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Review

Neurological Syndromes Associated with Anti-GAD Antibodies

1
AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Service de Neurologie 2-Mazarin, 75013 Paris, France
2
Sorbonne Université, Inserm, CNRS, UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, 75013 Paris, France
3
Neuroncology Unit, IRCCS Mondino Foundation, 27100 Pavia, Italy
4
Department of Neuroscience, Hospital Universitari Germans Trias i Pujol, Universitat Autònoma de Barcelona, 08916 Badalona, Spain
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2020, 21(10), 3701; https://doi.org/10.3390/ijms21103701
Received: 27 April 2020 / Revised: 19 May 2020 / Accepted: 21 May 2020 / Published: 24 May 2020
(This article belongs to the Special Issue Molecular Biomarkers in Multiple Sclerosis and Its Related Disorders)
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. View Full-Text
Keywords: glutamic acid decarboxylase; GAD65 autoimmunity; neuronal antibodies; paraneoplastic neurological syndromes; limbic encephalitis; autoimmune epilepsy; cerebellar ataxia; stiff-person syndrome glutamic acid decarboxylase; GAD65 autoimmunity; neuronal antibodies; paraneoplastic neurological syndromes; limbic encephalitis; autoimmune epilepsy; cerebellar ataxia; stiff-person syndrome
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MDPI and ACS Style

Dade, M.; Berzero, G.; Izquierdo, C.; Giry, M.; Benazra, M.; Delattre, J.-Y.; Psimaras, D.; Alentorn, A. Neurological Syndromes Associated with Anti-GAD Antibodies. Int. J. Mol. Sci. 2020, 21, 3701. https://doi.org/10.3390/ijms21103701

AMA Style

Dade M, Berzero G, Izquierdo C, Giry M, Benazra M, Delattre J-Y, Psimaras D, Alentorn A. Neurological Syndromes Associated with Anti-GAD Antibodies. International Journal of Molecular Sciences. 2020; 21(10):3701. https://doi.org/10.3390/ijms21103701

Chicago/Turabian Style

Dade, Maëlle, Giulia Berzero, Cristina Izquierdo, Marine Giry, Marion Benazra, Jean-Yves Delattre, Dimitri Psimaras, and Agusti Alentorn. 2020. "Neurological Syndromes Associated with Anti-GAD Antibodies" International Journal of Molecular Sciences 21, no. 10: 3701. https://doi.org/10.3390/ijms21103701

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