Ataxia associated with anti-glutamic acid decarboxylase antibodies

Anti-glutamic acid decarboxylase (GAD) antibody-associated ataxia is a rarely diagnosed but potentially curable disease associated with autoimmune damage to and death of Purkinje cells in the cerebellar cortex. In Russia, the authors have provided for the first time descriptions of three own observa...

Full description

Bibliographic Details
Main Authors: E. P. Nuzhnyi, M. Yu. Krasnov, D. R. Akhmadullina, A. A. Abramova, E. Yu. Fedotova, S. N. Illarioshkin
Format: Article
Language:Russian
Published: IMA-PRESS LLC 2020-10-01
Series:Неврология, нейропсихиатрия, психосоматика
Subjects:
Online Access:https://nnp.ima-press.net/nnp/article/view/1449
_version_ 1797876389741854720
author E. P. Nuzhnyi
M. Yu. Krasnov
D. R. Akhmadullina
A. A. Abramova
E. Yu. Fedotova
S. N. Illarioshkin
author_facet E. P. Nuzhnyi
M. Yu. Krasnov
D. R. Akhmadullina
A. A. Abramova
E. Yu. Fedotova
S. N. Illarioshkin
author_sort E. P. Nuzhnyi
collection DOAJ
description Anti-glutamic acid decarboxylase (GAD) antibody-associated ataxia is a rarely diagnosed but potentially curable disease associated with autoimmune damage to and death of Purkinje cells in the cerebellar cortex. In Russia, the authors have provided for the first time descriptions of three own observations of this disease, which had a number of clinical features, such as slow progression, mild ataxia, stroke-like episodes with stem symptoms, concomitant gluten sensitivity, onset of ataxia after hepatitis C with cerebellar hemiataxia and hemiatrophy. In the all patients, the diagnosis was verified based on the determination of high anti-GAD antibody titers in serum and cerebrospinal fluid. All the patients lacked intrathecal synthesis of oligoclonal antibodies; protein levels and cytosis were normal. Pulse therapy with methylprednisolone at a total dose of 3–5 g led to a slight reduction in ataxia in one case (a female patient with subacute onset of the disease); the treatment was ineffective in two other cases (patients with a primary chronic course). The paper analyzes the literature covering the pathogenesis and clinical presentations of this type of ataxia, and difficulties in its diagnosis and treatment.
first_indexed 2024-04-10T02:01:21Z
format Article
id doaj.art-d4fc00214f164fa895dfc4c649a5d1f8
institution Directory Open Access Journal
issn 2074-2711
2310-1342
language Russian
last_indexed 2024-04-10T02:01:21Z
publishDate 2020-10-01
publisher IMA-PRESS LLC
record_format Article
series Неврология, нейропсихиатрия, психосоматика
spelling doaj.art-d4fc00214f164fa895dfc4c649a5d1f82023-03-13T08:42:20ZrusIMA-PRESS LLCНеврология, нейропсихиатрия, психосоматика2074-27112310-13422020-10-01125667010.14412/2074-2711-2020-5-66-701050Ataxia associated with anti-glutamic acid decarboxylase antibodiesE. P. Nuzhnyi0M. Yu. Krasnov1D. R. Akhmadullina2A. A. Abramova3E. Yu. Fedotova4S. N. Illarioshkin5Research Center of NeurologyResearch Center of NeurologyResearch Center of NeurologyResearch Center of NeurologyResearch Center of NeurologyResearch Center of NeurologyAnti-glutamic acid decarboxylase (GAD) antibody-associated ataxia is a rarely diagnosed but potentially curable disease associated with autoimmune damage to and death of Purkinje cells in the cerebellar cortex. In Russia, the authors have provided for the first time descriptions of three own observations of this disease, which had a number of clinical features, such as slow progression, mild ataxia, stroke-like episodes with stem symptoms, concomitant gluten sensitivity, onset of ataxia after hepatitis C with cerebellar hemiataxia and hemiatrophy. In the all patients, the diagnosis was verified based on the determination of high anti-GAD antibody titers in serum and cerebrospinal fluid. All the patients lacked intrathecal synthesis of oligoclonal antibodies; protein levels and cytosis were normal. Pulse therapy with methylprednisolone at a total dose of 3–5 g led to a slight reduction in ataxia in one case (a female patient with subacute onset of the disease); the treatment was ineffective in two other cases (patients with a primary chronic course). The paper analyzes the literature covering the pathogenesis and clinical presentations of this type of ataxia, and difficulties in its diagnosis and treatment.https://nnp.ima-press.net/nnp/article/view/1449ataxiaglutamic acid decarboxylaseantibodiesclinical presentationdiagnosis
spellingShingle E. P. Nuzhnyi
M. Yu. Krasnov
D. R. Akhmadullina
A. A. Abramova
E. Yu. Fedotova
S. N. Illarioshkin
Ataxia associated with anti-glutamic acid decarboxylase antibodies
Неврология, нейропсихиатрия, психосоматика
ataxia
glutamic acid decarboxylase
antibodies
clinical presentation
diagnosis
title Ataxia associated with anti-glutamic acid decarboxylase antibodies
title_full Ataxia associated with anti-glutamic acid decarboxylase antibodies
title_fullStr Ataxia associated with anti-glutamic acid decarboxylase antibodies
title_full_unstemmed Ataxia associated with anti-glutamic acid decarboxylase antibodies
title_short Ataxia associated with anti-glutamic acid decarboxylase antibodies
title_sort ataxia associated with anti glutamic acid decarboxylase antibodies
topic ataxia
glutamic acid decarboxylase
antibodies
clinical presentation
diagnosis
url https://nnp.ima-press.net/nnp/article/view/1449
work_keys_str_mv AT epnuzhnyi ataxiaassociatedwithantiglutamicaciddecarboxylaseantibodies
AT myukrasnov ataxiaassociatedwithantiglutamicaciddecarboxylaseantibodies
AT drakhmadullina ataxiaassociatedwithantiglutamicaciddecarboxylaseantibodies
AT aaabramova ataxiaassociatedwithantiglutamicaciddecarboxylaseantibodies
AT eyufedotova ataxiaassociatedwithantiglutamicaciddecarboxylaseantibodies
AT snillarioshkin ataxiaassociatedwithantiglutamicaciddecarboxylaseantibodies