Stiff Leg Syndrome and Myelitis with Anti-Amphiphysin Antibodies: A Common Physiopathology

Chamard, Ludivine; Magnin, Éloi; Berger, Eric G.; Hagenkötter, Beate; Rumbach, L.; Bataillard, Marc

doi:10.1159/000331592

Cited by 16 publications

(6 citation statements)

References 19 publications

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“…We believe that this category can potentially include autoimmune cases for which a known antibody is either present in titers that evade detection, or are positive for antibodies that are yet to be recognized. The response to 75 Kosseifi et al 76 Schmidt et al 77 Thumen et al 78 Chamard et al 79 Lemieux et al 80 Byrne et al 81 Krishna et al 82 Aghajanzadeh et al 83 Badzek et al 84 Derksen et al 38 Rakcevic et al 85 Kelly et al 86 Kobayashi et al 87 Koca et al 88 Laroumagne et al Hegyi. 40 Iwata et al 34 Prasad 89 Sanefuji et al 90 Schreiber et al 41 Lorenzoni et al 33 Clardy et al 14 Newton et al 42 Vicente-Valor et al 43 Sharma et al 91 Pakeerappa et al 92 Ueno et al 35 Tremor and Other Hyperkinetic Movements http://www.tremorjournal.org…”

Section: Discussionmentioning

confidence: 99%

Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports

Sarva

Deik

Ullah

et al. 2016

Tremor and Other Hyperkinetic Movements

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Background: ''Classic'' stiff person syndrome (SPS) features stiffness, anti-glutamic acid decarboxylase (anti-GAD) antibodies, and other findings. Anti-GAD antibodies are also detected in some neurological syndromes (such as ataxia) in which stiffness is inconsistently present. Patients with otherwise ''classic'' SPS may either lack anti-GAD antibodies or be seropositive for others. Hence, SPS cases appear to fall within a clinical spectrum that includes conditions such as progressive encephalomyelitis with rigidity and myoclonus (PERM), which exhibits brainstem and autonomic features. We have compiled herein SPS-spectrum cases reported since 2010, and have segregated them on the basis of likely disease mechanism (autoimmune, paraneoplastic, or cryptogenic) for analysis. Methods: The phrases ''stiff person syndrome'', ''PERM'', ''anti-GAD antibody syndrome'', and ''glycine receptor antibody neurological disorders'' were searched for in PubMed in January 2015. The results were narrowed to 72 citations after excluding non-English and duplicate reports. Clinical descriptions, laboratory data, management, and outcomes were categorized, tabulated, and analyzed. Results: Sixty-nine autoimmune, 19 paraneoplastic, and 13 cryptogenic SPS-spectrum cases were identified. SPS was the predominant diagnosis among the groups. Roughly two-thirds of autoimmune and paraneoplastic cases were female. Anti-GAD antibodies were most frequently identified, followed by antiamphiphysin among paraneoplastic cases and by anti-glycine receptor antibodies among autoimmune cases. Benzodiazepines were the most commonly used medications. Prognosis seemed best for cryptogenic cases; malignancy worsened that of paraneoplastic cases. Discussion: Grouping SPS-spectrum cases by pathophysiology provided insights into work-up, treatment, and prognosis. Ample phenotypic and serologic variations are present within the categories. Ruling out malignancy and autoimmunity is appropriate for suspected SPS-spectrum cases.

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Section: Discussionmentioning

confidence: 99%

Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports

Sarva

Deik

Ullah

et al. 2016

Tremor and Other Hyperkinetic Movements

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“…Anti-Ri (ANNA-2) Anti-Ri antibodies are usually associated with lung or breast carcinoma 393,394 Anti-amphiphysin IgG Classically associated with breast cancer and stiff man syndrome 395,396 Anti-glutamic acid decarboxylase (GAD65)…”

Section: Paraneoplastic Antibody Comment Referencesmentioning

confidence: 99%

Transverse Myelitis

et al. 2013

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Transverse myelitis (TM) includes a pathobiologically heterogeneous syndrome characterized by acute or subacute spinal cord dysfunction resulting in paresis, a sensory level, and autonomic (bladder, bowel, and sexual) impairment below the level of the lesion. Etiologies for TM can be broadly classified as parainfectious, paraneoplastic, drug/toxin-induced, systemic autoimmune disorders, and acquired demyelinating diseases. We discuss the clinical evaluation, workup, and acute and long-term management of patients with TM. Additionally, we briefly discuss various disease entities that may cause TM and their salient distinguishing features, as well as disorders that may mimic TM.

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“…Immunoglobulin may suppress the activities of anti-GAD65 antibody by accelerating the rate of IgG catabolism, and the other immunomodulatory effects of immunoglobulin on the neutralization of cytokines and T cells may also play a complementary role (7,40). Glucocorticoids (e.g., induced as pulse therapy with 500-1,000 mg/day methylprednisolone for 3-5 days and an oral maintenance dose with prednisone 1 mg/kg or 60-80 mg po., oral tapering) could be efficient in treating SLS (24,27,30,32). Plasma exchange may benefit patients with SLS who failed to respond to baclofen and diazepam and cannot tolerate IVIG and glucocorticoids by reducing serum levels of antibodies and other proinflammatory mediators (13,29).…”

Section: Discussionmentioning

confidence: 99%

Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature

Huang

Meng

Xian³

et al. 2020

Front. Neurol.

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Stiff limb syndrome (SLS) is a rare autoimmune-related central nervous system disorder, resulting in stiffness and spasms of limbs since onset with rare involvement of the truncal muscles. However, SLS patients will gain notable effects by appropriate therapy focusing on symptomatic treatment and immunotherapy. We reported on a 55-year-old female who showed typical painful spasms in both lower limbs and abduction of the right eyeball that partially responded to low-dose diazepam and had high-titer anti-glutamic acid decarboxylase (anti-GAD) antibody. Electromyography (EMG) only showed continuous motor unit activity (CMUA) in the anterior tibialis and right triceps. Eventually, our patient was diagnosed with SLS and treated with intravenous immunoglobulin (IVIG) and glucocorticoid combined simultaneously. She obtained notable effects. We also review and summarize the current literature on clinical characteristics, coexisting disease, treatment, and outcome of 40 patients with SLS. We hope that this report will provide a basis for further understanding of SLS and promote the formation of more advanced diagnosis and treatment processes.

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Stiff Leg Syndrome and Myelitis with Anti-Amphiphysin Antibodies: A Common Physiopathology

Cited by 16 publications

References 19 publications

Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports

Clinical Spectrum of Stiff Person Syndrome: A Review of Recent Reports

Transverse Myelitis

Effectiveness of Combined Immunoglobulin and Glucocorticoid Treatments in a Patient With Stiff Limb Syndrome: Case Report and Review of the Literature

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