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Cerebellar Ataxia With Anti–Glutamic Acid Decarboxylase Antibodies
Study of 14 Patients
Jérôme Honnorat, MD, PhD;
Albert Saiz, MD;
Bruno Giometto, MD;
Angela Vincent, FRCPath;
Luis Brieva, MD;
Clara de Andres, MD;
José Maestre, MD;
Nicole Fabien, MD;
Alain Vighetto, MD;
Roser Casamitjana, MD;
Charles Thivolet, MD;
Bruno Tavolato, MD;
Jean Christophe Antoine, MD;
Paul Trouillas, MD;
Francesc Graus, MD
Arch Neurol. 2001;58:225-230.
Background Antibodies to glutamic acid decarboxylase (GAD-Ab) are described in
patients with insulin-dependent (type 1) diabetes mellitus (IDDM), in stiff-man
syndrome, and, recently, in a few patients with cerebellar ataxia.
Objectives To show a link between GAD-Ab and some patients with cerebellar ataxia
and to clarify their clinical and immunologic profiles.
Methods Serum samples were selected from 9000 samples of 4 laboratories. The
selection criterion was an immunohistochemical pattern compatible with GAD-Ab
that was confirmed by radioimmunoassay. We identified 22 patients with stiff-man
syndrome and 14 with cerebellar ataxia and GAD-Ab.
Results Thirteen of the 14 patients with cerebellar ataxia and GAD-Ab were women,
and 11 had late-onset IDDM. Patients did not have clinical or radiologic evidence
of brainstem involvement. Ten patients had oligoclonal IgG bands in the cerebrospinal
fluid, and intrathecal GAD-Ab synthesis was observed in 5 of the 6 patients
studied. The level of GAD-Ab of these patients was similar to those with stiff-man
syndrome and significantly higher than those with IDDM or with polyendocrine
autoimmunity (P<.001). However, the GAD-Ab levels
of 6 of the 9 patients with polyendocrine autoimmunity overlapped with those
of patients with cerebellar ataxia.
Conclusions These results suggest a link between high level of GAD-Ab and some cases
of cerebellar ataxia, particularly women with IDDM. If high serum levels of
GAD-Ab are detected, the cerebrospinal fluid should be evaluated for the presence
of oligoclonal IgG bands and intrathecal synthesis of GAD-Ab to further prove
an autoimmune origin of the syndrome.
From the Ataxia Research Center, Neurology B (Drs Honnorat and Trouillas)
and Department of Neurology C (Dr Vighetto), Hôpital Neurologique, Lyon,
France; Institut d'Investigació Biomèdica August Pi i Sunyer
(Drs Saiz, Casamitjana, and Graus) and Department of Neurology and Laboratorio
de Hormonal (Dr Casamitjana), Hospital Clinic, University of Barcelona, Barcelona,
Spain; Department of Neurology and Psychiatry (Second Neurologic Clinic),
University of Padua, Padua, Italy (Drs Giometto and Tavolato); Neurosciences
Group, Institute of Molecular Medicine, John Radcliffe Hospital, Oxford, England
(Dr Vincent); Department of Neurology, Hospital General Universitari, Vall
d'Hebrón, Barcelona (Dr Brieva); Department of Neurology, Hospital
Gregorio Marañón, Madrid, Spain (Dr de Andres); Department of
Neurology, Hospital Virgen de las Nieves, Granada, Spain (Dr Maestre); Department
of Immunology, Centre Hospitalier Lyon-Sud, Pierre-Benite, France (Dr Fabien);
Institut National de la Santé et de la Récherche Médicale
U 449, Faculté de Médecine Laennec, Lyon (Dr Thivolet); and
Department of Neurology, Hôpital Bellevue, St Etienne, France (Dr Antoine).
Corresponding author and reprints: Francesc Graus, MD, Service of
Neurology, Hospital Clínic, Villarroel 170, 08036 Barcelona, Spain
(e-mail: graus{at}medicina.ub.es).
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