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.
Index Terms
Paraneoplastic or autoimmune disorders of the central nervous system, brain or spinal cordParaneoplastic encephalitisParaneoplastic encephalitis, neural autoantibody positiveParaneoplastic encephalitis, neural autoantibody negativeAutoimmune encephalitisHashimoto encephalitisnon-vasculitic autoimmune meningoencephalitisAutoimmune encephalitis, neural autoantibody positiveAutoimmune encephalitis, neural autoantibody negativeParaneoplastic encephalopathyAutoimmune encephalopathyHashimoto’s encephalopathyimmunotherapy responsive encephalopathyautoimmune dementiaSREAT - [steroid-responsive-encephalopathy-associated with autoimmune thyroiditis]Paraneoplastic limbic encephalitisParaneoplastic limbic encephalitis, neural autoantibody positiveParaneoplastic limbic encephalitis, neural autoantibody negativeAutoimmune limbic encephalitisAutoimmune limbic encephalitis, Neural autoantibody positiveAutoimmune limbic encephalitis, Neural autoantibody negativeParaneoplastic brainstem encephalitisParaneoplastic rhomboencephalitisparaneoplastic brain stem syndromeParaneoplastic brainstem encephalitis, neural autoantibody positiveParaneoplastic brainstem encephalitis, neural autoantibody negativeAutoimmune brainstem encephalitisautoimmune rhomboencephalitisAutoimmune brainstem encephalitis, neural autoantibody positiveAutoimmune brainstem encephalitis, neural autoantibody negativeParaneoplastic cerebellar degenerationparaneoplastic ataxiaParaneoplastic cerebellar degeneration, neural autoantibody positiveParaneoplastic cerebellar degeneration, neural autoantibody negativeAutoimmune cerebellar degeneration or cerebellitisautoimmune ataxiaAutoimmune cerebellar degeneration or cerebellitis, neural autoantibody positiveAutoimmune cerebellar degeneration or cerebellitis, neural autoantibody negativeParaneoplastic encephalomyelitisParaneoplastic encephalomyelitis, neural autoantibody positiveParaneoplastic encephalomyelitis, neural autoantibody negativeParaneoplastic movement disorderParaneoplastic movement disorder, autoantibody positiveParaneoplastic movement disorder, autoantibody negativeParaneoplastic choreaParaneoplastic dyskinesiaAutoimmune movement disorderpaediatric autoimmune neuropsychiatric disorders associated with streptococcal infections [PANDAS]Autoimmune movement disorder, autoantibody positiveAutoimmune movement disorder, autoantibody negativeAutoimmune choreaAutoimmune dyskinesiaAutoimmune dystoniaAutoimmune myoclonusParaneoplastic myelopathyParaneoplastic myelitisParaneoplastic myelopathy, neural autoantibody positiveParaneoplastic myelopathy, neural autoantibody negativeAutoimmune myelopathyautoimmune myelitisAutoimmune myelopathy, neural autoantibody positiveAutoimmune myelopathy, neural autoantibody negativeStiff person syndromestiff man syndromeMoersch-Woltman 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 positiveParaneoplastic stiff person syndrome classic formStiff person syndrome variant formStiff person syndrome variant form, glutamic acid decarboxylase autoantibody positiveStiff person syndrome variant form, other neural antibody positiveStiff person syndrome variant form, neural antibody negativeParaneoplastic stiff person syndrome variant formNeuromyelitis optica spectrum disorderCertain specified neurological disorder aquaporin-4 antibody positiveChronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroidsCLIPPERS - [Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids]Anti-NMDA receptor encephalitisPost infectious encephalitisAcute demyelinating encephalomyelopathyParaneoplastic dystonia