Tilikete Caroline, Vighetto Alain, Trouillas Paul, Honnorat Jérome
Neuro-Ophthalmology Unit, Hôpital Neurologique, Hospices civils de Lyon, Bron, France.
Arch Neurol. 2005 Aug;62(8):1300-3. doi: 10.1001/archneur.62.8.1300.
Autoantibodies directed against glutamic acid decarboxylase (GAD-Ab) have recently been described in a few patients with progressive cerebellar ataxia, suggesting an autoimmune physiopathologic mechanism.
To determine the exact role of GAD-Ab and gamma-aminobutyric acid (GABA)-ergic neurotransmission in the pathogenesis of cerebellar ataxia.
Case report.
University neurological hospital.
We report the case of a patient with subacute cerebellar ataxia associated with GAD-Ab showing periodic alternating nystagmus (PAN).
Baclofen, a GABAergic medication, was given to the patient.
Eye movement recording of spontaneous nystagmus and postrotatory vestibular responses.
Baclofen was effective in suppressing PAN and improving postrotatory vestibular responses but not for improving cerebellar ataxia.
The presence of PAN and the response to baclofen provide a unique opportunity to suggest a direct role of GAD-Ab in cerebellar dysfunction in this patient.
最近在少数进行性小脑共济失调患者中发现了针对谷氨酸脱羧酶的自身抗体（GAD - Ab），提示存在自身免疫性病理生理机制。
确定GAD - Ab和γ-氨基丁酸（GABA）能神经传递在小脑共济失调发病机制中的确切作用。
病例报告。
大学神经科医院。
我们报告了1例伴有GAD - Ab的亚急性小脑共济失调患者，该患者表现为周期性交替性眼球震颤（PAN）。
给予患者巴氯芬，一种GABA能药物。
自发性眼球震颤的眼动记录和旋转后前庭反应。
巴氯芬可有效抑制PAN并改善旋转后前庭反应，但对改善小脑共济失调无效。
PAN的存在以及对巴氯芬的反应为提示GAD - Ab在该患者小脑功能障碍中起直接作用提供了独特的机会。
