Vial

INDIVIDUAL TEST 838

Glycine receptor (GlyR) antibodies

Indication Suspected Stiff person syndrome and especially PERM (Progressive encephalomyelitis with rigidity and myoclonus). 
 
Method The assay is performed by indirect immuno fluorescence (IIF). 

Result The result is reported as titer.  

Interpretation The analysis establishes if the patient sample contains autoantibodies against the glycine receptor (GlyR). Publications show that in most cases the antibodies bind to the glycine receptor alpha 1 (GlyRalfa 1). These antibodies are particularly associated with PERM (Progressive encephalomyelitis with rigidity and myoclonus) and Stiff person syndrome. In less than 10% of the cases, the anti-GlyR positive patients showed a correlation with paraneoplastic cancers. 

The antigen is a sub unit to pentameric inhibitory glycine receptor. This receptor mediates postsynaptic inhibition of the central nervous system. 

Generally, treatment with immunotherapy is more effective against auto-antibodies directed to extracellular antigen than with auto-antibodies against intracellular antigens. 

Antibodies against GlyR are graded as Lower-risk antibodies with a frequency of <10% of underlying cancer and a positive result yield 0 points PNS score, according to Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. 

References
  • Dalmau J, Geis C, Graus F. Autoantibodies to Synaptic Receptors and Neuronal Cell Surface Proteins in Autoimmune Diseases of the Central Nervous System. Physiol Rev. 2017 Apr;97(2):839-887. PMID: 28298428 
  • Alexopoulos H, Dalakas MC. The immunobiology of autoimmune encephalitides. J Autoimmun. 2019 Nov;104:102339. PMID: 31611142. 
  • Graus F, et al. Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. Neurol Neuroimmunol Neuroinflamm. 2021 May 18;8(4):e1014. PMID: 34006622

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Vial

ENSKILD ANALYS 838

Glycinreceptor-antikroppar

Indikation Misstanke om Stiff person syndrom och framför allt PERM (Progressiv encefalomyelit med rigiditet och myoklonier). 

Metod Analysen utföres med indirekt immunofluorescens (IIF).  

Svar Svaret anges som titer.  

Tolkning Analysen ger svar om patientprovet innehåller autoantikroppar riktade mot Glycinreceptor (GlyR). Publikationer visar att i flesta tillfällen binder antikropparna till Glycinreceptor alfa 1 (GlyRalfa 1). Dessa antikroppar är framför allt associerade med  varianten PERM (Progressiv encefalomyelit med rigiditet och myoklonier) vid stiff person syndrom. I mindre än 10% av fallen har anti-GlyR positiva patienter visat en korrelation med paraneoplastiska cancerformer. Antigenet är en subenhet till pentameric inhibitory glycine receptor. Denna receptor medierar postsynaptisk inhibering i det centrala nervsystemet.  

Generellt sett är behandling med immunoterapi oftare effektfull vid auto-antikroppar riktade mot extracellulära antigen än vid auto-antikroppar mot intracellulära antigen.    

Antikropparna riktade mot GlyR bedöms vara Lower-risk antibodies med en förkomst på <10% för en underliggande cancer och positivitet ger 0 poäng i PNS score enligt Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. 

Referenser
  • Dalmau J, Geis C, Graus F. Autoantibodies to Synaptic Receptors and Neuronal Cell Surface Proteins in Autoimmune Diseases of the Central Nervous System. Physiol Rev. 2017 Apr;97(2):839-887. PMID: 28298428 
  • Alexopoulos H, Dalakas MC. The immunobiology of autoimmune encephalitides. J Autoimmun. 2019 Nov;104:102339. PMID: 31611142. 
  • Graus F, et al. Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. Neurol Neuroimmunol Neuroinflamm. 2021 May 18;8(4):e1014. PMID: 34006622

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