Title: Autoantibodies to metabotropic glutamate receptors ...



Appendix e-1

Case report (cerebellar degeneration and antibodies to mGluR1)

In 2007 a 69-year-old man noted unsteady gait and speech problems. Over the next several months, he developed cerebellar ataxia of the upper and lower extremities, with rapidly progressive decline in his speech and ability to walk. He lost the ability to sit straight or walk without assistance. In addition, he had nystagmus and difficulty directing and maintaining fixation of gaze. He was treated with intravenous methylprednisolone (1 g daily for 3 days) every 4 weeks for 5 cycles, then every 12 weeks for 3 more cycles. There was a transient improvement of symptoms (score in Scale for the Assessment and Rating of Ataxiae3 improved from 19 to 14), but subsequently symptoms worsened and stabilized at score of 17.5. Since then, symptoms have not changed substantially despite monthly treatments with intravenous methylprednisolone. At the last follow-up in January 2011, he had severe dysarthria and disabling limb, truncal, and ocular ataxia.

Brain MRI at symptom onset showed signs compatible with small vessel ischemic disease. Follow-up MRI 1 year later showed cerebellar atrophy. Initial CSF studies demonstrated 8 white blood cells /µl (predominantly lymphocytes; normal ................
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