What is voltage gated potassium channel antibody syndrome?
Voltage-gated potassium channel antibody encephalopathy, a rare cause of limbic encephalopathy, typically presents with memory impairment and seizures. Psychiatric symptoms have not been emphasised in the literature.
What antibodies are autoimmune encephalitis?
Introduction. Autoimmune encephalitis (AE) is an often rapidly progressive inflammatory neurological disease with subacute onset. The best characterized and most common form of AE is anti-NMDA receptor (NMDAR) encephalitis, defined by cerebrospinal fluid (CSF) IgG antibodies targeting the NMDA type glutamate receptor.
What is VGKC antibody?
Voltage gated potassium channel antibodies (VGKC Abs) are known to cause three rare neurological syndromes- neuromyotonia, Morvan’s syndrome and limbic encephalitis although an increasing array of other associated neurological symptoms are becoming recognised.
What is Morvan syndrome?
Morvan syndrome (MoS) is a rare autoimmune syndrome associated with antibodies against two kinds of potassium channel proteins, contactin associated protein-like 2 (CASPR2) and leucine-rich glioma inactivated protein 1 (LGI1). MoS patients with only LGI1-antibody seropositivity have rarely been reported.
Can autoimmune limbic encephalitis be cured?
“Autoimmune encephalitis is a treatable disease. But some cases end up being very complex,” Dr. Day says. “We see patients who have spent an inordinate amount of time in the hospital and even the intensive care unit due to the many complications of this disease.”
How common is Morvan’s syndrome?
There are only about 14 reported cases of Morvan’s syndrome in the English literature. With only a limited number of reported cases, the complete spectrum of the central nervous system (CNS) symptomatology has not been well established. The natural history of Morvan’s is highly variable.
What bacteria causes encephalitis?
Most diagnosed cases of encephalitis in the United States are caused by herpes simplex virus types 1 and 2, arboviruses (such as West Nile Virus), which are transmitted from infected animals to humans through the bite of an infected tick, mosquito, or other blood-sucking insect, or enteroviruses.
What virus causes encephalitis?
Encephalitis is most often due to a virus, such as:
- herpes simplex viruses, which cause cold sores (this is the most common cause of encephalitis)
- the varicella zoster virus, which causes chickenpox and shingles.
- measles, mumps and rubella viruses.
Does autoimmune encephalitis go away?
“They told us autoimmune encephalitis never goes away completely,” Chris says, “but once you get past two or three years from onset, you’re less likely to relapse.”
What is voltage gated potassium channel antibody encephalitis?
Voltage gated potassium channel (VGKC) antibody encephalitis. Voltage gated potassium channel (VGKC) antibody encephalitis is an autoimmune encephalitis with antibodies against the voltage gated potassium channel. It is one of the most common forms of autoimmune limbic encephalitis in the absence of primary extra-CNS tumours.
What is VGKC antibody encephalitis?
Voltage-gatedpotassium channel (VGKC) antibody encephalitis describes a small group of autoimmune encephalitides with antibodies against components of the voltage-gated potassium channel. It is one of the most common forms of autoimmune limbic encephalitis in the absence of primary extra-CNS tumors.
What is the pathophysiology of autoimmune encephalitis?
Pathophysiology Patients with autoimmune encephalitis have antibodies towards synaptic proteins with neuronal functions. The antibodies are presumed to be disease causing, just like the acetylcholine receptor antibodies in myasthenia gravis. The voltage-gated potassium channel (VGKC), like other ion channels, belong to a multiprotein complex.
Are VGKC antibodies targeted against the channel itself?
Antibodies directed against VGKC were first reported in neuromyotonia. Further studies led to VGKC antibodies being recognized in Morvan’s syndrome and limbic encephalitis as well. It was for many years erroneously presumed that antibodies detected in a VGKC assay were targeted against the channel itself.