Cerebellar ataxia with bilateral vestibulopathy: description of a syndrome and its characteristic clinical sign.

@article{Migliaccio2004CerebellarAW,
  title={Cerebellar ataxia with bilateral vestibulopathy: description of a syndrome and its characteristic clinical sign.},
  author={Americo A. Migliaccio and G. Michael Halmagyi and Leigh A. McGarvie and Phillip Cremer},
  journal={Brain : a journal of neurology},
  year={2004},
  volume={127 Pt 2},
  pages={280-93}
}
We report four patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV) and, using search coil oculography, we validate its characteristic clinical sign, namely impairment of the visually enhanced vestibulo-ocular reflex (VVOR) or doll's head reflex. In our four patients, CABV began in the sixth decade of life; they are still ambulant and self-caring 8-20 years after onset. The cause of CABV in our four patients is unknown. None has a family history of cerebellar or… CONTINUE READING
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