Other diseases of the central and peripheral nervous system

Stiff-person syndrome

Stiff-person syndrome (SPS) is a disease of the CNS, which manifests with progressive muscle stiffness, typically in the trunk and extremities, as well as spontaneous or triggered spasms. Up to 80% of patients show a high serum titer and intrathecal synthesis of anti-glutamic acid decarboxylase (GAD) antibodies. Around 5 % of all SPS cases are paraneoplastic and usually associated with antibodies against amphiphysin.

Diseases with demyelination

These diseases are characterised by progressive destruction of the myelin sheath. The demyelinating foci are predominantly in the brain and spinal cord. The loss of myelin impairs neuronal signal transduction, leading to motor, visual and sensory disorders. These encompass neuromyelitis optica spectrum disorders (NMOSD), which affect in particular the optical nerve and the spinal cord. NMOSD is associated with pathogenic antibodies against the CNS water-channel protein aquaporin-4 (AQP-4). Antibodies against myelin oligodendrocyte glycoprotein (MOG) occur in around 20 % of AQP-4-negative NMOSD patients. Antibodies against MOG also occur in other demyelinating diseases of the CNS, e.g. acute demyelinating encephalomyelitis (ADEM). The determination of anti-AQP-4 and anti-MOG antibodies enables early delimitation from multiple sclerosis, the most important differential diagnosis.

Autoimmune neuropathies

The peripheral nervous system can also be the target of autoaggression, affecting nerves, ganglia or myelin sheaths. Manifestations encompass motor paralysis, sensitivity disorders or dysautonomy. Autoantibodies against cell-membrane glycolipids or glycoproteins of neurons or glial cells are diagnostically definitive for many forms of peripheral neuropathy. Antibodies against gangliosides are characteristic markers for Guillain-Barré syndrome and its variants, for example, acute motor axonal neuropathy (GM1/ GM1b/ GD1a/ GalNac-GD1a IgG), Miller-Fisher syndrome (GQ1b/GT1a IgG), and multifocal motor neuropathy (GM1/GD1b/ asialo-GM1 IgM). Further, IgM antibodies against myelin-associated glycoprotein (MAG) typically occur in demyelinating polyneuropathy with monoclonal IgM gammopathy.

Myasthenia syndrome

In myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) the dominant symptom is muscle weakness, which is mainly due to antibody-mediated transmission disorders of the neuromuscular synapses. Antibodies against nicotinic acetylcholine receptors (AChR) are detected in 85 to 90% of patients with generalised MG. Additional reactivities against antigens of the striated muscle (e.g. titin) often occur in connection with neoplasia (thymoma in 15% of all myasthenia cases) and a severe disease course.

Alzheimer's disease

Techniques

Method
Parameter
Substrate
Species
IIFT
antibodies against glutamic acid decarboxylase
(GAD ab control)
IIFT
glutamic acid decarboxylase (GAD) 65 kDa
(stiff-person syndrome)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
ELISA CSF sample buffer
IIFT
brain: grey and white matter,
Purkinje cell cytoplasm (Yo),
Hu and Ri antigen
CV2, Ma, amphiphysin
cerebellum
monkey
IIFT
Neurology Mosaic 1
Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
non-medullated nerves
3 BIOCHIPs per field:
cerebellum
nerves
intestinal tissue

monkey
monkey
monkey
IIFT
Neurology Mosaic 1 EUROPattern
Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
non-medullated nerves
3 BIOCHIPs per field:
cerebellum
nerves
intestinal tissue

monkey
monkey
monkey
IIFT
Neurology Mosaic14
cerebellum antigens
non-medullated nerves
2 BIOCHIPs per field:
cerebellum
non-medullated nerves

monkey
monkey
IIFT
Neurology Mosaic 14 EUROPattern
cerebellum antigens
medullated nerves
2 BIOCHIPs per field
cerebellum
intestinal tissue

monkey
monkey
IIFT
Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
non-medullated nerves
cell nuclei (ANA)
cerebellum
nerves
intestinal tissue
HEp-2 cells
(4 BIOCHIPs per field)
monkey
monkey
monkey
human
IIFT
Neurology Mosaic 17
Yo, Hu, Ri, CV2, Ma, amphiphysin, NMO
cerebrum antigens, NMO
NMO, optic nerve antigens
aquaporin-4
5 BIOCHIPs per field
cerebellum
cerebrum
optic nerve
transfected cells
control transfection

monkey
monkey
monkey
EU 90
EU 90
IIFT
Neurology Mosaic 2
Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
2 BIOCHIPs per field:
cerebellum
nerves

monkey
monkey
EUROLINE
Neuronal Antigens Profile
SOX1, titin
EUROLINE
EUROLINE
Paraneoplastic Neurologic Syndromes - 12 Ag
(amphiphysin, CV2, PNMA2 (Ma-2/Ta), Ri, Yo, Hu,
recoverin, SOX1, titin, Zic4, GAD65, Tr (DNER) separately)
EUROLINE
IIFT
Neurology Mosaic 8
Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
non-medullated nerves
pancreas islets
4 BIOCHIPs per field
cerebellum
nerves
intestinal tissue
pancreas

monkey
monkey
monkey
monkey
IIFT
Neurology Mosaic 8 EUROPattern
Yo, Hu, Ri, CV2, Ma, amphiphysin
medullated nerves
non-medullated nerves
pancreas islets
4 BIOCHIPs per field:
cerebellum
nerves
intestinal tissue
pancreas

monkey
monkey
monkey
monkey
IIFT
medullated nerves
nerves
monkey
adjustment solution
IIFT
antibodies against myelin-associated
glycoprotein (MAG ab control)
IIFT
antibodies against aquaporin-4
(neuromyelitis optica control)
IIFT
NMOSD Screen 1
aquaporin-4 (AQP-4)
Myelin-oligodendrocyte glycoprotein (MOG)
3 BIOCHIPs per field:
transfected cells
transfected cells
control transfection

EU 90
EU 90
EU 90
IIFT
NMOSD Screen 1 EUROPattern
aquaporin-4 (AQP-4)
Myelin-oligodendrocyte glycoprotein (MOG)
3 BIOCHIPs per field:
transfected cells
transfected cells
control transfection

EU 90
EU 90
EU 90
IIFT
aquaporin-4
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IIFT
aquaporin-4 EUROPattern
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
Secondary reagents immunoblot (IgA)
Secondary reagents immunoblot (IgG)

Secondary reagents immunoblot (IgM)
Additional Reagents
EUROLINE (IgE)
EUROLINE
Positive control serum: IgM, human, 50x concentrated
for DL 1130-X M
EUROLINE
Positive control serum: IgG, human, 50x concentrated
for DL 1130-X G
EUROLINE
Gangliosides Profile 1
(GM1, GD1b, GQ1b separately)
EUROLINE
EUROLINE
Gangliosides Profile 2
(GM1, GM2, GM3, GD1a, GD1b, GT1b, GQ1b separately)
EUROLINE
IIFT
antibodies against myelin oligodendrocytes glycoprotein
(MOG CBA control)
IIFT
Myelin-oligodendrocyte glycoprotein (MOG)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IIFT
Myelin-Oligodendrocyte-Glycoprotein (MOG)
EUROPattern
transfected cells
control transfection
EU 90
EU 90
ELISA
acetylcholine receptor
antigen-coated
microplate wells
IIFT
Myasthenia gravis Mosaic 2
adult acetylcholine receptor (AChR-E)
fetal acetylcholine receptor (AChR-G)
Muscle-specific Kinase (MuSK)
4 BIOCHIPs per field:
transfected cells
transfected cells
transfected cells
control transfection

EU 90
EU 90
EU 90
EU 90
IIFT
Anti-Acetylcholine Receptor (AChR) IIFT
adulter Acetylcholinrezeptor (AChR-E)
fetaler Acetylcholinrezeptor (AChR-G)
---
3 BIOCHIPs per field:
transfected cells
transfected cells
control transfection

EU 90
EU 90
EU 90
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