IgM MGUS anti myelin-associated glycoprotein neuropathy can rarely express as a predominantly distal motor neuropathy

2016 ◽  
Vol 53 (5) ◽  
pp. 827-828 ◽  
Author(s):  
Giuliana Galassi ◽  
Manuela Tondelli
Keyword(s):  
Motor Neuropathy ◽  
Myelin Associated Glycoprotein

scholarly journals Proximal motor neuropathy, IgA paraproteinaemia and anti-myelin-associated glycoprotein reactivity.

1989 ◽  
Vol 65 (767) ◽  
pp. 662-664 ◽  
Author(s):  
T. Hemachudha ◽  
P. Phanuphak ◽  
K. Phanthumchinda ◽  
S. Kasempimolporn
Keyword(s):  
Motor Neuropathy ◽  
Myelin Associated Glycoprotein

scholarly journals Treatment Approaches for Atypical CIDP

2021 ◽  
Vol 12 ◽  
Author(s):  
Deepak Menon ◽  
Hans Dieter Katzberg ◽  
Vera Bril
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Immunosuppressive Agents ◽  
Conduction Block ◽  
Multifocal Motor Neuropathy ◽  
Motor Neuropathy ◽  
First Line ◽  
Treatment Approaches ◽  
First Line Therapy ◽  
Myelin Associated Glycoprotein ◽  
Pure Motor

The variants of chronic inflammatory demyelinating polyneuropathy (CIDP) differ not just in their clinical, pathological and electrophysiological characteristics, but often in their indifferent response to conventional immunosuppressive agents which are effective in typical CIDP. High quality evidence is lacking as far as the management of these atypical variants is concerned. In this review, we summarize the treatment approaches to each of these CIDP variants based on existing data. Distal acquired demyelinating symmetric polyneuropathy (DADS) has the phenotype of a symmetric, demyelinating sensory, length-dependent polyneuropathy and is frequently associated with paraproteinemia and anti myelin associated glycoprotein (MAG) antibodies. While the management of idiopathic DADS (DADS-I) is the same as CIDP, DADS-M responds suboptimally and has a favorable response to rituximab. Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) manifests as a chronic progressive demyelinating mononeuropathy multiplex which can evolve to a confluent pattern indistinguishable from CIDP. Evidence favors treating MADSAM with conventional immunomodulatory therapy (IMT), but this disorder responds less favorably than CIDP. Some patients present with purely sensory symptoms, known as pure sensory CIDP or chronic inflammatory sensory polyradiculoneuropathy (CISP), the latter localizing to a pre-ganglionic pathology. Both respond well to first line IMT, particularly to intravenous immunoglobulin (IVIG), but patients relapse without maintenance therapy. Pure motor CIDP resembles multifocal motor neuropathy with conduction block (MMNCB), but the previously reported worsening status after steroid treatment was not reproduced in recent studies, and IVIG remains the first-line therapy. Some focal forms of CIDP defy exact classification, but respond well to first-line IMT including IVIG. Overall, atypical CIDP responds to treatment with first-line IMT, but has a suboptimal response compared to CIDP. There is evidence for effectiveness with agents such as rituximab, especially in DADS-M, and this medication can also be used in cases refractory to conventional IMTs. Rituximab is also effective in CIDP with IgG4 antibodies which has distinct clinical features and is mostly refractory to first-line IMT.

Motor neuropathy and alacrima: oligosymptomatic triple A syndrome

2006 ◽  
Vol 37 (03) ◽  
Author(s):  
K Brockmann ◽  
K Koehler ◽  
M Krumbholz ◽  
J Gärtner ◽  
A Hübner
Keyword(s):  
Motor Neuropathy ◽  
Triple A Syndrome

Changes in the muscles of mastication before and after primary stereotactic radiosurgery in patients with idiopathic trigeminal neuralgia

2019 ◽  
pp. 1-8 ◽  
Author(s):  
Nasser Mohammed ◽  
Yi-Chieh Hung ◽  
Thomas J. Eluvathingal Muttikkal ◽  
Roy C. Bliley ◽  
Zhiyuan Xu ◽  
...  
Keyword(s):  
Trigeminal Neuralgia ◽  
Muscle Atrophy ◽  
Motor Neuropathy ◽  
Significant Pain ◽  
Medial Pterygoid ◽  
Before And After ◽  
Pterygoid Muscles ◽  
Masseter Muscles ◽  
Muscles Of Mastication ◽  
New Onset

OBJECTIVEThe motor root of the trigeminal nerve runs close to the sensory root and receives considerable radiation during Gamma Knife radiosurgery (GKRS) for trigeminal neuralgia (TN). The object of this study was to evaluate via MRI the changes in the muscles of mastication before and after upfront GKRS in patients with idiopathic TN.METHODSIn this single-institution retrospective cohort study, all patients with idiopathic unilateral TN treated with primary GKRS at the University of Virginia in the period from 2007 to 2017 were included provided that they had pre- and post-GKRS MRI data. The thicknesses of the temporalis, pterygoid, and masseter muscles were measured on both pre- and post-GKRS MRI in a blinded fashion. Changes in the muscles like fatty infiltration, MRI signal, or atrophy were noted.RESULTSAmong the 68 patients eligible for inclusion in the study, 136 temporalis muscles, 136 medial pterygoid muscles, 136 lateral pterygoid muscles, and 136 masseter muscles were assessed. A subset of patients was found to have muscle atrophy even prior to GKRS. Pre-GKRS atrophy of the masseter, medial pterygoid, lateral pterygoid, and temporalis muscles was seen in 18 (26%), 16 (24%), 9 (13%), and 16 (24%) patients, respectively. Logistic regression analysis showed that distribution of pain in the V3 territory (p = 0.01, OR 5.43, 95% CI 1.46–20.12) and significant pain on chewing (p = 0.02, OR 5.32, 95% CI 1.25–22.48) were predictive of pre-GKRS atrophy. Reversal of atrophy of these muscles occurred after GKRS in a majority of the patients. The incidence of new-onset permanent post-GKRS muscle atrophy was 1.5%. The median follow-up was 39 months (range 6–108 months).CONCLUSIONSA subset of patients with TN with significant pain on chewing have pre-GKRS disuse atrophy of the muscles of mastication. A reversal of the atrophy occurs in a majority of the patients following GKRS. New-onset motor neuropathy post-GKRS was rare.

Neurofilament Proteins as Prognostic Biomarkers in Neurological Disorders

2020 ◽  
Vol 25 (43) ◽  
pp. 4560-4569 ◽  
Author(s):  
Yichen Lee ◽  
Bo H. Lee ◽  
William Yip ◽  
Pingchen Chou ◽  
Bak-Sau Yip
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Nervous System ◽  
Trinucleotide Repeat ◽  
Muscular Atrophy ◽  
Electric Signal ◽  
Motor Neuropathy ◽  
Post Translational Modification ◽  
Neurofilament Proteins ◽  
Type Iv ◽  
Lateral Sclerosis

Neurofilaments: light, medium, and heavy (abbreviated as NF-L, NF-M, and NF-H, respectively), which belong to Type IV intermediate filament family (IF), are neuron-specific cytoskeletal components. Neurofilaments are axonal structural components and integral components of synapses, which are important for neuronal electric signal transmissions along the axons and post-translational modification. Abnormal assembly of neurofilaments is found in several human neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS), infantile spinal muscular atrophy (SMA), and hereditary sensory-motor neuropathy (HSMN). In addition, those pathological neurofilament accumulations are known in α-synuclein in Parkinson’s disease (PD), Aβ and tau in Alzheimer’s disease (AD), polyglutamine in CAG trinucleotide repeat disorders, superoxide dismutase 1 (SOD1), TAR DNA-binding protein 43 (TDP43), neuronal FUS proteins, optineurin (OPTN), ubiquilin 2 (UBQLN2), and dipeptide repeat protein (DRP) in amyotrophic lateral sclerosis (ALS). When axon damage occurs in central nervous disorders, neurofilament proteins are released and delivered into cerebrospinal fluid (CSF), which are then circulated into blood. New quantitative analyses and assay techniques are well-developed for the detection of neurofilament proteins, particularly NF-L and the phosphorylated NF-H (pNF-H) in CSF and serum. This review discusses the potential of using peripheral blood NF quantities and evaluating the severity of damage in the nervous system. Intermediate filaments could be promising biomarkers for evaluating disease progression in different nervous system disorders.

Sign in / Sign up

Export Citation Format

Share Document