Neurological Syndromes Associated with Anti-GAD Antibodies
Dade, Maëlle (Sorbonne Université, Inserm, CNRS)
Berzero, Giulia (Neuroncology Unit, Italy)
Izquierdo, Cristina (Universitat Autònoma de Barcelona. Departament de Medicina)
Giry, Marine (Sorbonne Université, Inserm)
Benazra, Marion (Sorbonne Université, Inserm)
Delattre, Jean-Yves (Sorbonne Université, Inserm)
Psimaras, Dimitri (Sorbonne Université, Inserm)
Alentorn, Agusti (Sorbonne Université, Inserm)
| Data: |
2020 |
| Resum: |
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. |
| Nota: |
Altres ajuts: French National Research Agency (ANR-18-RHUS-0012) |
| Drets: |
Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.  |
| Llengua: |
Anglès |
| Document: |
Article ; recerca ; Versió publicada |
| Matèria: |
Glutamic acid decarboxylase ;
GAD65 autoimmunity ;
Neuronal antibodies ;
Paraneoplastic neurological syndromes ;
Limbic encephalitis ;
Autoimmune epilepsy ;
Cerebellar ataxia ;
Stiff-person syndrome |
| Publicat a: |
International journal of molecular sciences, Vol. 21 (may 2020) , ISSN 1422-0067 |
DOI: 10.3390/ijms21103701
PMID: 32456344
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