What is CASPR2 antibody?

CASPR2 (Anti-contactin-associated protein-like 2) antibody is a neural specific antibody associated with varied autoimmune neurological disorders including autoimmune encephalitis and autoimmune epilepsy. CASPR2 is a cell adhesion molecule expressed in both central and peripheral nervous system.

What is CASPR2 IgG?

The presence of CASPR2 IgG antibody is associated with a wide spectrum of clinical manifestations, including acquired neuromyotonia, limbic encephalitis, painful neuropathy, and Morvan syndrome. Tumors such as thymoma, small cell lung cancer, and other rarer tumors may occur.

What is Rasmussen disease?

General Discussion. Rasmussen encephalitis, sometimes referred to as Rasmussen syndrome, is a rare disorder of the central nervous system characterized by chronic progressive inflammation (encephalitis) of one cerebral hemisphere.

What is peripheral nerve hyperexcitability?

Peripheral nerve hyperexcitability (PNH) is a syndrome of spontaneous and continuous muscle fiber activity, cramps, and slow relaxation of muscles of peripheral nerve origin. 1,2. The electrophysiologic basis consists of spontaneous bursts of single motor unit discharges with a high intraburst frequency.

What is VGKC blood test?

This assay detects autoantibodies to the voltage-gated potassium channel complex antibody. Voltage gated potassium channel (VGKC) antibodies are found in some cases of acquired neuromyotonia, Morvan’s syndrome and voltage-gated potassium channel antibody-related limbic encephalitis.

What is the difference between Myokymia and Fasciculations?

Myokymia is differentiated from benign fasciculations by electromyographic features of regular groups of motor unit discharges, especially doublets and triplets, occurring with a regular rhythmic discharge (Denny-Brown and Foley, 1948; Fahn and Jankovic, 2007a).

How is Morvan’s 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.

How is Rasmussen syndrome diagnosed?

Rasmussen encephalitis may be diagnosed clinically based upon a thorough clinical evaluation, a detailed patient history, and a complete neurological evaluation including advanced techniques such as electroencephalography (EEG), and magnetic resonance imaging (MRI).

What is Rasmussen syndrome treatment?

Because Rasmussen syndrome is difficult or impossible to control with medication, in many cases, the most effective treatment option is surgery, including hemispherectomy.