COVID-19 restrictions to remain in place at The Walton Centre

Restrictions remain in place across the NHS in relation to the COVID-19 pandemic. The safety measures in place over the 18 months therefore remain in place at The Walton Centre - and in our other clinic settings within the community – until further notice.

Due to the increased transmission risk posed by the Omicron variant, visiting has been suspended within The Walton Centre except for exceptional circumstances.

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 

 

emailwcf-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 & 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, AMPA2 & 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 

 

External Quality Assurance (EQA) 

 

Sample exchange – multiple lab sample exchange on a quarterly basis (NMDAR, AMPA, GABA-B, LGI1 & CASPR2). 

In-house blinded samples run regularly. 

Further Information 

 

emailwcf-tr.neurobiochemistry@nhs.net 

 

Telephone: 0151 5563262 

 

References 

 

To be updated 

Date last updated 

Tuesday, 17 August 2021 

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 

 

emailwcf-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, AMPA2 & 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 

Accredited Assay 

 

UKAS 8642 

 

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 

 

emailwcf-tr.neurobiochemistry@nhs.net 

 

Telephone: 0151 5563262 

 

References 

 

To be updated 

Date last updated 

Tuesday, 17 August 2021 

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 

 

emailwcf-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 

 

emailwcf-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 

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 AMPA1, AMPA2 & 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 on a quarterly basis (NMDAR, AMPA, GABA-B, LGI1 & CASPR2).  

In-house blinded samples run regularly. 

Further Information 

 

emailwcf-tr.neurobiochemistry@nhs.net 

 

Telephone: 0151 5563262 

 

References 

 

To be updated 

 

Date last updated 

Tuesday, 17 August 2021 

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 

 

emailwcf-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 

 

emailwcf-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 

 

emailwcf-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 

 

£80.00 

 

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 

 

emailwcf-tr.neurobiochemistry@nhs.net 

 

Telephone: 0151 5563262 

 

References 

To follow

Date last updated 

21/09/2021

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 

 

emailwcf-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 

 

emailwcf-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 

21 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 

 

emailwcf-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 

 

emailwcf-tr.neurobiochemistry@nhs.net 

Telephone: 0151 5563262 

 

References 

 

To follow 

Date last updated 

Wednesday, 18 August 2021 

NMDA 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 knnown with neuronal antibodies.

Related Tests

AMPA1 & AMPA2 antibodies

LG1 & CASPR2 antibodies

GABA-B antibodies

Accredited Assay

 

UKAS 8642

 

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

Monday, 06 September 2021

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 

 

emailwcf-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 

 

emailwcf-tr.neurobiochemistry@nhs.net 

Telephone: 0151 5563262 

 

References 

 

To follow 

Date last updated 

Wednesday, 18 August 2021 

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 

Cost 

 

£38.00 

 

Transport 

 

First class post 

 

 

Frequency of Analysis/Turnaround Time 

21 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 

 

emailwcf-tr.neurobiochemistry@nhs.net 

Telephone: 0151 5563262 

 

References 

 

  1. Motomuraet al. An improved diagnostic assay for Lambert-Eaton myasthenic syndrome. J. Neurol. Neurosurg. Psychiatry (1995) 58:85-87 
  2. 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 

 

emailwcf-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 

 

Page last updated: 22 September 2021