多巣性脱髄性ニューロパチー
WordNet
- any pathology of the peripheral nerves
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English Journal
- What's in the Literature?
- El-Dokla A1, Silvestri NJ, Wolfe GI, Lacomis D.Author information 1*Department of Neurology, University of Pittsburgh School of Medicine, Pittsburgh, PA; †Department of Neurology, School of Medicine and Biomedical Sciences, University at Buffalo, The State University of New York, Buffalo, NY; and ‡Division of Neuropathology, Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA.AbstractWe examine the expanded spectrum of congenital myasthenic syndromes. There is a report of the clinical and electrodiagnostic features of patients with limb-girdle predominant, treatment-responsive N-acetylglucosamine phosphotransferase 1 mutations and a study of phenotypic variability from mutations in muscle-specific tyrosine kinase. In myasthenia gravis, fatigue is a commonly encountered symptom, and a recent report from Norway suggested that fatigue may be associated with symptoms of autonomic disturbance. Regarding childhood neuropathy/neuronopathy, Brown-Vialetto-Van Laere syndrome is a rare disorder also known as Fazio-Londe syndrome. It causes non-length dependent motor-sensory neuropathy, gait ataxia, hearing loss, and tongue fasciculations. There is now evidence that mutations in SLC52A2 encoding for the riboflavin transporter are perhaps the most common cause; and remarkably, it is responsive to riboflavin administration. In adults with asymmetric, lower motor neuron limb weakness, motor neuronopathy is one cause, but treatable entities such as multifocal motor neuropathy need to be considered. A recent study shows that there is a low rate of intravenous immunoglobulin response when used empirically in the absence of conduction block in this setting. In the myositis category, inclusion body myositis can be difficult to confirm pathologically, and a new report recommends that a panel of immunostains that assess autophagy and protein aggregation can be very sensitive and specific. Cancer-associated myositis was found to be more common in older age and in men and in the presence of dysphagia and lower serum creatine kinase levels. We also cover neuromuscular ultrasound and new measures for assessing carpal tunnel syndrome, findings in posterior interosseous neuropathy, and ultrasound use in distinguishing chronic inflammatory demyelinating polyneuropathy from demyelinating inherited neuropathy. The use of serum fibroblast growth factor 21 as a biomarker of mitochondrial disease is reviewed and the expanding spectrum of mitochondrial disorders into later adulthood and as a new cause of potassium channel dysfunction and periodic paralysis. Last, the topics in amyotrophic lateral sclerosis that are addressed include regional and distant disease spread, the clinical and prognostic utility of neck flexor weakness, and exercise and focal brain lesions as risk factors.
- Journal of clinical neuromuscular disease.J Clin Neuromuscul Dis.2014 Mar;15(3):129-42. doi: 10.1097/CND.0000000000000023.
- We examine the expanded spectrum of congenital myasthenic syndromes. There is a report of the clinical and electrodiagnostic features of patients with limb-girdle predominant, treatment-responsive N-acetylglucosamine phosphotransferase 1 mutations and a study of phenotypic variability from mutations
- PMID 24534836
- [Diabetes mellitus and autoimmune neuropathy].
- Deguchi T1, Nishio Y, Takashima H.Author information 1Diabetes and Endocrime Medicine, Hematology, Endocrinology, and Diabetology Center, Kagoshima University Hospital.AbstractAbstract The term "diabetic neuropathy" refers to many varieties of neuropathies, including diabetic peripheral neuropathies (DPNs). DPNs are categorized into generalized and focal/multifocal varieties. Diabetic sensorimotor polyneuropathy (DSPN) and diabetic autonomic neuropathy (DAN) are typical DPNs, and their development is clearly linked to hyperglycemia and subsequent metabolic and ischemic change. On the other hand, other forms of neuropathy, including multifocal diabetic neuropathies (e.g., lumbosacral, thoracic, and cervical radiculoplexus neuropathies) are thought to be associated with inflammatory or immune processes. Diabetic patients can also develop chronic inflammatory demyelinating polyneuropathy (CIDP). CIDP in diabetic patients (DM-CIDP) should be ruled out, especially in patients with advanced DSPN. Recently, it was reported that diabetic radiculoplexus neuropathies as well as CIDP respond favorably to immunotherapy. Thus, these immune-mediated diabetic neuropathies are treatable, and should be differentiated from advanced DSPN.
- Brain and nerve = Shinkei kenkyū no shinpo.Brain Nerve.2014 Feb;66(2):135-47.
- Abstract The term "diabetic neuropathy" refers to many varieties of neuropathies, including diabetic peripheral neuropathies (DPNs). DPNs are categorized into generalized and focal/multifocal varieties. Diabetic sensorimotor polyneuropathy (DSPN) and diabetic autonomic neuropathy (DAN) are typical D
- PMID 24523312
- Treatment of multifocal motor neuropathy.
- Jinka M1, Chaudhry V.Author information 1Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA.AbstractOPINION STATEMENT: Multifocal motor neuropathy (MMN) is a treatable immune disorder of the peripheral nerves that is characterized clinically by slowly progressive or stepwise asymmetric distal > proximal, upper > lower limb weakness in multiple motor nerve distributions; electrophysiologically by multifocal motor demyelination, specifically partial motor conduction block; laboratory evidence of high serum anti-GM1 IgM antibodies; and remarkable treatment response to intravenous immunoglobulin (IVIG). IVIG has become the treatment of choice, and the U.S. Food and Drug Administration (FDA) has approved Gammagard Liquid 10 % [immune globulin infusion (human)] as a treatment for multifocal motor neuropathy (MMN). Response to IVIG in MMN is dose- and frequency-dependent, most patients needing high (2 g/kg) and frequent (every 4-8 weeks) doses for several years. Over time, response to IVIG may decrease despite higher and more frequent dosing of IVIG treatment. Subcutaneous immunoglobulin (dose equivalent to IVIG) given in weekly fashion has recently been used with equal efficacy and fewer side effects. There are some case reports and non-randomized trials suggesting variable results from therapeutic or adjunctive use of other immunosuppressive or immunomodulatory agents such as cyclophosphamide, cyclosporine, methotrexate, azathioprine, interferon beta-1a, and rituximab. Of these, cyclophosphamide and rituximab are the only immune treatments that have shown some benefits in case reports. One randomized controlled trial of mycophenolate mofetil used as adjunctive agent did not prove efficacious in altering the disease course. Although MMN, like chronic inflammatory demyelinating polyneuropathy (CIDP), is a chronic immune-mediated demyelinating neuropathy, the use of corticosteroids and plasma exchange - two other therapies used in CIDP - is not beneficial for MMN. Further investigations are warranted to evaluate the immunopathogenesis of MMN and to explore options for dose, frequency, and duration of IVIG treatment as well as the use of alternative immunomodulatory agents either as primary therapeutic or adjunctive agents.
- Current treatment options in neurology.Curr Treat Options Neurol.2014 Feb;16(2):269. doi: 10.1007/s11940-013-0269-y.
- OPINION STATEMENT: Multifocal motor neuropathy (MMN) is a treatable immune disorder of the peripheral nerves that is characterized clinically by slowly progressive or stepwise asymmetric distal > proximal, upper > lower limb weakness in multiple motor nerve distributions; electrophysiologicall
- PMID 24395647
Japanese Journal
- ニューロパチーの診断と治療-日常診療でよく見る疾患を中心に-
- 全身脱毛症をともなった多巣性脱髄性感覚運動型chronic inflammatory demyelinating polyradiculoneuropathy（CIDP）の1例
Related Links
- Abstract Background: Several patients have been reported with an asymmetric sensory or sensorimotor demyelinating neuropathy not fulfilling the diagnostic criteria for chronic inflammatory demyelinating polyneuropathy ...
- The co-occurrence of multiple sclerosis and peripheral demyelinating neuropathy is rare. It has been disputed whether these are pathologically related or coincidental findings. We report a 36-year-old woman who ...
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- 英
- multifocal demyelinating neuropathy
- 関
- 慢性炎症性脱髄性多発ニューロパチー
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持続性伝導ブロックを伴う多巣性脱髄性ニューロパチー
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ニューロパチー
- 同
- NARP
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- 関
- demyelinative