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Related Experiment Video

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Induction of Paralysis and Visual System Injury in Mice by T Cells Specific for Neuromyelitis Optica Autoantigen Aquaporin-4

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Immune-mediated acquired ataxias.

Marios Hadjivassiliou¹

¹Department of Neurology, The Royal Hallamshire Hospital, Sheffield, UK. m.hadjivassiliou@sheffield.ac.uk

Handbook of Clinical Neurology

|August 11, 2011

Summary

The cerebellum is a common target in autoimmune diseases, particularly affecting Purkinje cells. This review details immune-mediated ataxias like gluten ataxia, focusing on potentially treatable conditions.

Area of Science:

Neuroimmunology
Cerebellar Disorders
Autoimmune Diseases

Background:

The cerebellum, especially Purkinje cells, is frequently targeted in systemic autoimmune diseases.
This immunological susceptibility may stem from the cerebellum's size, conservation, and Purkinke cells' antigenic properties.
Immune-mediated ataxias encompass conditions like paraneoplastic cerebellar degeneration and post-infective cerebellitis.

Purpose of the Study:

To provide a detailed review of the epidemiology, clinical characteristics, pathophysiology, and treatment of specific immune-mediated ataxias.
To emphasize gluten ataxia as a common and treatable form of autoimmune cerebellar ataxia.
To introduce the concept of primary autoimmune cerebellar ataxia as an organ-specific autoimmune disease.

Main Methods:

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Literature review and synthesis of existing research on immune-mediated cerebellar ataxias.
Detailed examination of gluten ataxia and anti-GAD antibody-associated ataxia.
Discussion of speculative pathophysiological mechanisms leading to cerebellar damage.

Main Results:

Gluten ataxia is highlighted as a prevalent and treatable immune-mediated cerebellar ataxia.
The chapter introduces primary autoimmune cerebellar ataxia as a distinct entity.
Pathophysiological pathways for cerebellar damage and ataxia are explored, though remain speculative.

Conclusions:

Immune-mediated cerebellar ataxias, particularly gluten ataxia, represent significant neurological challenges.
Further research is needed to elucidate the precise pathophysiology of these conditions.
Recognition of primary autoimmune cerebellar ataxia expands the understanding of organ-specific autoimmune diseases.