Anti-GAD negative stiff person syndrome with a favorable response to intravenous methylprednisolone: An experience over evidence

Sharma, Bhawna; Nagpal, Kadam; Prakash, Swayam; Gupta, Pankaj

doi:10.4103/0028-3886.128332

Cited by 4 publications

(3 citation statements)

References 1 publication

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“…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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“…Meinck et al23 found some rare symptoms in some SPS patients: these symptoms consist of (1) an aura-like feeling preceding spontaneous spasmodic attacks; (2) a motor pattern observed during spasmodic jerks, with brief opisthotonos, stiffening of the slightly abducted legs and inversion of the plantar-flexed feet; (3) a paroxysmal fear while crossing a free space unaided, or even thinking about it 23. Stiff-person syndrome can also be found without anti-GAD antibodies,24 however, over 60% of SPS patients have these antibodies in the blood and the CSF,25 while some have other antibodies like anti-amphiphysin, anti-gephyrin, and anti-gamma-amino butyric acid A receptor-associated protein (GABARAP) antibodies 26. Blood tests often contain anti-thyroid, anti-nuclear, anti-ribonucleoprotein (RNP), anti-gliadin, and anti-intrinsic factor 27…”

Section: Stiff-person Syndromementioning

confidence: 99%

Anti-glutamic acid decarboxylase antibody positive neurological syndromes

Tohid¹

2016

NSJ

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A rare kind of antibody, known as anti-glutamic acid decarboxylase (GAD) autoantibody, is found in some patients. The antibody works against the GAD enzyme, which is essential in the formation of gamma aminobutyric acid (GABA), an inhibitory neurotransmitter found in the brain. Patients found with this antibody present with motor and cognitive problems due to low levels or lack of GABA, because in the absence or low levels of GABA patients exhibit motor and cognitive symptoms. The anti-GAD antibody is found in some neurological syndromes, including stiff-person syndrome, paraneoplastic stiff-person syndrome, Miller Fisher syndrome (MFS), limbic encephalopathy, cerebellar ataxia, eye movement disorders, and epilepsy. Previously, excluding MFS, these conditions were called ‘hyperexcitability disorders’. However, collectively, these syndromes should be known as “anti-GAD positive neurological syndromes.” An important limitation of this study is that the literature is lacking on the subject, and why patients with the above mentioned neurological problems present with different symptoms has not been studied in detail. Therefore, it is recommended that more research is conducted on this subject to obtain a better and deeper understanding of these anti-GAD antibody induced neurological syndromes.

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“…La determinación de los anticuerpos antiglutamato descarboxilasa (específicamente las isoformas GAD65 o GAD67) en sangre o líquido cefalorraquídeo es complementaria para el diagnóstico. Si bien los anticuerpos anti-GAD están presentes en el SPS, pueden ser negativos 18,19 . En los casos de SPS con anti-GAD positivos, la proteinorraquia y la pleiocitosis constituyen los hallazgos más frecuentes y en algunos casos pueden encontrarse bandas oligoclonales y anticuerpos anti-GAD 20 .…”

Section: A R C H I V O S D E N E U R O C I E N C I a S C O Munclassified

Síndrome de la persona rígida Caso clínico y revisión de la literatura

Cabrera-Espinosa¹,

Enríquez-Coronel²

2016

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Anti-GAD negative stiff person syndrome with a favorable response to intravenous methylprednisolone: An experience over evidence

Cited by 4 publications

References 1 publication

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

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

Anti-glutamic acid decarboxylase antibody positive neurological syndromes

Síndrome de la persona rígida Caso clínico y revisión de la literatura

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