A Breakdown of Immune Tolerance in the Cerebellum

Cerebellar dysfunction can be associated with ataxia, dysarthria, dysmetria, nystagmus and cognitive deficits. While cerebellar dysfunction can be caused by vascular, traumatic, metabolic, genetic, inflammatory, infectious, and neoplastic events, the cerebellum is also a frequent target of autoimmun...

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Bibliographic Details
Published in:Brain sciences 2022-02, Vol.12 (3), p.328
Main Authors: Hampe, Christiane S, Mitoma, Hiroshi
Format: Article
Language:English
Subjects:
Antigens
Ataxia
Autoantigens
Autoimmune diseases
Autoimmunity
Blood-brain barrier
blood–CSF barrier
Cerebellum
Cerebrospinal fluid
Chemokines
Cognitive ability
Cytokines
Dendritic cells
Endothelium
immune surveillance
Immune system
Immunological tolerance
Immunosurveillance
Infections
Infectious diseases
Inflammation
Ligands
Lymphocytes
Membrane permeability
Meninges
Mimicry
Nervous system
Nystagmus
Permeability
Physiology
Proteins
Purkinje cells
Rabies
Review
Spinal cord
Traumatic brain injury
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Summary:Cerebellar dysfunction can be associated with ataxia, dysarthria, dysmetria, nystagmus and cognitive deficits. While cerebellar dysfunction can be caused by vascular, traumatic, metabolic, genetic, inflammatory, infectious, and neoplastic events, the cerebellum is also a frequent target of autoimmune attacks. The underlying cause for this vulnerability is unclear, but it may be a result of region-specific differences in blood-brain barrier permeability, the high concentration of neurons in the cerebellum and the presence of autoantigens on Purkinje cells. An autoimmune response targeting the cerebellum-or any structure in the CNS-is typically accompanied by an influx of peripheral immune cells to the brain. Under healthy conditions, the brain is protected from the periphery by the blood-brain barrier, blood-CSF barrier, and blood-leptomeningeal barrier. Entry of immune cells to the brain for immune surveillance occurs only at the blood-CSF barrier and is strictly controlled. A breakdown in the barrier permeability allows peripheral immune cells uncontrolled access to the CNS. Often-particularly in infectious diseases-the autoimmune response develops because of molecular mimicry between the trigger and a host protein. In this review, we discuss the immune surveillance of the CNS in health and disease and also discuss specific examples of autoimmunity affecting the cerebellum.
ISSN:2076-3425
2076-3425
DOI:10.3390/brainsci12030328