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  1. Article ; Online: Editorial

    Glenn Lopate

    Degenerative Neurological and Neuromuscular Disease, Vol 2011, Iss default, Pp 1-

    2011  Volume 2

    Abstract: Glenn LopateDepartment of Neurology, Washington University of St. Louis, St. Louis, MO, USAThe ...

    Abstract Glenn LopateDepartment of Neurology, Washington University of St. Louis, St. Louis, MO, USAThe proposition to start a new journal is always a difficult one. However, the recent trend of open access journals has allowed publishers to expand the number of journals available to all scientists worldwide. The Directory of Open Access Journals1 lists 522 medical journals and 94 neurological journals, but only one with a focus on neurodegeneration. This new journal, Degenerative Neurological and Neuromuscular Disease, published by Dove Medical Press, will hopefully be able to fill that void. The idea for this journal was inspired by the aging population and the frequent occurrence of neurodegenerative diseases in this large group of patients. According to the United Nations Department of Economic and Social Affairs,2 in 1950 at the global level, 1 in 12 individuals was at least 60 years old and 1 in 20 was at least 65. By 2050 those numbers are projected to be 1 in 5 over 60 years old and 1 in 6 over 65. A recent PubMed search supports the idea that neurodegenerative diseases are quite common, with almost 600 articles published in 2010 under the search terms degenerative neurological disease or degenerative neuromuscular disease, a 25% increase from the number published 10 years earlier.
    Keywords Neurology. Diseases of the nervous system ; RC346-429 ; Neurosciences. Biological psychiatry. Neuropsychiatry ; RC321-571 ; Internal medicine ; RC31-1245 ; Medicine ; R ; DOAJ:Neurology ; DOAJ:Medicine (General) ; DOAJ:Health Sciences
    Subject code 050
    Language English
    Publishing date 2011-03-01T00:00:00Z
    Publisher Dove Medical Press
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  2. Article ; Online: Myelinated and unmyelinated endoneurial axon quantitation and clinical correlation.

    Dori, Amir / Lopate, Glenn / Choksi, Rati / Pestronk, Alan

    Muscle & nerve

    2016  Volume 53, Issue 2, Page(s) 198–204

    Abstract: Introduction: Different disease patterns result from loss of myelinated and unmyelinated axons, but quantitation to define their loss has been difficult.: Methods: We measured large and small endoneurial axons in axonal neuropathies by staining them ... ...

    Abstract Introduction: Different disease patterns result from loss of myelinated and unmyelinated axons, but quantitation to define their loss has been difficult.
    Methods: We measured large and small endoneurial axons in axonal neuropathies by staining them with peripherin and comparing their area to that of nonmyelinating Schwann cells stained with neural cell adhesion molecule (NCAM).
    Results: Loss of myelinated and unmyelinated axons was typically proportional, with predominant myelinated or unmyelinated axon loss in a few patients. Myelinated axon loss was associated with loss of distal vibration sense and sensory potentials (P < 0.0001) and was selective in patients with bariatric and bowel resection surgery (P < 0.001). Unmyelinated axon measurements correlated with skin (ankle P = 0.01; thigh P = 0.02) and vascular (nerve P < 0.0001; muscle P = 0.01) innervation.
    Conclusions: Myelinated and unmyelinated axons can be quantitated by comparing areas of axons and nonmyelinating Schwann cells. Clinical features correlate with myelinated axon loss, and unmyelinated axon loss correlates with skin and vascular denervation.
    MeSH term(s) Adolescent ; Adult ; Aged ; Aged, 80 and over ; Biopsy/methods ; Female ; Humans ; Male ; Middle Aged ; Muscle, Skeletal/pathology ; Nerve Fibers, Myelinated/metabolism ; Nerve Fibers, Myelinated/pathology ; Nerve Fibers, Unmyelinated/metabolism ; Nerve Fibers, Unmyelinated/pathology ; Neural Cell Adhesion Molecules/metabolism ; Peripheral Nerves/pathology ; Peripheral Nervous System Diseases/diagnosis ; Retrospective Studies ; Severity of Illness Index ; Skin/innervation ; Skin/pathology ; Statistics as Topic ; Young Adult
    Chemical Substances NCAM protein (681-695), human ; Neural Cell Adhesion Molecules
    Language English
    Publishing date 2016-02
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 438353-9
    ISSN 1097-4598 ; 0148-639X
    ISSN (online) 1097-4598
    ISSN 0148-639X
    DOI 10.1002/mus.24740
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Prevalence of Axonal Sensory Neuropathy With IgM Binding to Trisulfated Heparin Disaccharide in Patients With Fibromyalgia.

    Malik, Asma / Lopate, Glenn / Hayat, Ghazala / Jones, Jacqueline / Atluri, Rama / Malo, Bassam / Pestronk, Alan

    Journal of clinical neuromuscular disease

    2019  Volume 20, Issue 3, Page(s) 103–110

    Abstract: Objective: To assess the intraepidermal nerve fiber density in patients diagnosed with fibromyalgia (FM) and to evaluate the role of IgM binding to trisulfated heparin disaccharide (TS-HDS) in these patients.: Methods: FM is a poorly understood pain ... ...

    Abstract Objective: To assess the intraepidermal nerve fiber density in patients diagnosed with fibromyalgia (FM) and to evaluate the role of IgM binding to trisulfated heparin disaccharide (TS-HDS) in these patients.
    Methods: FM is a poorly understood pain disorder with several proposed pathophysiologic mechanisms. It is characterized by widespread pain, fatigue, and sleep abnormalities. Small fiber neuropathy (SFN) has been proposed as an underlying mechanism, and patients with FM have been shown to have a reduction in the intraepidermal nerve fiber density. An underlying inflammatory process that could be a result of autoimmune phenomena has also been suggested. Non-length-dependent SFN (NLDSFN) has been shown to have a higher incidence of autoimmune disease. Twenty-two patients with established diagnosis of FM underwent skin biopsy at 2 sites; 10 cm above the lateral malleolus and 10 cm above the patella. Serum IgM binding to TS-HDS was assayed using an ELISA method.
    Results: A total of 5/22 patients had positive TS-HDS antibodies; of these, 4 had NLDSFN (P = 0.0393). Comparison with a control group at Washington University showed no significant difference in percentage with TS-HDS antibodies (P = 0.41). When compared with Washington University database of skin biopsy, there was a trend for an increased percentage of NLDSFN in patients with FM (P = 0.06).
    Conclusions: This study further supports the hypothesis that a subgroup of patients with FM has SFN. We suggest a correlation between the presence of NLDSFN and TS-HDS antibodies.
    MeSH term(s) Adult ; Antibodies, Monoclonal/blood ; Disaccharides/immunology ; Disaccharides/metabolism ; Female ; Fibromyalgia/complications ; Fibromyalgia/epidemiology ; Heparin/analogs & derivatives ; Heparin/metabolism ; Humans ; Immunoglobulin M/pharmacology ; Male ; Middle Aged ; Prevalence ; Protein Binding ; Small Fiber Neuropathy/epidemiology ; Small Fiber Neuropathy/etiology
    Chemical Substances Antibodies, Monoclonal ; Disaccharides ; Immunoglobulin M ; heparin disaccharide ; Heparin (9005-49-6)
    Language English
    Publishing date 2019-01-20
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1454947-5
    ISSN 1537-1611 ; 1522-0443
    ISSN (online) 1537-1611
    ISSN 1522-0443
    DOI 10.1097/CND.0000000000000236
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Myovascular innervation: axon loss in small-fiber neuropathies.

    Dori, Amir / Lopate, Glenn / Keeling, Richard / Pestronk, Alan

    Muscle & nerve

    2015  Volume 51, Issue 4, Page(s) 514–521

    Abstract: Introduction: Vascular denervation occurs in some neuropathies, but measurement of small perivascular axons has been difficult.: Methods: We evaluated 31 consecutive patients who had both muscle and skin biopsies. We quantitated myovascular ... ...

    Abstract Introduction: Vascular denervation occurs in some neuropathies, but measurement of small perivascular axons has been difficult.
    Methods: We evaluated 31 consecutive patients who had both muscle and skin biopsies. We quantitated myovascular innervation by staining unmyelinated axons with peripherin and non-myelinating Schwann cells with neural cell adhesion molecule and comparing their areas.
    Results: Perivascular unmyelinated axon-Schwann (UAS) ratios correlated with axon density in skin (r = 0.679; P < 0.0001). Low UAS ratios (≤0.25) had a sensitivity of 90% and specificity of 91% for a clinical diagnosis of small-fiber neuropathy (P < 0.0001). Autonomic features were more common in patients with low perivascular UAS ratios (P = 0.002). A patient subgroup with myovascular, but not skin, denervation commonly had muscle discomfort and autonomic features.
    Conclusions: UAS ratio measurements, comparing axons and associated non-myelinating Schwann cells, can quantitate perivascular innervation. Small-fiber neuropathies are often associated with myovascular denervation. Some patients with muscle discomfort have selective myovascular denervation.
    MeSH term(s) Adult ; Aged ; Axons/pathology ; Biopsy ; Erythromelalgia/diagnosis ; Erythromelalgia/physiopathology ; Female ; Humans ; Male ; Middle Aged ; Schwann Cells/pathology ; Skin/innervation ; Sural Nerve/pathology
    Language English
    Publishing date 2015-04
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 438353-9
    ISSN 1097-4598 ; 0148-639X
    ISSN (online) 1097-4598
    ISSN 0148-639X
    DOI 10.1002/mus.24356
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Inflammatory demyelinating neuropathies.

    Lopate, Glenn / Pestronk, Alan

    Current treatment options in neurology

    2011  Volume 13, Issue 2, Page(s) 131–142

    Abstract: Opinion statement: The primary goal of therapy in patients with the Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is improved strength and functional ability. Improvement in pain, sensory loss, gait ... ...

    Abstract Opinion statement: The primary goal of therapy in patients with the Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is improved strength and functional ability. Improvement in pain, sensory loss, gait disorder, and autonomic instability are other goals of therapy. Patients with very mild symptoms that do not interfere with activities of daily living can be observed for deterioration without treatment. For GBS, standard care includes plasma exchange (PE) or human immune globulin (HIG), both of which have similar efficacy. Supportive care in the intensive care unit may be needed for those patients with severe bulbar or respiratory weakness. We treat most patients with PE, usually performing an exchange every other day for a total of five exchanges. We use HIG in children, if there are antiglycolipid antibodies (eg, anti-GM1 or anti-GQ1b) or if there is a contraindication to PE, such as hemodynamic instability; severe renal, hepatic, or cardiac disease; or poor venous access. For CIDP, there are no guidelines concerning the initial choice of therapy. Corticosteroids, HIG, and PE have all been shown to be effective in prospective, randomized controlled trials, and comparison trials have shown equal efficacy among these three immunomodulating therapies. The choice of therapy depends on several factors including disease severity, concomitant illnesses, side-effect profile, potential drug interactions, venous access, age-related risks, and cost of treatment. In patients with moderate to severe symptoms, treatment with corticosteroids or HIG should be used. We usually use high-dose, intermittent methylprednisolone as the initial drug of choice. We believe intermittent corticosteroids are better than HIG because of their good safety profile, low cost, ease of administration (can be given intravenously or by mouth), and proven efficacy. If there is a major contraindication to corticosteroids, then HIG is offered. PE is less well tolerated and is primarily used as a third choice and only for a few weeks to months to induce initial improvement. Once symptoms are improving, the dose of corticosteroids or HIG should be tapered with the goal of eventual discontinuation depending on patient response. Patients who do not respond to initial therapy, experience adverse effects from the initial immunomodulating agent, or require chronic treatment can be treated with another first-line agent or one of several second-line agents.
    Language English
    Publishing date 2011-01-15
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2057342-X
    ISSN 1534-3138 ; 1092-8480
    ISSN (online) 1534-3138
    ISSN 1092-8480
    DOI 10.1007/s11940-011-0114-0
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: CANOMAD and other chronic ataxic neuropathies with disialosyl antibodies (CANDA).

    Garcia-Santibanez, Rocio / Zaidman, Craig M / Sommerville, R Brian / Lopate, Glenn / Weihl, Conrad C / Pestronk, Alan / Bucelli, Robert C

    Journal of neurology

    2018  Volume 265, Issue 6, Page(s) 1402–1409

    Abstract: Introduction: CANOMAD/CANDA are syndromes characterized by ataxic neuropathy, ophthalmoplegia, monoclonal gammopathy, cold agglutinins and disialosyl antibodies.: Methods: A retrospective review of our neuromuscular autoantibody panel database was ... ...

    Abstract Introduction: CANOMAD/CANDA are syndromes characterized by ataxic neuropathy, ophthalmoplegia, monoclonal gammopathy, cold agglutinins and disialosyl antibodies.
    Methods: A retrospective review of our neuromuscular autoantibody panel database was performed. Anti-GD1b seropositive patients with ataxia were included.
    Results: Eleven patients were identified. Median age at onset was 56 years. Median disease duration was 6 years. All patients had gait disorders. Nine had ocular motility abnormalities. Most had a monoclonal protein and all had elevated serum IgM. Electrodiagnostic studies showed a mixed axonal/demyelinating pattern (6), an axonal pattern (4), or a pure demyelinating pattern (1). Ultrasounds showed nerve enlargement patterns consistent with acquired demyelination. A nerve biopsy showed near complete loss of myelinated axons with preservation of smaller axons. Rituximab was the most effective immunotherapy.
    Conclusion: CANOMAD/CANDA are rare and debilitating disorders with characteristic clinical and diagnostic findings. In our cohort, nerve ultrasound showed regional nerve enlargement and rituximab was the most effective immunomodulatory therapy.
    MeSH term(s) Adult ; Aged ; Ataxia/diagnostic imaging ; Ataxia/immunology ; Ataxia/pathology ; Ataxia/therapy ; Autoantibodies/blood ; Chronic Disease ; Female ; Follow-Up Studies ; Gangliosides/immunology ; Humans ; Immunologic Factors/therapeutic use ; Immunomodulation ; Male ; Middle Aged ; Neural Conduction ; Peripheral Nerves/diagnostic imaging ; Peripheral Nerves/pathology ; Peripheral Nerves/physiopathology ; Retrospective Studies ; Rituximab/therapeutic use ; Ultrasonography
    Chemical Substances Autoantibodies ; Gangliosides ; Immunologic Factors ; ganglioside, GD1b (19553-76-5) ; Rituximab (4F4X42SYQ6)
    Language English
    Publishing date 2018-04-09
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 187050-6
    ISSN 1432-1459 ; 0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
    ISSN (online) 1432-1459
    ISSN 0340-5354 ; 0012-1037 ; 0939-1517 ; 1619-800X
    DOI 10.1007/s00415-018-8853-4
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Cramps and small-fiber neuropathy.

    Lopate, Glenn / Streif, Elizabeth / Harms, Matthew / Weihl, Christopher / Pestronk, Alan

    Muscle & nerve

    2013  Volume 48, Issue 2, Page(s) 252–255

    Abstract: Introduction: Muscle cramps are a common complaint and are thought to arise from spontaneous discharges of the motor nerve terminal. Polyneuropathy is often causative, but small-fiber neuropathy (SFN) has not been assessed.: Methods: We performed ... ...

    Abstract Introduction: Muscle cramps are a common complaint and are thought to arise from spontaneous discharges of the motor nerve terminal. Polyneuropathy is often causative, but small-fiber neuropathy (SFN) has not been assessed.
    Methods: We performed skin biopsies on consecutive patients with cramps but without neuropathic complaints. Twelve patients were biopsied, 8 with normal small-fiber sensation.
    Results: Seven patients had decreased intraepidermal nerve fiber density (IENFD), 2 with non-length-dependent loss. A cause for neuropathy was found in 1 patient with cramp-fasciculation syndrome. Creatine kinase was elevated in 8 patients, 4 with decreased IENFD. Muscle biopsy, performed in 8 patients, but was diagnostic in only 1, with McArdle disease.
    Conclusions: Our data show that 60% of patients with muscle cramps who lack neuropathic complaints have SFN, as documented by decreased IENFD. Cramps may originate as local mediators of inflammation released by damaged small nerve that excite intramuscular nerves.
    MeSH term(s) Action Potentials/physiology ; Adult ; Biopsy ; Creatine Kinase/metabolism ; Electromyography ; Female ; Humans ; Male ; Middle Aged ; Muscle Cramp/complications ; Muscle Cramp/diagnosis ; Nerve Fibers/physiology ; Neural Conduction/physiology ; Peripheral Nervous System Diseases/complications ; Peripheral Nervous System Diseases/diagnosis ; Skin/pathology
    Chemical Substances Creatine Kinase (EC 2.7.3.2)
    Language English
    Publishing date 2013-08
    Publishing country United States
    Document type Journal Article
    ZDB-ID 438353-9
    ISSN 1097-4598 ; 0148-639X
    ISSN (online) 1097-4598
    ISSN 0148-639X
    DOI 10.1002/mus.23757
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article: Motor neuron disease associated with copper deficiency.

    Weihl, Conrad C / Lopate, Glenn

    Muscle & nerve

    2006  Volume 34, Issue 6, Page(s) 789–793

    Abstract: Copper deficiency in humans is a rare cause of myeloneuropathy that usually presents with a spastic ataxic gait, hyperreflexia, and distal sensory loss similar to that seen in patients with subacute combined degeneration. We describe three copper- ... ...

    Abstract Copper deficiency in humans is a rare cause of myeloneuropathy that usually presents with a spastic ataxic gait, hyperreflexia, and distal sensory loss similar to that seen in patients with subacute combined degeneration. We describe three copper-deficient patients, two of whom were referred with a presumptive diagnosis of amyotrophic lateral sclerosis, who had progressive asymmetric weakness or electrodiagnostic findings of proximal and distal denervation suggestive of lower motor neuron disease. Copper replacement resulted in stabilization or mild improvement in weakness. The clinical spectrum of human copper deficiency should include lower motor neuron disease in addition to a syndrome of spastic ataxia.
    MeSH term(s) Adult ; Amyotrophic Lateral Sclerosis/diagnosis ; Amyotrophic Lateral Sclerosis/drug therapy ; Amyotrophic Lateral Sclerosis/etiology ; Copper/blood ; Copper/deficiency ; Electrodiagnosis ; Female ; Gluconates/therapeutic use ; Humans ; Male ; Middle Aged ; Motor Neuron Disease/diagnosis ; Motor Neuron Disease/drug therapy ; Motor Neuron Disease/etiology ; Treatment Outcome
    Chemical Substances Gluconates ; Copper (789U1901C5)
    Language English
    Publishing date 2006-12
    Publishing country United States
    Document type Case Reports ; Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 438353-9
    ISSN 1097-4598 ; 0148-639X
    ISSN (online) 1097-4598
    ISSN 0148-639X
    DOI 10.1002/mus.20631
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Autophagic vacuolar pathology in desminopathies.

    Weihl, Conrad C / Iyadurai, Stanley / Baloh, Robert H / Pittman, Sara K / Schmidt, Robert E / Lopate, Glenn / Pestronk, Alan / Harms, Matthew B

    Neuromuscular disorders : NMD

    2015  Volume 25, Issue 3, Page(s) 199–206

    Abstract: Autophagic vacuolar myopathies are an emerging group of muscle diseases with common pathologic features. These include autophagic vacuoles containing both lysosomal and autophagosomal proteins sometimes lined with sarcolemmal proteins such as dystrophin. ...

    Abstract Autophagic vacuolar myopathies are an emerging group of muscle diseases with common pathologic features. These include autophagic vacuoles containing both lysosomal and autophagosomal proteins sometimes lined with sarcolemmal proteins such as dystrophin. These features have been most clearly described in patients with Danon's disease due to LAMP2 deficiency and X-linked myopathy with excessive autophagy (XMEA) due to mutations in VMA21. Disruptions of these proteins lead to lysosomal dysfunction and subsequent autophagic vacuolar pathology. We performed whole exome sequencing on two families with autosomal dominantly inherited myopathies with autophagic vacuolar pathology and surprisingly identified a p.R454W tail domain mutation and a novel p.S6W head domain mutation in desmin, DES. In addition, re-evaluation of muscle tissue from another family with a novel p.I402N missense DES mutation also identified autophagic vacuoles. We suggest that autophagic vacuoles may be an underappreciated pathology present in desminopathy patient muscle. Moreover, autophagic vacuolar pathology can be due to genetic etiologies unrelated to primary defects in the lysosomes or autophagic machinery. Specifically, cytoskeletal derangement and the accumulation of aggregated proteins such as desmin may activate the autophagic system leading to the pathologic features of an autophagic vacuolar myopathy.
    MeSH term(s) Adult ; Aged ; Cardiomyopathies/genetics ; Cardiomyopathies/pathology ; Cardiomyopathies/physiopathology ; Desmin/genetics ; Family ; Female ; Hand/pathology ; Humans ; Immunohistochemistry ; Lysosomal Storage Diseases/genetics ; Lysosomal Storage Diseases/pathology ; Lysosomal Storage Diseases/physiopathology ; Male ; Microscopy, Electron ; Middle Aged ; Muscle, Skeletal/pathology ; Muscular Diseases/genetics ; Muscular Diseases/pathology ; Muscular Diseases/physiopathology ; Muscular Dystrophies/genetics ; Muscular Dystrophies/pathology ; Muscular Dystrophies/physiopathology ; Mutation ; Pedigree ; Sequence Analysis, DNA
    Chemical Substances Desmin
    Language English
    Publishing date 2015-03
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ZDB-ID 1077681-3
    ISSN 1873-2364 ; 0960-8966
    ISSN (online) 1873-2364
    ISSN 0960-8966
    DOI 10.1016/j.nmd.2014.12.002
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article: Treatment of chronic inflammatory demyelinating polyneuropathy with high-dose intermittent intravenous methylprednisolone.

    Lopate, Glenn / Pestronk, Alan / Al-Lozi, Muhammad

    Archives of neurology

    2005  Volume 62, Issue 2, Page(s) 249–254

    Abstract: Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) causes progressive disability due to weakness but responds to immunomodulating medication, including oral prednisone and intravenous (IV) immunoglobulin (IVIg). However, there ... ...

    Abstract Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) causes progressive disability due to weakness but responds to immunomodulating medication, including oral prednisone and intravenous (IV) immunoglobulin (IVIg). However, there is no consensus on initial therapy, and both of these treatments have drawbacks with long-term treatment.
    Objective: To review the efficacy and safety of high-dose, intermittent IV methylprednisolone (IVMP) as initial and long-term maintenance therapy for patients with CIDP.
    Design: A retrospective medical record review between 1992 and 2003 of outcomes in CIDP, comparing patients in 3 cohorts depending on whether their primary treatment was IVMP, IVIg, or oral immunosuppression with prednisone or cyclosporine.
    Setting: Washington University Neuromuscular Disease Center (St Louis, Mo), outpatient and inpatient records.
    Patients: Patients with clinical and electrophysiologic evidence of CIDP were identified. Of 57 patients, 39 had sufficient data for full analysis.
    Main outcome measures: Quantitative muscle testing with a handheld dynamometer. Medication profiles and adverse effects were also recorded.
    Results: There was no significant difference in the mean improvement in quantitative muscle testing at 6 months or at the last clinic visit (an average of 4.5 years later) among the 3 groups. Fewer patients treated with oral immunosuppression improved at 6 months, but at the last visit, 81% to 88% improved in all 3 groups. Less weight gain and fewer cushingoid features affected patients treated with IVMP (19%) compared with patients treated with oral prednisone (58%).
    Conclusions: Treatment of patients with CIDP using high-dose intermittent IVMP results in improved strength equal to that with IVIg and oral prednisone. The frequency of occurrences of weight gain and cushingoid features with IVMP is less than that with oral prednisone. Intravenous methylprednisolone should be considered for initial and long-term therapy in CIDP when patients have disability due to weakness.
    MeSH term(s) Administration, Oral ; Adult ; Aged ; Aged, 80 and over ; Anti-Inflammatory Agents/administration & dosage ; Anti-Inflammatory Agents/adverse effects ; Chi-Square Distribution ; Clinical Trials as Topic ; Cohort Studies ; Female ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Infusions, Intravenous/methods ; Male ; Methylprednisolone/administration & dosage ; Methylprednisolone/adverse effects ; Middle Aged ; Muscle, Skeletal/drug effects ; Muscle, Skeletal/physiopathology ; Neural Conduction/drug effects ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/drug therapy ; Retrospective Studies ; Time Factors ; Treatment Outcome
    Chemical Substances Anti-Inflammatory Agents ; Immunoglobulins, Intravenous ; Methylprednisolone (X4W7ZR7023)
    Language English
    Publishing date 2005-02
    Publishing country United States
    Document type Comparative Study ; Journal Article
    ZDB-ID 80049-1
    ISSN 1538-3687 ; 0003-9942
    ISSN (online) 1538-3687
    ISSN 0003-9942
    DOI 10.1001/archneur.62.2.249
    Database MEDical Literature Analysis and Retrieval System OnLINE

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