Cerebellar ataxia associated to anti-glutamic acid decarboxylase autoantibody (anti-GAD): partial improvement with intravenous immunoglobulin therapy

Pedroso, José Luiz; Braga‐Neto, Pedro; Dutra, Lívia Almeida; Barsottini, Orlando Graziani Póvoas

doi:10.1590/s0004-282x2011000700030

Cited by 23 publications

(16 citation statements)

References 5 publications

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“…All the patients reported here had global cerebellar ataxia; however, in Patient 2 the cerebellar ataxia was predominantly axial. Gait ataxia appears to be a severe and common problem in this condition 1,5,6,13 . Few reports have been published on patients with CA-GAD-ab presenting with neuro-ophthalmic abnormalities 14,15,16,17,18,19 .…”

Section: Discussionmentioning

confidence: 99%

“…the spectrum of ocular manifestations in patients with CA-GAD-ab may be broader 13,14,15,16,17 . Recent studies have shown that spontaneous downbeat nystagmus can represent an important clue for a diagnosis of CA-GAD-ab 18 .…”

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confidence: 99%

“…The majority of cases of CA-GAD-ab reported in the literature involved high levels of GAD-ab 1,6,25,26 but, even if the titers are low, the condition may be associated with an autoimmune mechanism 13,27 . A diagnosis of CA-GAD-ab is supported by the positivity for GAD-ab.…”

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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%

“…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 .…”

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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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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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confidence: 99%

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confidence: 99%

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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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Cerebellar Ataxia and Glutamic Acid Decarboxylase Antibodies

et al. 2014

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Cerebellar disease associated with anti-glutamic acid decarboxylase antibodies: review

Baizabal‐Carvallo

Alonso-Juárez

2017

J Neural Transm

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Several neurological syndromes have been recognized associated to GAD antibodies. Among those disorders, cerebellar ataxia (CA) is one of the most common, along with stiff-person syndrome. Patients with GAD associated CA present with a progressive pancerebellar syndrome, with a subacute or chronic evolution, along with other neurological manifestations such as stiffness, oculomotor dysfunction, epilepsy, and cognitive dysfunction. These symptoms may be preceded by the so-called "brainstem attacks", where manifestations consistent with transient dysfunction of the brainstem may be observed. These patients frequently have extra-neurologic autoimmune manifestations such as diabetes mellitus type 1, polyendocrine autoimmune syndrome, pernicious anemia, vitiligo, etc. A proportion of patients may present with an underlying neoplasia, but the course is less aggressive than in those patients with classical paraneoplastic CA with onconeural antibodies. The diagnosis is based on the present of high serum and CSF titers of GAD antibodies, with intrathecal production of such antibodies. Treatment is aimed to decrease the immunological response with intravenous immunoglobulin, steroids, rituximab and oral immunosuppressive drugs. A subacute presentation and rapid initiation of immunotherapy seem to be the predictors of a favorable clinical response.

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Cerebellar ataxia associated to anti-glutamic acid decarboxylase autoantibody (anti-GAD): partial improvement with intravenous immunoglobulin therapy

Cited by 23 publications

References 5 publications

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

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

Cerebellar Ataxia and Glutamic Acid Decarboxylase Antibodies

Cerebellar disease associated with anti-glutamic acid decarboxylase antibodies: review

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