Cortical dysfunction in cerebellar ataxia with antibodies to glutamic acid decarboxylase
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Antibodies against glutamic acid decarboxylase (GAD) have been identified in a number of neurological disorders, including cerebellar ataxia  and stiff-person syndrome (SPS) . Recent studies have suggested a role for glutamic acid decarboxylase antibodies (GAD-Ab) in producing cortical dysfunction in SPS , but the pathogenesis of other neurological disorders with GAD-Ab remains to be fully elucidated. In order to further define the pathogenic role of GAD-Ab in cerebellar ataxia, the cortical modulating effects of GAD-Ab were evaluated in a patient with cerebellar ataxia with GAD-Ab.
A 52-year-old female, with a background of mature-onset type 1 diabetes mellitus, presented with 12 months of progressive cerebellar dysfunction characterised by gait and truncal ataxia, dysarthria, complex nystagmus, and left upper limb dysmetria and intention tremor. Cerebellar dysfunction was quantified using the Brief Ataxia Rating Scale, with a score of 14 out of a maximum of 30 ...
KeywordsBaclofen Cerebellar Ataxia Glutamic Acid Decarboxylase Cortical Excitability Rest Motor Threshold
Conflicts of interest
Dr. Neil Simon received funding from the National Health and Medical Research Council of Australia and the Motor Neurone Disease Research Institute of Australia (grant #1039520). The study was supported by a National Health and Medical Research Council Program Grant (#1037746). The authors declare that they have no conflicts of interest.
The study protocol was approved by the South-Eastern Sydney and Illawarra Area Health Service Human Research Ethics Committee. Written consent was obtained from each subject.
- 1.Honnorat J, Saiz A, Giometto B, Vincent A, Brieva L, de Andres C, Maestre J, Fabien N, Vighetto A, Casamitjana R, Thivolet C, Tavolato B, Antoine J, Trouillas P, Graus F (2001) Cerebellar ataxia with anti-glutamic acid decarboxylase antibodies: study of 14 patients. Arch Neurol 58:225–230CrossRefPubMedGoogle Scholar
- 7.Wessel K, Tegenthoff M, Vorgerd M, Otto V, Nitschke MF, Malin JP (1996) Enhancement of inhibitory mechanisms in the motor cortex of patients with cerebellar degeneration: a study with transcranial magnetic brain stimulation. Electroencephalogr Clin Neurophysiol 101:273–280CrossRefPubMedGoogle Scholar