Neurological Syndromes Associated with Anti-GAD Antibodies
Glutamic acid decarboxylase (GAD) is an intracellular enzyme whose physiologic function is the decarboxylation of glutamate to gamma-aminobutyric acid (GABA), the main inhibitory neurotransmitter within the central nervous system. GAD antibodies (Ab) have been associated with multiple neurological s...
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| Format: | Article |
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MDPI AG
2020-05-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/21/10/3701 |
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| author | Maëlle Dade Giulia Berzero Cristina Izquierdo Marine Giry Marion Benazra Jean-Yves Delattre Dimitri Psimaras Agusti Alentorn |
| author_facet | Maëlle Dade Giulia Berzero Cristina Izquierdo Marine Giry Marion Benazra Jean-Yves Delattre Dimitri Psimaras Agusti Alentorn |
| author_sort | Maëlle Dade |
| collection | DOAJ |
| description | Glutamic acid decarboxylase (GAD) is an intracellular enzyme whose physiologic function is the decarboxylation of glutamate to gamma-aminobutyric acid (GABA), the main inhibitory neurotransmitter within the central nervous system. GAD antibodies (Ab) have been associated with multiple neurological syndromes, including stiff-person syndrome, cerebellar ataxia, and limbic encephalitis, which are all considered to result from reduced GABAergic transmission. The pathogenic role of GAD Ab is still debated, and some evidence suggests that GAD autoimmunity might primarily be cell-mediated. Diagnosis relies on the detection of high titers of GAD Ab in serum and/or in the detection of GAD Ab in the cerebrospinal fluid. Due to the relative rarity of these syndromes, treatment schemes and predictors of response are poorly defined, highlighting the unmet need for multicentric prospective trials in this population. Here, we reviewed the main clinical characteristics of neurological syndromes associated with GAD Ab, focusing on pathophysiologic mechanisms. |
| first_indexed | 2024-03-10T19:38:06Z |
| format | Article |
| id | doaj.art-d8eb1c8f156948c382a5026b497f94eb |
| institution | Directory Open Access Journal |
| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-10T19:38:06Z |
| publishDate | 2020-05-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | International Journal of Molecular Sciences |
| spelling | doaj.art-d8eb1c8f156948c382a5026b497f94eb2023-11-20T01:34:55ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672020-05-012110370110.3390/ijms21103701Neurological Syndromes Associated with Anti-GAD AntibodiesMaëlle Dade0Giulia Berzero1Cristina Izquierdo2Marine Giry3Marion Benazra4Jean-Yves Delattre5Dimitri Psimaras6Agusti Alentorn7AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Service de Neurologie 2-Mazarin, 75013 Paris, FranceAP-HP, Groupe Hospitalier Pitié-Salpêtrière, Service de Neurologie 2-Mazarin, 75013 Paris, FranceDepartment of Neuroscience, Hospital Universitari Germans Trias i Pujol, Universitat Autònoma de Barcelona, 08916 Badalona, SpainSorbonne Université, Inserm, CNRS, UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, 75013 Paris, FranceSorbonne Université, Inserm, CNRS, UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, 75013 Paris, FranceAP-HP, Groupe Hospitalier Pitié-Salpêtrière, Service de Neurologie 2-Mazarin, 75013 Paris, FranceAP-HP, Groupe Hospitalier Pitié-Salpêtrière, Service de Neurologie 2-Mazarin, 75013 Paris, FranceAP-HP, Groupe Hospitalier Pitié-Salpêtrière, Service de Neurologie 2-Mazarin, 75013 Paris, FranceGlutamic acid decarboxylase (GAD) is an intracellular enzyme whose physiologic function is the decarboxylation of glutamate to gamma-aminobutyric acid (GABA), the main inhibitory neurotransmitter within the central nervous system. GAD antibodies (Ab) have been associated with multiple neurological syndromes, including stiff-person syndrome, cerebellar ataxia, and limbic encephalitis, which are all considered to result from reduced GABAergic transmission. The pathogenic role of GAD Ab is still debated, and some evidence suggests that GAD autoimmunity might primarily be cell-mediated. Diagnosis relies on the detection of high titers of GAD Ab in serum and/or in the detection of GAD Ab in the cerebrospinal fluid. Due to the relative rarity of these syndromes, treatment schemes and predictors of response are poorly defined, highlighting the unmet need for multicentric prospective trials in this population. Here, we reviewed the main clinical characteristics of neurological syndromes associated with GAD Ab, focusing on pathophysiologic mechanisms.https://www.mdpi.com/1422-0067/21/10/3701glutamic acid decarboxylaseGAD65 autoimmunityneuronal antibodiesparaneoplastic neurological syndromeslimbic encephalitisautoimmune epilepsy |
| spellingShingle | Maëlle Dade Giulia Berzero Cristina Izquierdo Marine Giry Marion Benazra Jean-Yves Delattre Dimitri Psimaras Agusti Alentorn Neurological Syndromes Associated with Anti-GAD Antibodies International Journal of Molecular Sciences glutamic acid decarboxylase GAD65 autoimmunity neuronal antibodies paraneoplastic neurological syndromes limbic encephalitis autoimmune epilepsy |
| title | Neurological Syndromes Associated with Anti-GAD Antibodies |
| title_full | Neurological Syndromes Associated with Anti-GAD Antibodies |
| title_fullStr | Neurological Syndromes Associated with Anti-GAD Antibodies |
| title_full_unstemmed | Neurological Syndromes Associated with Anti-GAD Antibodies |
| title_short | Neurological Syndromes Associated with Anti-GAD Antibodies |
| title_sort | neurological syndromes associated with anti gad antibodies |
| topic | glutamic acid decarboxylase GAD65 autoimmunity neuronal antibodies paraneoplastic neurological syndromes limbic encephalitis autoimmune epilepsy |
| url | https://www.mdpi.com/1422-0067/21/10/3701 |
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