Case 27-2012

Byrne, Thomas N.; Isakoff, Steven J.; Rincon, Sandra; Gudewicz, Thomas M

doi:10.1056/nejmcpc1114036

Cited by 10 publications

(1 citation statement)

References 20 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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Limbic Encephalitis and Related Cortical Syndromes

Rubio-Agustí

Salavert

Bataller

2012

Curr Treat Options Neurol

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Patients with limbic encephalitis and related cortical syndromes develop neurologic and psychiatric symptoms due to autoimmune damage of limbic and extralimbic brain areas. Some patients develop limbic encephalitis as a paraneoplastic manifestation of systemic cancer, where immune tolerance is thought to be lost. Serum and cerebrospinal antibodies against neuronal antigens can be detected in most patients. According to the type of antibodies found, patients may be classified into two broad groups, with implications for neurological and cancer diagnosis, treatment and prognosis. Patients with antibodies to intracellular neuronal antigens (onconeuronal antibodies: Hu, Ma2, amphiphysin, CV2/CRMP5) almost invariably have an underlying neoplasm (lung, testis, breast, etc.), often require aggressive combined antineoplastic and immunomodulatory treatment and their prognosis is usually poor. Patients with antibodies to cell-membrane antigens may present with classical limbic encephalitis (anti-LGI1 antibodies, other) or with a more diffuse stereotyped encephalitis, combining psychiatric symptoms, seizures, dyskinesias, catatonia and central hypoventilation (NMDAR-encephalitis). These patients may or may not have an associated tumor (thymoma, ovarian teratoma, etc.), are likely to improve with immunomodulatory treatment (and tumor removal for paraneoplastic cases) and have, overall, a better prognosis. Immunotherapy in patients with limbic encephalitis may be escalated according to the severity of neurological symptoms, the associated antibodies and the predicted success of antineoplastic treatment. First-line immunotherapies include high dose steroids, intravenous immunoglobulins and plasma exchange. Second-line drugs include Rituximab and Cyclophosphamide. For some patients, maintenance treatment with immunosuppressants might be required to prevent deterioration or relapses. Supportive treatment includes antiepileptic drugs, psychopharmacologic agents and scrutiny and prophylaxis of opportunistic infections.

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Imaging characteristics of neuroimmunological disorders

Haralur,

Nallani

2023

Translational Neuroimmunology, Volume 7

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Case 27-2012

Cited by 10 publications

References 20 publications

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

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

Limbic Encephalitis and Related Cortical Syndromes

Imaging characteristics of neuroimmunological disorders

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