Epilepsia partialis continua is a rare type of brain disorder in which a patient experiences recurrent motor epileptic seizures that are focal (hands and face), and recur every few seconds or minutes for extended periods (days to years). It is sometimes called Kozhevnikovs epilepsia named after Russian psychiatrist Aleksei Yakovlevich Kozhevnikov who first described this type of epilepsy. Signs and symptoms
During these seizures, there is repetitive focal myoclonus or Jacksonian march. After a seizure has subsided, Todds phenomenon may be observed, which includes transient unilateral weakness. Causes
There are numerous causes for this kind of seizure and they differ depending somewhat on the age at which the seizures begin. Epilepsy most often occurs at the extremes of life – in childhood or in very old age – but can develop at any time throughout ones life. Although these seizures are usually due to large, acute brain lesions resulting from strokes in adults and focal cortical inflammatory processes in children (Rasmussens encephalitis), possibly caused by chronic viral infections, edema, or autoimmune processes. They are very medication and therapy-resistant, and the primary therapeutic goal is to stop secondary generalization. There are also many other reasons why these seizures occur. For example, they could be due to genetics, infections, or problems with brain development. Commonly the cause is unknown. Genetic background determines such features as height, eye color, and potential to develop certain diseases like diabetes, but it also determines all the chemicals and structures that make up the brain, therefore playing a role in epilepsia partialis continua.
Pegvisomant, sold under the brand name Somavert, is a growth hormone receptor antagonist used in the treatment of acromegaly. It is primarily used if the pituitary gland tumor causing the acromegaly cannot be controlled with surgery or radiation, and the use of somatostatin analogues is unsuccessful, but is also effective as a monotherapy. It is delivered as a powder that is mixed with water and injected under the skin. Medical uses
Pegvisomant is indicated for the treatment of adults with acromegaly. Side effects
Side effects of pegvisomant include reactions at the injection site, swelling of the limbs, chest pain, hypoglycemia, nausea and hepatitis. Discovery
Pegvisomant was discovered at Ohio University in 1987 by Distinguished Professor John Kopchick and graduate student Wen Chen at the Edison Biotechnology Institute. After completing clinical trials, it was approved for the treatment of acromegaly by the FDA in 2003 and marketed by Pfizer. Structure
Pegvisomant is a protein containing 191 amino acid residues to which several polyethylene glycol polymers have been covalently bound in order to slow clearance from the blood. The protein is a modified version of human growth hormone designed to bind to and block the growth hormone receptor. It is manufactured using genetically modified E. coli bacteria. Mechanism of action
Pegvisomant blocks the action of growth hormone on the growth hormone receptor to reduce the production of IGF-1.