 35 (33 %) biopsies were non-contributive and 4 (4 %) were inadequate. In 66 % (71/107) of cases, the nerve biopsy did not reveal a definite pathological diagnosis. However, in the remainder, a diagnosis of vasculitis (18 %, 19/107), followed by amyloidosis (10 %, 11/107) could be determined. For 32/71 biopsies with undetermined pathological diagnosis, neuropathy remained cryptogenic in 22 % (7/32) upon follow up.
CONCLUSIONS: With the exception of vasculitis and amyloidosis, there is limited value in performing nerve biopsies in the evaluation of patients with peripheral neuropathy. However, this should be interpreted with caution as the number of patients with a clinical diagnosis of vasculitis and amyloidosis were relatively larger than patients with other diagnosis. Refinement and careful selection of cases are required to increase the diagnostic yield of nerve biopsy.

Matched MeSH terms: Polyradiculoneuropathy, Chronic Inflammatory Demyelinating*
Conventional and unconventional therapies in typical and atypical chronic inflammatory demyelinating polyneuropathy with different clinical course of progression

Hung SKY, Hiew FL, Viswanathan S, Puvanarajah S

J Peripher Nerv Syst, 2018 Sep;23(3):183-189.
PMID: 30027593 DOI: 10.1111/jns.12282

Intravenous immunoglobulin (IVIG), corticosteroids and therapeutic plasma exchange (TPE) are evidence-based conventional treatments for chronic inflammatory demyelinating polyneuropathy (CIDP). In many centres, unconventional treatments are frequently used as alternatives. We evaluated the outcome of conventional and unconventional therapies in 31 CIDP patients. Overall response rate with conventional first-line immunotherapies was 77% (20/26), comparable between IVIG and corticosteroids (80% vs 70%). Use of TPE was limited. Treatment response among typical and atypical CIDP were comparable (76 vs 80%). Non-responders were patients with progressive form of typical CIDP and DADS. Majority (21/26, 81%) of patients with persistent neurological deficits received maintenance therapy. Two subgroups of patients frequently treated with maintenance immunosuppressants were those with improving or stable disease following first-line treatment (12, 57%) and those with progressive form of CIDP (2, 10%). Primary indications for immunosuppressant use were corticosteroids-sparing and additional immunosuppression effects. Nine (64%) patients with improving or stable disease given azathioprine were taken off corticosteroids after a median duration of 14 months (range 12-108). Two (14%) eventually achieved cure or clinical remission without treatment. Maintenance IVIg was given to 6 (29%) relapsing CIDP patients; none of achieved cure or remission after similar median duration of treatment. Less potent immunosuppressant drugs (azathioprine, mycophenolate mofetil, and methotrexate) were frequently used, with moderate adverse effect profiles. In resource limited setting, unconventional treatments were commonly used among CIDP patients with different clinical course of progression. In most cases, careful risk-benefit re-assessment is required to justify its further use.

Matched MeSH terms: Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/therapy*