Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cord
Definition
Paraneoplastic and autoimmune disorders of the central nervous system, brain and spinal cord nervous system result from a targeted immune attack on neurons or glial cells in the central (e.g. encephalopathy, ataxia, myelopathy, myelitis) nervous system. In the paraneoplastic context, this attack is a consequence of a potentially effective tumour immune response initiated by onco-neural antigens derived from a systemic cancer. In the non-paraneoplastic context termed ‘autoimmune’ the etiology remains elusive though increasing evidence indicates a preceding infectious trigger in at least some cases. These disorders are commonly multifocal causing injury and symptoms arising from involvement at many levels of the central nervous system. A personal or family history of autoimmunity is often found. Accompanying neural and non-organ specific (thyroid peroxidase [TPO] antibodies) autoantibodies may be found. The neural autoantibody profile may be predictive of a specific cancer type and may be associated with a particular neurological phenotype. Exclusion of alternative etiologies (e.g. infections) is important. Response to immunotherapy may support the diagnosis.
Also indexed as
Anti-NMDA receptor encephalitisAutoimmune brainstem encephalitisAutoimmune brainstem encephalitis, neural autoantibody negativeAutoimmune brainstem encephalitis, neural autoantibody positiveAutoimmune cerebellar degeneration or cerebellitisAutoimmune cerebellar degeneration or cerebellitis, neural autoantibody negativeAutoimmune cerebellar degeneration or cerebellitis, neural autoantibody positiveAutoimmune choreaAutoimmune dyskinesiaAutoimmune dystoniaAutoimmune encephalitisAutoimmune encephalitis, neural autoantibody negativeAutoimmune encephalitis, neural autoantibody positiveAutoimmune encephalopathyAutoimmune limbic encephalitisAutoimmune limbic encephalitis, Neural autoantibody negativeAutoimmune limbic encephalitis, Neural autoantibody positiveAutoimmune movement disorderAutoimmune movement disorder, autoantibody negativeAutoimmune movement disorder, autoantibody positiveAutoimmune myelopathyAutoimmune myelopathy, neural autoantibody negativeAutoimmune myelopathy, neural autoantibody positiveAutoimmune myoclonusCLIPPERS - [Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids]Certain specified neurological disorder aquaporin-4 antibody positiveChronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroidsHashimoto encephalitisHashimoto’s encephalopathyMoersch-Woltman syndromeNeuromyelitis optica spectrum disorderParaneoplastic brainstem encephalitisParaneoplastic brainstem encephalitis, neural autoantibody negativeParaneoplastic brainstem encephalitis, neural autoantibody positiveParaneoplastic cerebellar degenerationParaneoplastic cerebellar degeneration, neural autoantibody negativeParaneoplastic cerebellar degeneration, neural autoantibody positiveParaneoplastic choreaParaneoplastic dyskinesiaParaneoplastic dystoniaParaneoplastic encephalitisParaneoplastic encephalitis, neural autoantibody negativeParaneoplastic encephalitis, neural autoantibody positiveParaneoplastic encephalomyelitisParaneoplastic encephalomyelitis, neural autoantibody negativeParaneoplastic encephalomyelitis, neural autoantibody positiveParaneoplastic encephalopathyParaneoplastic limbic encephalitisParaneoplastic limbic encephalitis, neural autoantibody negativeParaneoplastic limbic encephalitis, neural autoantibody positiveParaneoplastic movement disorderParaneoplastic movement disorder, autoantibody negativeParaneoplastic movement disorder, autoantibody positiveParaneoplastic myelitisParaneoplastic myelopathyParaneoplastic myelopathy, neural autoantibody negativeParaneoplastic myelopathy, neural autoantibody positiveParaneoplastic or autoimmune disorders of the central nervous system, brain or spinal cordParaneoplastic rhomboencephalitisParaneoplastic stiff person syndrome classic formParaneoplastic stiff person syndrome variant formSREAT - [steroid-responsive-encephalopathy-associated with autoimmune thyroiditis]Stiff person syndromeStiff person syndrome classic formStiff person syndrome classic form, glutamic acid decarboxylase-autoantibody positiveStiff person syndrome classic form, other neural autoantibody negativeStiff person syndrome classic form, other neural autoantibody positiveStiff person syndrome variant formStiff person syndrome variant form, glutamic acid decarboxylase autoantibody positiveStiff person syndrome variant form, neural antibody negativeStiff person syndrome variant form, other neural antibody positiveautoimmune ataxiaautoimmune dementiaautoimmune myelitisautoimmune rhomboencephalitisimmunotherapy responsive encephalopathynon-vasculitic autoimmune meningoencephalitispaediatric autoimmune neuropsychiatric disorders associated with streptococcal infections [PANDAS]paraneoplastic ataxiaparaneoplastic brain stem syndromestiff man syndrome
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