What is VGKC antibody syndrome?
Background: VGKC encephalitis is a recently recognized autoimmune condition with antibodies against components of the VGKC protein complex. Clinical presentation can vary, but patients typically present with limbic encephalitis involving cognitive impairment, seizures and psychiatric symptoms.
What is Morvan’s 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.
What are some neurological Channelopathies associated with voltage dependent K+ channels?
These include acquired neuromyotonia, episodic ataxia type‐1, hereditary deafness syndromes, benign familial neonatal convulsions and hypokalaemic periodic paralysis….Abstract
- potassium.
- nervous system disorders.
- ataxia.
- hypokalemic periodic paralysis.
- isaacs syndrome.
- potassium channel.
- genetics.
- congenital deafness.
What are voltage gated potassium channel antibodies?
Voltage-gated potassium channel (VGKC)-complex antibodies are defined by the radioimmunoprecipitation of Kv1 potassium channel subunits from brain tissue extracts and were initially discovered in patients with peripheral nerve hyperexcitability (PNH).
What is the role of voltage gated potassium channels?
Voltage-gated potassium ion channels (Kv) play an important role in a variety of cellular processes, including the functioning of excitable cells, regulation of apoptosis, cell growth and differentiation, the release of neurotransmitters and hormones, maintenance of cardiac activity, etc.
What causes VGKC?
Anti-VGKC-complex encephalitis are caused by antibodies against the voltage gated potassium channel-complex (VGKC-complex) and are implicated in several autoimmune conditions including limbic encephalitis, epilepsy and neuromyotonia (i.e. Isaacs’ Syndrome).
What causes Isaacs syndrome?
The exact cause of Isaacs’ syndrome is poorly understood. There appear to be hereditary and acquired (non- inherited ) forms of the condition. The acquired forms are often associated with malignancies, peripheral neuropathies , and a variety of autoimmune disorders of the nervous system.
How is Morvans syndrome diagnosed?
A high index of clinical suspicion is needed to diagnose Morvan syndrome when a patient presents with a combination of above mentioned diverse clinical features. Most of the investigations that are usually performed in such patients, such as CSF analysis, brain MRI, EEG, PET scan, are typically unyielding.
Is episodic ataxia hereditary?
People who have EA experience episodes of poor coordination and/or balance (ataxia) which can last from several seconds to several hours. There are at least eight recognized types of EA. All are hereditary, though different types are associated with different genetic causes, ages of onset, and symptoms.
How is Channelopathy diagnosed?
Diagnosis of Cardiac Channelopathies (ECG) is used to make the diagnosis. But sometimes the pattern of ECG abnormalities is less clear. In such cases, doctors may try to provoke the heart rhythm disturbance with a drug or with exercise, enabling doctors to make a diagnosis.
What happens when voltage-gated potassium channels open?
A set of voltage-gated potassium channels open, allowing potassium to rush out of the cell down its electrochemical gradient. These events rapidly decrease the membrane potential, bringing it back towards its normal resting state.
What is the role of the voltage-gated potassium channels?
Voltage-gated potassium channels (VGKC) are transmembrane channels responsible for returning the depolarized cell to a resting state after each nerve impulse. They are, therefore, important in modulating neuronal excitability in the CNS and peripheral nervous system.