• Title/Summary/Keyword: Chronic inflammatory demyelinating neuropathy

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Insight into the prognostic factors of chronic inflammatory demyelinating polyneuropathy

  • Baek, Seol-Hee
    • Annals of Clinical Neurophysiology
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    • v.22 no.1
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    • pp.8-12
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    • 2020
  • Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy with heterogeneous features. Appropriate treatment will produce a favorable outcome, but a poor treatment response and severe disability have also been reported. The roles of the clinical phenotypes and electrophysiological features of CIDP as well as of autoantibodies against nodal and paranodal proteins have been highlighted previously due to their association with the treatment response and long-term prognosis. This review addresses the diverse factors associated with the prognosis of CIDP.

Intravenous Immunoglobulin Therapy in Peripheral Neuropathy (말초신경병증에 대한 정맥내 면역글로불린 요법)

  • Kim, Nam Hee;Park, Kyung Seok
    • Annals of Clinical Neurophysiology
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    • v.8 no.1
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    • pp.6-15
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    • 2006
  • Intravenous immunoglobulin (IVIg) is the treatment of choice for many autoimmune neuropathic disorders such as Guillain-Barre syndrome (GBS), chronic inflammatory Demyelinating neuropathy (CIDP), and multifocal motor neuropathy (MMN). IVIg is preferred because the adverse reactions are milder and fewer than the other immune-modulating methods such as steroid, other immunosuppressant such as azathioprine, and plasmapheresis. IVIg also has been used in other autoimmune neuromuscular disorders (inflammatory myopathy, myasthenia gravis, and Lambert-Eaton myasthenic syndrome) and has been known as safe and efficient agent in these disorders. Since IVIg would get more indications and be used more commonly, clinicians need to know the detailed mechanism of action, side effects, and practical points of IVIg.

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Electrophysiological and radiological evidence for the multifocal nature of a case of multifocal acquired demyelinating sensory and motor neuropathy

  • Seong, Gi-Hun;Bae, Jong Seok;Ryu, Sanghyo
    • Annals of Clinical Neurophysiology
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    • v.20 no.2
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    • pp.101-104
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    • 2018
  • Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy is a variant of chronic acquired demyelinating polyneuropathy. A 65-year-old women presented with upper arm weakness. A nerve conduction study showed conduction blocks over intermediate segments with sparing of distal compound action potentials. Magnetic resonance imaging revealed asymmetric hypertrophy of the brachial plexus on the affected side. These findings represent important electrophysiological and radiological evidence of MADSAM neuropathy. The condition of the patient began to improve after starting intravenous immunoglobulin administration.

Distal acquired demyelinating symmetric neuropathy associated with anti-GM1 and anti-GD1b antibodies

  • Ko, Keun Hyuk;Jwa, Seung-Joo;Park, Sung Joo;Kang, Sa-Yoon
    • Annals of Clinical Neurophysiology
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    • v.19 no.1
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    • pp.54-57
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    • 2017
  • Distal acquired demyelinating symmetric (DADS) neuropathy is a variant form of chronic inflammatory demyelinating polyradiculoneuropathy. A 54-year-old man presented with gait disturbance owing to weakness in both legs. Nerve conduction studies showed demyelinating sensorimotor polyneuropathy, and laboratory studies demonstrated anti-GM1 and anti-GD1b IgG antibodies, but no anti-myelin associated glycoprotein activity. We suggest that an antiganglioside antibodies assay needs to be applied when DADS neuropathy is suspected in order to improve the classification of dysimmune neuropathies.

Subacute Inflammatory Demyelinating Polyneuropathy Combined with Optic Neuritis (시신경염이 동반된 아급성염증탈수초다발신경병)

  • Kim, Sieun;Park, Kang Min;Park, Jinse;Ha, Sam Yeol;Kim, Sung Eun;Kim, Jong Kuk;Shin, Kyong Jin
    • Annals of Clinical Neurophysiology
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    • v.15 no.1
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    • pp.13-18
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    • 2013
  • It was sometimes difficult to differentiate between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and subacute inflammatory demyelinating polyneuropathy (SIDP). The CNS involvement of these polyneuropathies has rarely reported in the literature. We present the case of a 42-year-old man who developed rapidly developing inflammatory demyelinating polyneuropathy followed by right optic neuritis. This case showed progressive motor weakness and sensory dysfunction with time to nadir at 8 weeks, demyelination in nerve conduction study, no other etiology of neuropathy, no relapse during follow-up of 18 months, good response to steroid and complete recovery which favor SIDP more than A-CIDP. We experienced the case of SIDP associated with optic neuritis.

Adverse Events Associated with Intravenous Immunoglobulin Therapy in Neuromuscular Disorders (신경근질환의 정맥 내 면역글로불린 치료와 연관된 유해사례)

  • Na, Sang-Jun;Choi, Young-Chul
    • Annals of Clinical Neurophysiology
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    • v.8 no.1
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    • pp.48-52
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    • 2006
  • Background: Intravenous immunoglobulin (IVIg) has been administered for various immune-mediated neurological diseases such as autoimmune neuropathy, inflammatory myopathies, and other autoimmune neuromuscular disorders. The purpose of this study is to investigate side effects and complications of IVIg therapy in neuromuscular disorders. Methods: We enrolled 29 patients (age 8~63 years) with IVIg therapy for various neurological diseases including Guillain-Barre syndrome, myasthenia gravis, dermatomyositis, polymyositis, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. IVIg therapy was used at a dose of 0.4 g/kg body weight/day for 5 consecutive days. Results: 10 patients (34%) had adverse events. There are adverse events in 16 courses (11%) among total 145 courses. The majority of patients presented with mild side effects, mostly asymptomatic laboratory changes. Rash or mild headache occurred in 3 patients. One patient showed a serious side effect of deep vein thrombosis. Conclusions: IVIg therapy is safe for a variety of immune-mediated neurological diseases in our study.

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