Neuroimmunology diagnostic tests

Neuroimmunology diagnostic tests

Acetylcholine receptor (AChR) antibodies
Acetylcholine receptor antibodies (AChR)
Also known as	Anti-AChR
Assay Information	Autoantibodies to postsynaptic acetylcholine receptors are responsible for the muscle weakness and fatigabilities in myasthenia gravis. These antibodies are detectable in the serum of 80-90% of patients with generalized myasthenia gravis and in 55-70% of patients with ocular myasthenia. A good correlation is observed between antibody titres and muscle weakness in individual patients. The radio receptor assay for the determination of acetylcholine receptor antibodies in serum is highly specific and sensitive for use in the diagnosis of myasthenia gravis. Acetylcholine receptor from human muscle is used as the antigen. The receptors are labelled with 125I-alpha-bungarotoxin.This snake venom binds the receptors most specifically and almost irreversibly. Autoantibodies present in the patient’s serum attach to the labelled receptors. The resulting immune complexes are subsequently precipitated with anti IgG. The amount of radioactivity in the precipitate is directly proportional to the concentration of acetylcholine receptor autoantibodies in the sample.
Specimen Type(s) & Minimum Volume	Serum - 0.2mL
Cost	£18.00
Transport	First class post
Frequency of Analysis/Turnaround Time	7 Working Days
Assay Method	Radioimmunoassay
Reference Range & Units	< 2.5 X 10-10 mol/L Negative 2.5-4.0 X 10-10 mol/L Equivocal >4.0 X 10-10 mol/L Positive
Factors Affecting Performance of Examination	Gross Haemolysis
Related Tests	MuSK antibodies
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	UKNEQAS for immunology scheme for acetyl choline receptor antibodies. IBL quality assessment scheme for acetylcholine receptor autoantibodies. INSTAND ev scheme for autoimmune diseases 10
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Lindstrom J. (Clin. Immunol. Immunopathol. 1977; 7: 36 – 43) Vincent. A., Newsom-Davis. J. (Neurol. Neurosurg. & Psych. 1985; 48 1246 –1252)
Date last updated	Tuesday, 17 August 2021

AMPA antibodies

AMPA1 and AMPA2 antibodies
Also known as	Glutamate receptor (type AMPA) antibodies
Assay information	Autoantibodies against neuronal surface antigens are found in patients with autoimmune encephalitis. As well as other antigens, they may be directed against glutamate receptors (types NMDA and AMPA), GABA-B receptors, components of the voltage-gated potassium channels (VGKC, DPPX) or VGKC-associated proteins (LGI1, CASPR2, Contactin-2). As these antigens play a role in synaptic signal transduction and plasticity, patients often present with seizures and neuropsychiatric symptoms. The resulting conditions include forms of autoimmune limbic encephalitis, neuromyotonia or Morvan’s syndrome. Transfected cells expressing individual neuronal cell-surface antigens are incubated with diluted sample. If specific IgG antibodies to the expressed epitopes are present they will attach to the antigen. In a second incubation the attached antibodies are stained with fluorescein-labelled anti-human antibody and evaluated using a fluorescence microscope.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF - 0.2 mL
Cost	£80.00 Includes AMPA1 and AMPA2, and GABA-B antibodies
Transport	First class post
Frequency of analysis/Turnaround time	Turnaround time 10 working days
Assay method	Indirect Immunofluorescence
Reference Range & Units	Not applicable
Factors Affecting Performance of Examination	No interferences known with neuronal antibodies.
Related Tests	Autoimmune Encephalitis panel GABA-B antibodies NMDAR antibodies LGI1 & CASPR2 antibodies
Accredited Assay	UKAS 8642 CSF anti-AMPA1 & 2 assay has been fully verified for diagnostic testing, but is not currently UKAS accredited
External Quality Assurance (EQA)	Sample exchange – multiple lab sample exchange scheme. Results monitored via monthly EQA report and QC meeting. In-house blinded samples run regularly.
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Höftberger et al. Encephalitis and AMPA receptor antibodies. Neurology 2015; 84(24):2403-2412.
Date last updated	Tuesday 25 April 2023

Amphiphysin antibodies

GAD65 & Amphiphysin antibodies
Assay Information	Stiff-Person Syndrome is a rare neurological autoimmune disease that is characterised by rigidity and episodic spasms of muscles as a result of continuous motor unit activity. Antibodies to GAD (Glutamic Acid Decarboxylase) and Amphiphysin are considered as serological markers for Stiff-Person-Syndrome. The majority of patients have high titres of antibodies to both GAD isoforms, GAD65 and GAD67. These enzymes catalyse the conversion of glutamate to GABA (ã-aminobutyric acid), a major inhibitory neurotransmitter of the CNS. Amphiphysin expression is found in synaptic vesicles of neurons as well as in the skeletal musculature. The presence of anti-Amphiphysin antibodies (with or without GAD) indicates a paraneoplastic neurological syndrome caused by an underlying tumour. Nitrocellulose strips coated with recombinant antigens are incubated with patient serum. Specific antibodies in the serum will bind to the antigens whilst non-specific molecules will be removed by washing. Bound antibodies are detected by alkaline phosphate conjugated anti-human IgG using BCIP/NBT as substrate. Note – the assay is only suitable for neurological anti-GAD testing and will not detect the generally lower titres found in type 1 diabetes mellitus.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF – 0.2 mL
Cost	£30.00 (Includes GAD antibody titre, if needed)
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days
Assay Method	Recombinant Immunoblot
Reference Range & Units	GAD & amphiphysin antibodies are not normally present.
Factors Affecting Performance of Examination	No known interferences.
Related Tests	Not applicable
Accredited Assay	UKAS 864
External Quality Assurance (EQA)	NEQAS scheme for paraneoplastic antibodies Institute for Quality Assurance - autoantibodies against neuronal antigens scheme
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	To follow
Date last updated	Tuesday, 17 August 2021

Autoimmune Encephalitis panel

Autoimmune Encephalitis Antibody Panel
Also known as	NMDAR, LGI1, CASPR2, AMPA1 & 2, DPPX, and GABA-B antibodies
Assay Information	Autoantibodies against neuronal surface antigens are found in patients with autoimmune encephalitis. As well as other antigens, they may be directed against glutamate receptors (types NMDA and AMPA), GABA-B receptors, and components of the voltage-gated potassium channels (VGKC, DPPX) or VGKC-associated proteins (LGI1, CASPR2, Contactin-2). As these antigens play a role in synaptic signal transduction and plasticity, patients often present with seizures and neuropsychiatric symptoms. The resulting conditions include forms of autoimmune limbic encephalitis, neuromyotonia or Morvan’s syndrome. Transfected cells expressing individual neuronal cell-surface antigens are incubated with diluted sample. If specific IgG antibodies to the expressed epitopes are present they will attach to the antigen. In a second incubation the attached antibodies are stained with fluorescein-labelled anti-human antibody and evaluated using a fluorescence microscope.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF - 0.2 mL
Cost	£110.00
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days
Assay Method	Indirect Immunofluorescence
Reference Range & Units	Not applicable
Factors Affecting Performance of Examination	No interferences known with neuronal antibodies.
Related Tests	AMPA1 & AMPA2 antibodies NMDAR antibodies LG1 & CASPR2 antibodies GABA-B antibodies DPPX antibodies
Accredited Assay	UKAS 8642 CSF anti-AMPA and anti-DPPX assays have been fully verified for diagnostic testing, but are not currently UKAS accredited. All other autoimmune encephalitis antibody assays are UKAS accredited.
External Quality Assurance (EQA)	Institute for Quality Assurance - autoantibodies against neuronal antigens scheme (NMDAR, LGI1 & CASPR2) Sample exchange – multiple lab sample exchange on a quarterly basis (NMDAR, AMPA, GABA-B, LGI1 & CASPR2) In-house blinded samples run regularly
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	To be updated
Date last updated	Wednesday 25 May 2022

CASPR2 antibodies

LGI1& CASPR2 antibodies
Assay Information	Autoantibodies against neuronal surface antigens are found in patients with autoimmune encephalitis. Some antibodies are directed against voltage-gated potassium channel-associated proteins (VGKC-associated proteins.) Two important epitopes are LGI1 (leucine-rich glioma-inactivated protein 1) and CASPR2 (contactin-associated protein 2). As these antigens play a role in synaptic signal transduction and plasticity, patients often present with seizures and neuropsychiatric symptoms. The resulting conditions include forms of autoimmune limbic encephalitis, neuromyotonia or Morvan’s syndrome. Transfected cells expressing individual neuronal cell-surface antigens are incubated with diluted sample. If specific IgG antibodies to the expressed epitopes are present they will attach to the antigen. In a second incubation the attached antibodies are stained with fluorescein-labelled anti-human antibody and evaluated using a fluorescence microscope.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF - 0.2mL
Cost	£70.00 Includes LGI1 & CASPR2 antibodies
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days
Assay Method	Indirect Immunofluorescence
Reference Range & Units	Not Applicable
Factors Affecting Performance of Examination	No interferences known with neuronal antibodies.
Related Tests	Autoimmune Encephalitis panel GABA-B antibodies NMDAR antibodies AMPA1 & AMPA2 antibodies
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	Institute for Quality Assurance - autoantibodies against neuronal antigens scheme Sample exchange – multiple lab sample exchange scheme. Results monitored via monthly EQA report and QC meeting. In-house blinded samples run regularly.
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	van Sonderen A, Schreurs MW, Wirtz PW, Sillevis Smitt PA & Titulaer MJ From VGKC to LGI1 and Caspr2 encephalitis: The evolution of a disease entity over time. Autoimmun Revs 2016; 15(10): 970-974.
Date last updated	Tuesday, 17 August 2021

Disialosyl antibodies

GD1b & GQ1b antibodies

Ganglioside Antibodies
Also known as	Glycolipid antibodies GM1, GM2, GD1a, GD1b, GQ1b, disialosyl antibodies
Assay Information	Gangliosides are sialic acid-containing glycosphingolipids composed of a long-chain aliphatic amine, ceramide, attached to one to five hexoses, at least one of which must be sialylated. It is the presence of a sialic acid molecule(s) attached to a galactose residue(s) in the hexose core which defines a glycosphingolipid as a ganglioside. In the human nervous system, the sialic acid is N-acetylneuraminic acid (NeuNAc). Ganglioside nomenclature is assigned according to the system of Svennerholm, in which the prefix G refers to “ganglio,” M, D, T, and Q refer to the number of sialic acid molecules (mono, di, tri, and quad), and arabic numerals and lowercase letters refer to the order of migration of the gangliosides on thin-layer chromatograms (TLC). The major gangliosides in the brain are GM1, GD1a, GD1b, and GT1b, but there are also many minor gangliosides in both the brain and peripheral nerves and in other tissues. Although not usually essential, antibodies against gangliosides are diagnostically useful. When identified in patients with a compatible clinical syndrome, they are strongly supportive of a diagnosis and may identify a regional or pathological variant. Disialosyl antibodies are associated with a spectrum of acute and chronic ataxic neuropathies. The disialosyl epitope is common to a number of gangliosides including GD1b and GQ1b. Assay is a standardised anti-ganglioside ELISA originally proposed by European INCAT group and published by Willison et al (1999). Individual gangliosides are immobilised on the wells of a microtitre plate. After blocking, diluted patient serum is incubated overnight at 4oC. Bound immunoglobulin (IgG and IgM) is detected using peroxidase-conjugated anti-IgG gamma-chains and anti-IgM mu-chains. Absorbance is read at 492nm. GM1, GM2, GD1a, GD1b and GQ1b gangliosides are used as these will allow detection of most clinically significant antibodies. As there are hexose sequences that are shared, some antibodies will react with more than one ganglioside.
Specimen Type(s) & Minimum Volume	Serum - 0.2mL
Cost	£30.00
Transport	First class post
Frequency of Analysis/Turnaround Time	10 Working Days
Assay Method	ELISA
Reference Range & Units	Antibodies to some gangliosides may be found at low titres in both normal and disease control samples. The distribution of anti-ganglioside antibodies in the population does not follow a normal distribution but is skewed, with some control subjects having high antibody levels against some gangliosides (titres of >1/1,000) and most having undetectable levels (titres of <1/100).
Factors Affecting Performance of Examination	Circulating heterophile antibodies
Related Tests	MAG antibodies Protein electrophoresis
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	UK NEQAS for immunology scheme for ganglioside antibodies INSTAND ev scheme for autoimmune diseases 09
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Inter-laboratory validation of an ELISA for the determination of serum anti-ganglioside antibodies. Willison HJ et al. Eur J Neurol 1999;6:71-7. Willison HJ, Veitch J, Swan AV, Baumann N, Comi G, Gregson NA, Illa I, Zielasek J, Hughes RA. Inter-laboratory validation of an ELISA for the determination of serum anti-ganglioside antibodies. Eur J Neurol. 1999; 6:71–77. Willison HJ. 2016. Autoantibodies to Glycolipids in Peripheral Neuropathy, p 961-965. In Detrick B, Schmitz JL, Hamilton RG (ed), Manual of Molecular and Clinical Laboratory Immunology, 8th Edition. ASM Press, Washington, DC.
Date last updated	Wednesday, 18 August 2021

DPPX antibodies

DPPX Antibodies
Assay Information	Dipeptidylpeptidase–like protein 6 (DPPX) is a regulatory protein of the Kv4.2 potassium channels that is involved in signal integration and attenuation of action potentials. DPPX antibodies are predominantly IgG1 and IgG4 and associate with cognitive-mental deficits and symptoms of CNS hyperexcitability that are usually preceded by diarrhoea, other gastrointestinal symptoms, and weight loss. The disorder is responsive to immunotherapy. Transfected DPPX expressing cells are incubated with diluted sample. If specific IgG antibodies to DPPX are present they will attach to the antigen. In a second incubation the attached antibodies are stained with fluorescein-labelled anti-human antibody and evaluated using a fluorescence microscope.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF - 0.2 mL
Cost	£50.00
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days
Assay Method	Indirect immunofluorescence
Reference Range & Units	Antibodies against DPPX are not normally present.
Factors Affecting Performance of Examination	No known interferences with neuronal antibodies, haemolysis, lipaemia or bilirubin
Related Tests	Autoimmune Encephalitis panel GABA-B antibodies NMDAR antibodies AMPA1 and AMPA2 antibodies
Accredited Assay	UKAS 8642 (Serum assay only) CSF anti-DPPX assay has been fully verified for diagnostic testing, but is not currently UKAS accredited.
External Quality Assurance (EQA)	Sample exchange – multiple lab sample exchange scheme. Results monitored via monthly EQA report and QC meeting. In-house blinded samples run regularly
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Hara, Makoto et al (2017). DPPX antibody–associated encephalitis. Neurology, 88(14), 1340–1348
Date last updated	Tuesday 25 April 2023

GABA-B antibodies

GABA-B antibodies
Assay Information	Autoantibodies against neuronal surface antigens are found in patients with autoimmune encephalitis. As well as other antigens, they may be directed against glutamate receptors (types NMDA and AMPA), GABA-B receptors, components of the voltage-gated potassium channels (VGKC, DPPX) or VGKC-associated proteins (LGI1, CASPR2, Contactin-2). As these antigens play a role in synaptic signal transduction and plasticity, patients often present with seizures and neuropsychiatric symptoms. The resulting conditions include forms of autoimmune limbic encephalitis, neuromyotonia or Morvan’s syndrome. Transfected cells expressing individual neuronal cell-surface antigens are incubated with diluted sample. If specific IgG antibodies to the expressed epitopes are present they will attach to the antigen. In a second incubation the attached antibodies are stained with fluorescein-labelled anti-human antibody and evaluated using a fluorescence microscope.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF - 0.2 mL
Cost	£80.00 Includes AMPA and GABA-B antibodies
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days.
Assay Method	Indirect Immunofluorescence
Reference Range & Units	Not applicable
Factors Affecting Performance of Examination	No interferences known with neuronal antibodies.
Related Tests	Autoimmune Encephalitis panel AMPA1 & AMPA2 antibodies NMDAR antibodies LGI1 & CASPR2 antibodies
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	Sample exchange – multiple lab sample exchange scheme. Results monitored via monthly EQA report and QC meeting. In-house blinded samples run regularly.
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Höftberger et al. Encephalitis and GABAB receptor antibodies. Neurology 2013; 81(17):1500-1506.
Date last updated	Tuesday 25 April 2023

GAD65 antibodies

GAD65 & Amphiphysin antibodies
Assay Information	Stiff-Person Syndrome is a rare neurological autoimmune disease that is characterised by rigidity and episodic spasms of muscles as a result of continuous motor unit activity. Antibodies to GAD (Glutamic Acid Decarboxylase) and Amphiphysin are considered as serological markers for Stiff-Person-Syndrome. The majority of patients have high titres of antibodies to both GAD isoforms, GAD65 and GAD67. These enzymes catalyse the conversion of glutamate to GABA (ã-aminobutyric acid), a major inhibitory neurotransmitter of the CNS. Amphiphysin expression is found in synaptic vesicles of neurons as well as in the skeletal musculature. The presence of anti-Amphiphysin antibodies (with or without GAD) indicates a paraneoplastic neurological syndrome caused by an underlying tumour. Nitrocellulose strips coated with recombinant antigens are incubated with patient serum. Specific antibodies in the serum will bind to the antigens whilst non-specific molecules will be removed by washing. Bound antibodies are detected by alkaline phosphate conjugated anti-human IgG using BCIP/NBT as substrate. Note – the assay is only suitable for neurological anti-GAD testing and will not detect the generally lower titres found in type 1 diabetes mellitus.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF – 0.2 mL
Cost	£30.00 (Includes GAD antibody titre, if needed)
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days
Assay Method	Recombinant Immunoblot
Reference Range & Units	GAD & amphiphysin antibodies are not normally present.
Factors Affecting Performance of Examination	No known interferences.
Related Tests	Not applicable
Accredited Assay	UKAS 864
External Quality Assurance (EQA)	NEQAS scheme for paraneoplastic antibodies Institute for Quality Assurance - autoantibodies against neuronal antigens scheme
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	To follow
Date last updated	Tuesday, 17 August 2021

Ganglioside antibodies

GM1, GM2, GD1a, GD1b & GQ1b antibodies

Ganglioside Antibodies
Also known as	Glycolipid antibodies GM1, GM2, GD1a, GD1b, GQ1b, disialosyl antibodies
Assay Information	Gangliosides are sialic acid-containing glycosphingolipids composed of a long-chain aliphatic amine, ceramide, attached to one to five hexoses, at least one of which must be sialylated. It is the presence of a sialic acid molecule(s) attached to a galactose residue(s) in the hexose core which defines a glycosphingolipid as a ganglioside. In the human nervous system, the sialic acid is N-acetylneuraminic acid (NeuNAc). Ganglioside nomenclature is assigned according to the system of Svennerholm, in which the prefix G refers to “ganglio,” M, D, T, and Q refer to the number of sialic acid molecules (mono, di, tri, and quad), and arabic numerals and lowercase letters refer to the order of migration of the gangliosides on thin-layer chromatograms (TLC). The major gangliosides in the brain are GM1, GD1a, GD1b, and GT1b, but there are also many minor gangliosides in both the brain and peripheral nerves and in other tissues. Although not usually essential, antibodies against gangliosides are diagnostically useful. When identified in patients with a compatible clinical syndrome, they are strongly supportive of a diagnosis and may identify a regional or pathological variant. Disialosyl antibodies are associated with a spectrum of acute and chronic ataxic neuropathies. The disialosyl epitope is common to a number of gangliosides including GD1b and GQ1b. Assay is a standardised anti-ganglioside ELISA originally proposed by European INCAT group and published by Willison et al (1999). Individual gangliosides are immobilised on the wells of a microtitre plate. After blocking, diluted patient serum is incubated overnight at 4oC. Bound immunoglobulin (IgG and IgM) is detected using peroxidase-conjugated anti-IgG gamma-chains and anti-IgM mu-chains. Absorbance is read at 492nm. GM1, GM2, GD1a, GD1b and GQ1b gangliosides are used as these will allow detection of most clinically significant antibodies. As there are hexose sequences that are shared, some antibodies will react with more than one ganglioside.
Specimen Type(s) & Minimum Volume	Serum - 0.2mL
Cost	£30.00
Transport	First class post
Frequency of Analysis/Turnaround Time	10 Working Days
Assay Method	ELISA
Reference Range & Units	Antibodies to some gangliosides may be found at low titres in both normal and disease control samples. The distribution of anti-ganglioside antibodies in the population does not follow a normal distribution but is skewed, with some control subjects having high antibody levels against some gangliosides (titres of >1/1,000) and most having undetectable levels (titres of <1/100).
Factors Affecting Performance of Examination	Circulating heterophile antibodies
Related Tests	MAG antibodies Protein electrophoresis
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	UK NEQAS for immunology scheme for ganglioside antibodies INSTAND ev scheme for autoimmune diseases 09
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Inter-laboratory validation of an ELISA for the determination of serum anti-ganglioside antibodies. Willison HJ et al. Eur J Neurol 1999;6:71-7. Willison HJ, Veitch J, Swan AV, Baumann N, Comi G, Gregson NA, Illa I, Zielasek J, Hughes RA. Inter-laboratory validation of an ELISA for the determination of serum anti-ganglioside antibodies. Eur J Neurol. 1999; 6:71–77. Willison HJ. 2016. Autoantibodies to Glycolipids in Peripheral Neuropathy, p 961-965. In Detrick B, Schmitz JL, Hamilton RG (ed), Manual of Molecular and Clinical Laboratory Immunology, 8th Edition. ASM Press, Washington, DC.
Date last updated	Wednesday, 18 August 2021

Glycolipid antibodies

GM1, GM2, GD1a, GD1b & GQ1b antibodies

Ganglioside Antibodies
Also known as	Glycolipid antibodies GM1, GM2, GD1a, GD1b, GQ1b, disialosyl antibodies
Assay Information	Gangliosides are sialic acid-containing glycosphingolipids composed of a long-chain aliphatic amine, ceramide, attached to one to five hexoses, at least one of which must be sialylated. It is the presence of a sialic acid molecule(s) attached to a galactose residue(s) in the hexose core which defines a glycosphingolipid as a ganglioside. In the human nervous system, the sialic acid is N-acetylneuraminic acid (NeuNAc). Ganglioside nomenclature is assigned according to the system of Svennerholm, in which the prefix G refers to “ganglio,” M, D, T, and Q refer to the number of sialic acid molecules (mono, di, tri, and quad), and arabic numerals and lowercase letters refer to the order of migration of the gangliosides on thin-layer chromatograms (TLC). The major gangliosides in the brain are GM1, GD1a, GD1b, and GT1b, but there are also many minor gangliosides in both the brain and peripheral nerves and in other tissues. Although not usually essential, antibodies against gangliosides are diagnostically useful. When identified in patients with a compatible clinical syndrome, they are strongly supportive of a diagnosis and may identify a regional or pathological variant. Disialosyl antibodies are associated with a spectrum of acute and chronic ataxic neuropathies. The disialosyl epitope is common to a number of gangliosides including GD1b and GQ1b. Assay is a standardised anti-ganglioside ELISA originally proposed by European INCAT group and published by Willison et al (1999). Individual gangliosides are immobilised on the wells of a microtitre plate. After blocking, diluted patient serum is incubated overnight at 4oC. Bound immunoglobulin (IgG and IgM) is detected using peroxidase-conjugated anti-IgG gamma-chains and anti-IgM mu-chains. Absorbance is read at 492nm. GM1, GM2, GD1a, GD1b and GQ1b gangliosides are used as these will allow detection of most clinically significant antibodies. As there are hexose sequences that are shared, some antibodies will react with more than one ganglioside.
Specimen Type(s) & Minimum Volume	Serum - 0.2mL
Cost	£30.00
Transport	First class post
Frequency of Analysis/Turnaround Time	10 Working Days
Assay Method	ELISA
Reference Range & Units	Antibodies to some gangliosides may be found at low titres in both normal and disease control samples. The distribution of anti-ganglioside antibodies in the population does not follow a normal distribution but is skewed, with some control subjects having high antibody levels against some gangliosides (titres of >1/1,000) and most having undetectable levels (titres of <1/100).
Factors Affecting Performance of Examination	Circulating heterophile antibodies
Related Tests	MAG antibodies Protein electrophoresis
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	UK NEQAS for immunology scheme for ganglioside antibodies INSTAND ev scheme for autoimmune diseases 09
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Inter-laboratory validation of an ELISA for the determination of serum anti-ganglioside antibodies. Willison HJ et al. Eur J Neurol 1999;6:71-7. Willison HJ, Veitch J, Swan AV, Baumann N, Comi G, Gregson NA, Illa I, Zielasek J, Hughes RA. Inter-laboratory validation of an ELISA for the determination of serum anti-ganglioside antibodies. Eur J Neurol. 1999; 6:71–77. Willison HJ. 2016. Autoantibodies to Glycolipids in Peripheral Neuropathy, p 961-965. In Detrick B, Schmitz JL, Hamilton RG (ed), Manual of Molecular and Clinical Laboratory Immunology, 8th Edition. ASM Press, Washington, DC.
Date last updated	Wednesday, 18 August 2021

IgLON5 antibodies

IgLON5 Antibodies
Also known as
Assay Information	Autoantibodies against IgLON5 are a marker for neuroimmunological disease. Due to the heterogenous clinical presentation, determination of anti-IgLON5 may be considered in the differential diagnosis of autoimmune encephalitis, Creutzfeldt-Jakob disease or rapidly progressive neurodegenerative dementia. In differential diagnosis, IgLON5 antibodies are particularly relevant in patients with suspected limbic encephalitis. The cell membrane antigen IgLON5 is an immunological target for specific autoantibodies. IgLON5 autoantibodies are associated with sleep disorders from parasomnia to complete sleeplessness. The disorders occur in rapid-eye movement (REM) as well as non-REM sleep phases. The most frequent symptoms during these sleep dysfunctions are abnormal movement and behaviour, obstructive sleep apnoea, stridor, dysarthria, dysphagia, sleepwalking, ataxia and chorea. It is suspected that IgLON5 autoantibodies lead to pathological aggregation of tau protein in the brain and thus to neurodegenerative disease. No tumours have so far been associated with IgLON5 autoantibody –associated tauopathy. Transfected IgLON5 expressing cells are incubated with diluted sample. If specific IgG antibodies to IgLON5 are present they will attach to the antigen. In a second incubation the attached antibodies are stained with fluorescein-labelled anti-human antibody and evaluated using a fluorescence microscope.
Specimen Type(s) & Minimum Volume	Serum - 0.2mL CSF - 0.2 mL
Cost	£50.00
Transport	First class post
Frequency of Analysis/Turnaround Time	10 Working Days
Assay Method	Indirect Immunofluorescence
Reference Range & Units	Antibodies against IgLON5 are not normally present.
Factors Affecting Performance of Examination	No known interferences with neuronal antibodies, haemolysis, lipaemia or bilirubin.
Related Tests	Not applicable
Accredited Assay	UKAS 8642 (Serum assay only) CSF assay is for research only - non UKAS accredited
External Quality Assurance (EQA)	In-house blinded samples run regularly
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	To follow
Date last updated	14/04/2022

LGI1 antibodies

LGI1& CASPR2 antibodies
Assay Information	Autoantibodies against neuronal surface antigens are found in patients with autoimmune encephalitis. Some antibodies are directed against voltage-gated potassium channel-associated proteins (VGKC-associated proteins.) Two important epitopes are LGI1 (leucine-rich glioma-inactivated protein 1) and CASPR2 (contactin-associated protein 2). As these antigens play a role in synaptic signal transduction and plasticity, patients often present with seizures and neuropsychiatric symptoms. The resulting conditions include forms of autoimmune limbic encephalitis, neuromyotonia or Morvan’s syndrome. Transfected cells expressing individual neuronal cell-surface antigens are incubated with diluted sample. If specific IgG antibodies to the expressed epitopes are present they will attach to the antigen. In a second incubation the attached antibodies are stained with fluorescein-labelled anti-human antibody and evaluated using a fluorescence microscope.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF - 0.2mL
Cost	£70.00 Includes LGI1 & CASPR2 antibodies
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days
Assay Method	Indirect Immunofluorescence
Reference Range & Units	Not Applicable
Factors Affecting Performance of Examination	No interferences known with neuronal antibodies.
Related Tests	Autoimmune Encephalitis panel GABA-B antibodies NMDAR antibodies AMPA1 & AMPA2 antibodies
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	Institute for Quality Assurance - autoantibodies against neuronal antigens scheme Sample exchange – multiple lab sample exchange scheme. Results monitored via monthly EQA report and QC meeting. In-house blinded samples run regularly.
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	van Sonderen A, Schreurs MW, Wirtz PW, Sillevis Smitt PA & Titulaer MJ From VGKC to LGI1 and Caspr2 encephalitis: The evolution of a disease entity over time. Autoimmun Revs 2016; 15(10): 970-974.
Date last updated	Tuesday, 17 August 2021

MAG antibodies

Myelin Associated Glycoprotein (MAG) antibodies
Assay Information	IgM antibodies to MAG (myelin associated glycoprotein) have been associated with demyelinating peripheral neuropathy of both autoimmune and paraneoplastic origin. Where anti-MAG antibodies have a malignant/paraneoplastic origin, plasma cell dyscrasias have been implicated and IgM anti-MAG antibodies are frequently associated with IgM monoclonal gammopathies This method is intended to detect IgM antibodies to the 100 kDa isoform of MAG using a commercially available enzyme-linked immunosorbent assay (ELISA) method. The kit contains wells that are coated with MAG isolated from human brain. Any anti-MAG antibodies present in the sample are bound by the immobilized human MAG. Any unbound substances are washed away then a horseradish peroxidase (HRP) labelled antibody against human IgM is added. The substrate solution containing tetramethylbenzidine (TMB) is added and a blue colour develops in proportion to the amount of anti-MAG autoantibodies present in the original sample. The colour development is stopped by the addition of sulphuric acid which also turns the blue solution yellow. The intensity of the colour absorbance is measured at a wavelength of 450nm.
Specimen Type(s) & Minimum Volume	Serum - 0.2mL
Cost	£35.00
Transport	First class post
Frequency of Analysis/Turnaround Time	21 Working Days
Assay Method	ELISA
Reference Range & Units	<1000 BTU
Factors Affecting Performance of Examination	Not applicable
Related Tests	Ganglioside antibodies Protein electrophoresis
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	UK NEQAS for myelin associated glycoprotein IgM antibodies INSTAND ev scheme for autoimmune diseases 09
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Myelin-associated glycoprotein (MAG): past, present and beyond. Quarles RH. J Neurochem 100; 2007: 1431–1448. Placebo-Controlled Trial of Rituximab in IgM Anti–Myelin-Associated Glycoprotein Antibody Demyelinating Neuropathy. Dalakas M, Rakocevic G, Salajegheh M, Dambrosia JM, Hahn AF, Raju R, McElroy B. Ann Neurol 65; 2009: 286–293. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of paraproteinaemic demyelinating neuropathies: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. Haddena RDM, Nobile-Oraziob E, Sommerc C, Hahnd A, Illae I, Morraf E, Pollardg J, Hughesh RAC, Bouchei P, Cornblathj D, Eversk E, Koskil CL, Le ́germ JM, Van den Berghn P, van Doorno P, van Schaikp IN. Eur J Neurol 13; 2006: 809–818. Differential Diagnosis of Chronic Dysimmune Demyelinating Polyneuropathies with and without Anti-MAG Antibodies. Isoardo G, Migliaretti G, Ciaramtiaro P, Rota E, Poglio F, Tavella A, Paolasso I, Cavallo F, Bergamasco B, Cocito D. Muscle Nerve 31; 2005: 52–58
Date last updated	Tuesday, 17 August 2021

MuSK (Muscle-specific kinase) antibodies

Muscle specific tyrosine kinase (MuSK) antibodies
Assay Information	The measurement of autoantibodies to muscle-specific receptor tyrosine kinase (MuSK) is useful in the diagnosis of patients with acetylcholine receptor (AChRAb) antibody-negative myasthenia gravis (MG). Serum samples are incubated with I125 labelled MuSK and any immune complexes formed are precipitated with anti-human IgG. The radioactivity present in the washed precipitates is then counted and anti-MuSK calculated.
Specimen Type(s) & Minimum Volume	Serum - 0.2mL
Cost	£20.00
Transport	First class post
Frequency of Analysis/Turnaround Time	10 Working Days
Assay Method	Radioimmunoassay
Reference Range & Units	<0.05nmol/L
Factors Affecting Performance of Examination	No known interferences.
Related Tests	Acetylcholine receptor antibodies (AChR)
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	INSTAND ev scheme for autoimmune diseases 10
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Matthews I, Chen S, Hewer R, McGrath V, Furmaniak J & Rees Smith B. Muscle specific receptor tyrosine kinase autoantibodies – a new immunoprecipitation assay. Clin Chim Acta 348 (2004) 95-99.
Date last updated	Tuesday, 17 August 2021

Neuronal antibodies

Hu(D), Yo, CV2/CRMP5, Ri, Ma1, Ma2, amphiphysin,,Tr, SOX1, GAD65, Zic4, titin, recoverin, PKCγ & Purkinje cell antibodies

Paraneoplastic antibodies
Also known as	Onconeural antibodies, Neuronal antibodies Hu(D), Yo, CV2/CRMP5, Ri, Ma1, Ma2, amphiphysin,,Tr, SOX1, GAD65, Zic4, titin, recoverin, PKCγ & Purkinje cell antibodies
Assay Information	Paraneoplastic neurological syndromes (PNS) are a group of neurological disorders associated with malignancy that are not directly caused by either the primary tumour or metastases. An autoimmune process is considered to be the underlying pathophysiological mechanism. Detection of one or more of the well characterized autoantibodies in the presence of paraneoplastic neurological symptoms provides strong diagnostic evidence of a neoplasm, possibly occult. In many cases PNS precedes the detection of the underlying malignancy by up to five years. Paraneoplastic antibodies are antibodies that are associated with certain tumour types and can produce paraneoplastic syndromes, most of which have neurological involvement. Paraneoplastic antibodies are screened using two assays: Immunohistochemistry Serum/CSF samples are applied to sections of fresh rat brain and, following incubation developed using a DAB chromogen. If antibodies present, staining patterns will be dependent on specificity. Recombinant Immunoblot Nitrocellulose strips coated with recombinant Hu(D), Yo, CV2/CRMP5, Ri, Ma1, Ma2, amphiphysin,,Tr, SOX1, GAD65, Zic4, titin, recoverin and PKCg antigens are incubated with patient serum. Specific antibodies in the serum will bind to the antigens whilst non-specific molecules will be removed by washing. Bound antibodies are detected by alkaline phosphate conjugated anti-human IgG using BCIP/NBT as substrate.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF – 0.2 mL
Cost	£25.00
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days
Assay Method	Immunohistochemistry & recombinant Immunoblot
Reference Range & Units	Paraneoplastic antibodies are not normally present.
Factors Affecting Performance of Examination	No known interferences.
Related Tests	GAD65 antibodies
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	NEQAS scheme for paraneoplastic antibodies Institute for Quality Assurance - autoantibodies against neuronal antigens scheme
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	To follow
Date last updated	Wednesday, 18 August 2021

NMDAR receptor antibodies

N- methyl-D-aspartate receptor (NMDAR) antibodies
Also known as	Glutamate receptor (type NMDA) antibodies
Assay Information	Antibodies against glutamate receptor (type N- methyl-D-aspartate (NMDA)) are specific markers for anti-glutamate receptor (type NMDA) encephalitis, an inflammatory encephalopathic autoimmune disease which was first described in 2007 and is currently still widely underdiagnosed. NMDA receptors belong to the ionotropic glutamate receptors and were named according to their ability to be activated by the synthetic amino acid N-methyl-D-aspartate (NMDA). They are localised in the post-synaptic membrane and form cation canals with major significance for synaptic transmission and plasticity. The receptors consist of two subunits, NR1 and NR2. Their activity is regulated by the binding of ligands, such as the neurotransmitter glutamate. The serum from patients with anti-glutamate receptor (type NMDA) encephalitis contains autoantibodies directed against an extracellular epitope of the NR1 subunit. The occurrence of these specific antibodies and the possibility of immunotherapeutic intervention suggest an immune-mediated pathogenesis for anti-glutamate receptor (type NMDA) encephalitis. In cell culture experiments on hippocampal neurons it could be demonstrated that the binding of the antibodies induced a reversible reduction in glutamate receptors (type NMDA) on the neuronal cell surface. Furthermore, a pharmacological blockade of the receptors with NMDA antagonists causes clinical symptoms similar to those of anti-glutamate receptor (type NMDA) encephalitis, in particular psychosis.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF - 0.2 mL
Cost	£35.00
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days
Assay Method	Indirect Immunofluorescence
Reference Range & Units	Not applicable
Factors Affecting Performance of Examination	No interferences known with neuronal antibodies.
Related Tests	AMPA1 & AMPA2 antibodies LG1 & CASPR2 antibodies GABA-B antibodies NMDAR antibodies
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	UK NEQAS (Immunology, Immunochemistry & Allergy) – N-Methyl-D-Aspartate Receptor Antibodies Pilot Scheme Institute for Quality Assurance - autoantibodies against neuronal antigens scheme (NMDAR, LGI1 & CASPR2) Sample exchange – multiple lab sample exchange scheme. Results monitored via monthly EQA report and QC meeting. In-house blinded samples run regularly.
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Dalmau et al. An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models. The Lancet Neurology 2019; 18(11): 1045-1057.
Date last updated	Tuesday 25 April 2023

Onconeural antibodies

Hu(D), Yo, CV2/CRMP5, Ri, Ma1, Ma2, amphiphysin,,Tr, SOX1, GAD65, Zic4, titin, recoverin, PKCγ & Purkinje cell antibodies

Paraneoplastic antibodies
Also known as	Onconeural antibodies, Neuronal antibodies Hu(D), Yo, CV2/CRMP5, Ri, Ma1, Ma2, amphiphysin,,Tr, SOX1, GAD65, Zic4, titin, recoverin, PKCγ & Purkinje cell antibodies
Assay Information	Paraneoplastic neurological syndromes (PNS) are a group of neurological disorders associated with malignancy that are not directly caused by either the primary tumour or metastases. An autoimmune process is considered to be the underlying pathophysiological mechanism. Detection of one or more of the well characterized autoantibodies in the presence of paraneoplastic neurological symptoms provides strong diagnostic evidence of a neoplasm, possibly occult. In many cases PNS precedes the detection of the underlying malignancy by up to five years. Paraneoplastic antibodies are antibodies that are associated with certain tumour types and can produce paraneoplastic syndromes, most of which have neurological involvement. Paraneoplastic antibodies are screened using two assays: Immunohistochemistry Serum/CSF samples are applied to sections of fresh rat brain and, following incubation developed using a DAB chromogen. If antibodies present, staining patterns will be dependent on specificity. Recombinant Immunoblot Nitrocellulose strips coated with recombinant Hu(D), Yo, CV2/CRMP5, Ri, Ma1, Ma2, amphiphysin,,Tr, SOX1, GAD65, Zic4, titin, recoverin and PKCg antigens are incubated with patient serum. Specific antibodies in the serum will bind to the antigens whilst non-specific molecules will be removed by washing. Bound antibodies are detected by alkaline phosphate conjugated anti-human IgG using BCIP/NBT as substrate.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF – 0.2 mL
Cost	£25.00
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days
Assay Method	Immunohistochemistry & recombinant Immunoblot
Reference Range & Units	Paraneoplastic antibodies are not normally present.
Factors Affecting Performance of Examination	No known interferences.
Related Tests	GAD65 antibodies
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	NEQAS scheme for paraneoplastic antibodies Institute for Quality Assurance - autoantibodies against neuronal antigens scheme
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	To follow
Date last updated	Wednesday, 18 August 2021

Paraneoplastic antibodies

Hu(D), Yo, CV2/CRMP5, Ri, Ma1, Ma2, amphiphysin,,Tr, SOX1, GAD65, Zic4, titin, recoverin, PKCγ & Purkinje cell antibodies

Paraneoplastic antibodies
Also known as	Onconeural antibodies, Neuronal antibodies Hu(D), Yo, CV2/CRMP5, Ri, Ma1, Ma2, amphiphysin,,Tr, SOX1, GAD65, Zic4, titin, recoverin, PKCγ & Purkinje cell antibodies
Assay Information	Paraneoplastic neurological syndromes (PNS) are a group of neurological disorders associated with malignancy that are not directly caused by either the primary tumour or metastases. An autoimmune process is considered to be the underlying pathophysiological mechanism. Detection of one or more of the well characterized autoantibodies in the presence of paraneoplastic neurological symptoms provides strong diagnostic evidence of a neoplasm, possibly occult. In many cases PNS precedes the detection of the underlying malignancy by up to five years. Paraneoplastic antibodies are antibodies that are associated with certain tumour types and can produce paraneoplastic syndromes, most of which have neurological involvement. Paraneoplastic antibodies are screened using two assays: Immunohistochemistry Serum/CSF samples are applied to sections of fresh rat brain and, following incubation developed using a DAB chromogen. If antibodies present, staining patterns will be dependent on specificity. Recombinant Immunoblot Nitrocellulose strips coated with recombinant Hu(D), Yo, CV2/CRMP5, Ri, Ma1, Ma2, amphiphysin,,Tr, SOX1, GAD65, Zic4, titin, recoverin and PKCg antigens are incubated with patient serum. Specific antibodies in the serum will bind to the antigens whilst non-specific molecules will be removed by washing. Bound antibodies are detected by alkaline phosphate conjugated anti-human IgG using BCIP/NBT as substrate.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF – 0.2 mL
Cost	£25.00
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days
Assay Method	Immunohistochemistry & recombinant Immunoblot
Reference Range & Units	Paraneoplastic antibodies are not normally present.
Factors Affecting Performance of Examination	No known interferences.
Related Tests	GAD65 antibodies
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	NEQAS scheme for paraneoplastic antibodies Institute for Quality Assurance - autoantibodies against neuronal antigens scheme
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Graus F, Vogrif A, Muniz-Castrillo S, Antoine J-C, Desestret V, Dubey D, Giometto B et al. Updated Diagnostic Criteria for Paraneoplastic Neurological Syndromes. Neurol Neuroimmunol Neuroinflamm. July 2021; 8 (4):1-12.
Date last updated	Wednesday, 8 January 2025

Voltage-gated calcium channel (VGCC) antibodies

Voltage-Gated Calcium Channel antibodies ( VGCC)
Assay Information	The Voltage-Gated Calcium Channel autoantibody radioimmunoassay is used for the quantitative determination of P-type VGCC autoantibodies in human serum. Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disease, often associated with small cell lung cancer, in which autoantibodies are directed against voltage-gated calcium channels (VGCCs). The VGCCs can be classified by their electrophysiological characteristics into at least 4 subtypes (T, L, N and P). In the case of LEMS, autoantibodies to P-type VGCCs are most important. VGCC from human muscle is used as the antigen. The VGCCs are complexed with I125-labelled w-conotoxin, which is a neurotoxin produced by cone snails (.marine gastropods of the genus Conus). Conotoxin binds almost irreversibly to calcium channels. Autoantibodies present in the patient’s serum attach to the labelled receptors. The resulting immune complexes are subsequently precipitated with anti IgG. The amount of radioactivity in the precipitate is directly proportional to the concentration of VGCC autoantibodies in the sample.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL Plasma samples not accepted
Cost	£38.00
Transport	First class post
Frequency of Analysis/Turnaround Time	15 working days
Assay Method	Radioimmunoassay
Reference Range & Units	<40 pmol/L Negative 40 – 80 pmol/L Equivocal >80 pmol/L Positive
Factors Affecting Performance of Examination	Gross haemolysis
Related Tests	Paraneoplastic antibodies
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	Blinded sample regularly analysed
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	Motomuraet al. An improved diagnostic assay for Lambert-Eaton myasthenic syndrome. J. Neurol. Neurosurg. Psychiatry (1995) 58:85-87 A. Lennon et al. Calcium-channel antibodies in the Lambert-Eaton syndrome and other paraneoplastic syndromes. N. Engl. J. Med. (1995) 332:1467-1474.
Date last updated	Tuesday, 17 August 2021

Voltage-gated potassium channel (VGKC) associated protein antibodies

LGI1& CASPR2 antibodies
Assay Information	Autoantibodies against neuronal surface antigens are found in patients with autoimmune encephalitis. Some antibodies are directed against voltage-gated potassium channel-associated proteins (VGKC-associated proteins.) Two important epitopes are LGI1 (leucine-rich glioma-inactivated protein 1) and CASPR2 (contactin-associated protein 2). As these antigens play a role in synaptic signal transduction and plasticity, patients often present with seizures and neuropsychiatric symptoms. The resulting conditions include forms of autoimmune limbic encephalitis, neuromyotonia or Morvan’s syndrome. Transfected cells expressing individual neuronal cell-surface antigens are incubated with diluted sample. If specific IgG antibodies to the expressed epitopes are present they will attach to the antigen. In a second incubation the attached antibodies are stained with fluorescein-labelled anti-human antibody and evaluated using a fluorescence microscope.
Specimen Type(s) & Minimum Volume	Serum – 0.2 mL CSF - 0.2mL
Cost	£70.00 Includes LGI1 & CASPR2 antibodies
Transport	First class post
Frequency of Analysis/Turnaround Time	10 working days
Assay Method	Indirect Immunofluorescence
Reference Range & Units	Not Applicable
Factors Affecting Performance of Examination	No interferences known with neuronal antibodies.
Related Tests	Autoimmune Encephalitis panel GABA-B antibodies NMDAR antibodies AMPA1 & AMPA2 antibodies
Accredited Assay	UKAS 8642
External Quality Assurance (EQA)	Institute for Quality Assurance - autoantibodies against neuronal antigens scheme Sample exchange – multiple lab sample exchange scheme. Results monitored via monthly EQA report and QC meeting. In-house blinded samples run regularly.
Further Information	email: wcf-tr.neurobiochemistry@nhs.net Telephone: 0151 5563262
References	van Sonderen A, Schreurs MW, Wirtz PW, Sillevis Smitt PA & Titulaer MJ From VGKC to LGI1 and Caspr2 encephalitis: The evolution of a disease entity over time. Autoimmun Revs 2016; 15(10): 970-974.
Date last updated	Tuesday, 17 August 2021

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