Effect of intravenous immunoglobulin on cerebellar ataxia and neuropathic pain associated with celiac disease

Eur J Neurol. 2008 Dec;15(12):1300-3. doi: 10.1111/j.1468-1331.2008.02305.x.


Background: Cerebellar syndrome and small fiber neuropathy may complicate celiac disease (CD) and may be resistant to a strict gluten-free diet.

Methods: Case series.

Results: We report three patients with biopsy-proven CD who developed cerebellar ataxia and neuropathic pain despite strict adherence to a gluten-free diet. A small fiber neuropathy was suggested by skin biopsy findings in two patients. All patients' symptoms, including small fiber neuropathy symptoms, responded to treatment with intravenous immunoglobulin (IVIG). Discontinuation of IVIG in two patients resulted in worsened ataxia that reversed after resumption of IVIG.

Conclusion: Intravenous immunoglobulin may be effective in treating cerebellar ataxia and small fiber neuropathy associated with CD, suggesting an immune pathogenesis. Further prospective, controlled studies are necessary to determine the long-term response to IVIG or other immunomodulation therapy.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Afferent Pathways / drug effects
  • Afferent Pathways / immunology
  • Afferent Pathways / physiopathology
  • Celiac Disease / complications*
  • Cerebellar Ataxia / drug therapy*
  • Cerebellar Ataxia / immunology*
  • Cerebellar Ataxia / physiopathology
  • Cerebellum / drug effects
  • Cerebellum / immunology
  • Cerebellum / physiopathology
  • Disease Progression
  • Dose-Response Relationship, Drug
  • Female
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage*
  • Nerve Fibers, Unmyelinated / drug effects
  • Nerve Fibers, Unmyelinated / immunology
  • Nerve Fibers, Unmyelinated / pathology
  • Nociceptors / drug effects
  • Nociceptors / immunology
  • Nociceptors / pathology
  • Peripheral Nerves / drug effects
  • Peripheral Nerves / immunology
  • Peripheral Nerves / pathology
  • Peripheral Nervous System Diseases / drug therapy*
  • Peripheral Nervous System Diseases / immunology*
  • Peripheral Nervous System Diseases / physiopathology
  • Spinocerebellar Tracts / drug effects
  • Spinocerebellar Tracts / immunology
  • Spinocerebellar Tracts / physiopathology
  • Treatment Outcome


  • Immunoglobulins, Intravenous