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.

Selected Products

Method	Parameter	Substrate	Species
IIFT	glutamic acid decarboxylase (GAD) 65 kDa (stiff-person syndrome)	transfected cells control transfection (2 BIOCHIPs per field)	EU 90 EU 90	Details
IIFT	brain: grey and white matter, Purkinje cell cytoplasm (Yo), Hu and Ri antigen CV2, Ma, amphiphysin	cerebellum	monkey	Details
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	Details
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	Details
IIFT	Neurology Mosaic14 cerebellum antigens non-medullated nerves	2 BIOCHIPs per field: cerebellum non-medullated nerves	monkey monkey	Details
IIFT	Neurology Mosaic 14 EUROPattern cerebellum antigens medullated nerves	2 BIOCHIPs per field cerebellum intestinal tissue	monkey monkey	Details
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	Details
IIFT	Neurology Mosaic 2 Yo, Hu, Ri, CV2, Ma, amphiphysin medullated nerves	2 BIOCHIPs per field: cerebellum nerves	monkey monkey	Details
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	Details
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	Details
IIFT	medullated nerves	nerves	monkey	Details
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	Details
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	Details
IIFT	aquaporin-4 EUROPattern	transfected cells control transfection (2 BIOCHIPs per field)	EU 90 EU 90	Details
IIFT	aquaporin-4	transfected cells control transfection (2 BIOCHIPs per field)	EU 90 EU 90	Details
EUROLINE	Gangliosides Profile 1 (GM1, GD1b, GQ1b separately)	EUROLINE		Details
EUROLINE	Gangliosides Profile 2 (GM1, GM2, GM3, GD1a, GD1b, GT1b, GQ1b separately)	EUROLINE		Details
IIFT	Myelin-Oligodendrocyte-Glycoprotein (MOG) EUROPattern	transfected cells control transfection	EU 90 EU 90	Details
IIFT	Myelin-oligodendrocyte glycoprotein (MOG)	transfected cells control transfection (2 BIOCHIPs per field)	EU 90 EU 90	Details
ELISA	acetylcholine receptor	antigen-coated microplate wells		Details
RIA	acetylcholine receptor (ACHR) precipitation	labelled antigen		Details
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	Details
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	Details

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