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Effect of steroid treatment in cerebellar ataxia associated with anti-glutamic acid decarboxylase antibodies
  1. R Virgilio1,2,
  2. S Corti1,2,
  3. P Agazzi3,
  4. D Santoro1,2,
  5. S Lanfranconi1,
  6. L Candelise1,
  7. N Bresolin1,2,
  8. G P Comi1,2,
  9. A Bersano1
  1. 1
    Dino Ferrari Centre, Department of Neurological Sciences, University of Milan, IRCCS Foundation Ospedale Maggiore Policlinico Mangiagalli and Regina Elena, Milan, Italy
  2. 2
    Centre of Excellence on Neurodegenerative Diseases, University of Milan, Milan, Italy
  3. 3
    Civic Hospital of Lugano, Lugano, Switzerland
  1. Dr A Bersano, Department of Neurological Sciences, University of Milan, Fondazione Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Via F.Sforza 35, 20122 Milan, Italy; anna.bersano{at}unimi.it

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Glutamic acid decarboxylase (GAD) catalyses the transformation of glutamate into γ-aminobutyric acid (GABA). Anti-GAD autoantibodies (GAD-Ab) have been associated with insulin-dependent diabetes mellitus (IDDM) and with other possibly immunomediated syndromes affecting the CNS including stiff-man syndrome (SPS),1 progressive encephalomyelitis with rigidity and myoclonus (PERM) and cerebellar ataxia,1 2 mostly in association with IDDM. These findings support the autoimmune origin of the neurological symptoms, possibly induced by an anti-GAD-Ab-mediated neuronal dysfunction. Manto et al3 induced cerebellar and spinal cord symptoms in rats, by intrathecal injection of IgG from anti-GAD-Ab positive patients affected by SPS or cerebellar ataxia, suggesting a pathogenetic mechanisms involving a change of balance between glutamate and GABA, causing glutamate excitotoxicity.

High-dose intravenous immunoglobulins and plasmapheresis have been suggested as possible therapies, but cerebellar symptoms have been rarely found to improve. Recently Lauria et al4 induced clinical improvement in a patient with anti-GAD-Ab cerebellar ataxia through high doses of methylprednisolone, suggesting that it should be considered as first-line therapy in these patients. We describe a 76-year-old man developing an anti-GAD associated subacute cerebellar syndrome that improved dramatically after steroid treatment.

CASE REPORT

A 76-year-old man was admitted to our clinic in January 2007 for the development, since June 2006, of a subacute progressive gait instability and loss of fluency of movement execution. Within 3 months, …

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