Selective loss of Purkinje cells in a patient with anti-glutamic acid decarboxylase antibody-associated cerebellar ataxia

Ishida, Kazuyuki; Mitoma, Hiroshi; Wada, Yoshiaki; Oka, Teruaki; Shibahara, Junji; Saito, Yuko; Murayama, Shigeo; Mizusawa, Hidehiro

doi:10.1136/jnnp.2006.091116

Cited by 69 publications

(46 citation statements)

References 14 publications

(8 reference statements)

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“…Thus, the neuroinflammation-induced chain reactions accelerate the imbalance, leading to profound excitotoxicity. In agreement with this notion, the cerebellar neurons are completely lost in patients with advanced stage CAs [90].…”

Section: Association Of Gad65 Dysfunction With Neurological Disorderssupporting

confidence: 74%

GABA and Glutamate: Their Transmitter Role in the CNS and Pancreatic Islets

Hampe¹,

Mitoma²,

Manto³

2018

GABA and Glutamate - New Developments in Neurotransmission Research

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Glutamate and gamma-aminobutyric acid (GABA) are the major neurotransmitters in the mammalian brain. Inhibitory GABA and excitatory glutamate work together to control many processes, including the brain's overall level of excitation. The contributions of GABA and glutamate in extra-neuronal signaling are by far less widely recognized. In this chapter, we first discuss the role of both neurotransmitters during development, emphasizing the importance of the shift from excitatory to inhibitory GABAergic neurotransmission. The second part summarizes the biosynthesis and role of GABA and glutamate in neurotransmission in the mature brain, and major neurological disorders associated with glutamate and GABA receptors and GABA release mechanisms. The final part focuses on extra-neuronal glutamatergic and GABAergic signaling in pancreatic islets of Langerhans, and possible associations with type 1 diabetes mellitus.

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Section: Association Of Gad65 Dysfunction With Neurological Disorderssupporting

confidence: 74%

GABA and Glutamate: Their Transmitter Role in the CNS and Pancreatic Islets

Hampe¹,

Mitoma²,

Manto³

2018

GABA and Glutamate - New Developments in Neurotransmission Research

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“…In regards to autoimmune cerebellar ataxia, GADantibody-positive cerebellar ataxia (13,14), gluten ataxia (15) and thyroid-antibody-positive cerebellar ataxia (16,17) …”

Section: In the Present Study Ivig Therapy Was Administered In Sevenmentioning

confidence: 99%

Intravenous Immunoglobulin Therapy for Autoantibody-Positive Cerebellar Ataxia

et al. 2009

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“…The response to treatment is variable, with complete remission being rare. However, there are a few reports that show favorable response 13,30 . No correlation has been found between serum GAD-ab titers and the severity of the disease, its phenotype or response to treatment 5,7,25 .…”

mentioning

confidence: 99%

“…The reason behind the susceptibility of the cerebellum to this form of immune-mediated disease is not fully understood, but is probably related to the distribution of GAD in Purkinje cells and a direct excitotoxic effect of GAD-ab 6 . Some autopsy studies have revealed a selective loss of Purkinje cells 30 . The potential pathogenic role of GAD-ab in neurological disorders remains to be clarified; however, plausible hypotheses include the inhibition of GABA synthesis and interference with exocytosis 25 .…”

mentioning

confidence: 99%

“…The patients in this study had high titers, reinforcing the immune-mediated mechanism 1,6 . Despite the absence of randomized controlled studies, immunotherapy regimens such as glucocorticoid, intravenous immunoglobulin, plasma exchange, and immunosuppressive agents in varying combinations are the mainstay of the treatment 7,13,25,28,29,30 . The response to treatment is variable, with complete remission being rare.…”

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confidence: 99%

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Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies

Aguiar

Fragoso

Albuquerque

et al. 2017

Arq. Neuro-Psiquiatr.

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The enzyme glutamic acid decarboxylase (GAD), present in GABAergic neurons and in pancreatic beta cells, catalyzes the conversion of gamma-aminobutyric acid (GABA). The cerebellum is highly susceptible to immune-mediated mechanisms, with the potentially treatable autoimmune cerebellar ataxia associated with the GAD antibody (CA-GAD-ab) being a rare, albeit increasingly detected condition. Few cases of CA-GAD-ab have been described. Methods This retrospective and descriptive study evaluated the clinical characteristics and outcomes of patients with CA-GAD-ab. Result Three patients with cerebellar ataxia, high GAD-ab titers and autoimmune endocrine disease were identified. Patients 1 and 2 had classic stiff person syndrome and insidious-onset cerebellar ataxia, while Patient 3 had pure cerebellar ataxia with subacute onset. Patients received intravenous immunoglobulin therapy with no response in Patients 1 and 3 and partial recovery in Patient 2. Conclusion CA-GAD-ab is rare and its clinical presentation may hamper diagnosis. Clinicians should be able to recognize this potentially treatable autoimmune cerebellar ataxia.

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Selective loss of Purkinje cells in a patient with anti-glutamic acid decarboxylase antibody-associated cerebellar ataxia

Cited by 69 publications

References 14 publications

GABA and Glutamate: Their Transmitter Role in the CNS and Pancreatic Islets

GABA and Glutamate: Their Transmitter Role in the CNS and Pancreatic Islets

Intravenous Immunoglobulin Therapy for Autoantibody-Positive Cerebellar Ataxia

Clinical characteristics of patients with cerebellar ataxia associated with anti-GAD antibodies

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