Neuroimmunology: An Expanding Frontier in Autoimmunity
نویسنده
چکیده
Anti-neuronal autoimmune encephalitis (AIE) comprises a recently characterized group of immune-mediated disorders that result in limbic, multifocal, or diffuse encephalitis due to direct interaction of autoantibodies with neuronal surface or synaptic proteins. The pathological effects of the autoantibodies vary according to the target antigen but when they are removed, neuronal dysfunction is commonly reversed. Ongoing research on AIE constantly increases the number of novel autoantibodies and expands the spectrum of neurological syndromes that are important in the differential diagnosis of psychiatric illness, dementia, or viral encephalitis. This review summarizes recent advances in AIE, focusing on pathogenetic mechanisms and novel associations with other CNS disorders such as neurodegeneration, relapsing symptoms post-herpes simplex virus encephalitis, and demyelinating diseases. In addition, an algorithmic approach to detect and characterize neuronal cell surface autoantibodies is proposed.
منابع مشابه
Expanding spectrum of neurologic manifestations in patients with NLRP3 low-penetrance mutations
OBJECTIVE To evaluate the frequency of the cryoporin/NLRP3 low-penetrance mutations V198M and Q703K in patients who reported at least 2 symptoms compatible with cryopyrin-associated periodic syndromes (CAPS) and to characterize the phenotype in mutation-positive patients. METHODS The frequency of the V198M and Q703K mutations was investigated in a selected cohort of 108 patients from our neur...
متن کاملA comparison of tissue-based and recombinant protein–based assays for detecting PCA-Tr/DNER-IgG
Purkinje cell cytoplasmic antibody, type Tr (PCATr) is typically encountered in patients with Hodgkin lymphoma–related paraneoplastic cerebellar ataxia. Immunohistochemical techniques, including indirect immunofluorescence assays (IFAs), employing rodent brain as substrate, reveal a specific immunoglobulin G (IgG) binding pattern on cerebellar Purkinje cell cytoplasm and molecular layer dendrit...
متن کاملAutoimmune maintenance and neuroprotection of the central nervous system.
The genesis of immune privilege high in the evolutionary tree suggests that immune privilege is necessary, if not advantageous for the progressive development of the CNS. Upon reaching a certain degree of complexity, it seems as if the CNS was obliged to restrain the immune system from penetrating the blood-brain barrier. CNS autoimmunity against myelin proteins is known to be a contributory fa...
متن کاملCurrent trends in autoimmunity and the nervous system.
In the broad field of autoimmunity and clinical immunology, experimental evidence over the past few years have demonstrated several connections between the immune system and the nervous system, both central and peripheral, leading to the definition of neuroimmunology and of an immune-brain axis. Indeed, the central nervous system as an immune-privileged site, thanks to the blood-brain barrier, ...
متن کاملAn investigation of auto-reactivity after head injury.
Murine models of CNS injury show auto-reactive T cell responses directed at myelin antigens, associated with improved neuronal survival and functional recovery. This pilot study shows, for the first time, that similar immune responses against myelin occur in human traumatic brain injury (TBI), with an expansion of lymphocytes recognising myelin basic protein observed in 40% of patients studied....
متن کامل