infantile spasm sentence in Hindi
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- Generalized seizures occur in various seizure syndromes, including myoclonic epilepsy, familial neonatal convulsions, childhood absence epilepsy, absence epilepsy, infantile spasms ( West's syndrome ), Juvenile Myoclonic Epilepsy and Lennox-Gastaut syndrome.
- Infantile spasms, a leading cause of epilepsy in children and a condition that often results in retardation and physical impairment, affect about 3, 000 infants in the United States each year.
- West syndrome in modern usage is the triad of infantile spasms, a pathognomonic EEG pattern ( called hypsarrhythmia ), and developmental regression-although the international definition requires only two out of these three elements.
- At one time, mutations in the CDKL5 gene were said to cause a disorder called X-linked infantile spasm syndrome ( ISSX ) or West syndrome . but this research established CDKL5 disorder as a distinct clinical entity.
- Doctors specializing in treating infantile spasms, a potentially devastating seizure condition sometimes known as West's syndrome, have expressed concern for several weeks that supplies of the drug are dwindling rapidly because the manufacturer unexpectedly ceased production before the first of the year.
- "We'll be glad to have ACTH available again, " said Dr . Wendy G . Mitchell, a pediatric neurologist at Children's Hospital in Los Angeles, who is a specialist in infantile spasms, " This is good news for a lot of little children ."
- The name of William West is best known for the eponymous syndrome, even though the convention on the use of this name is relatively recent and dates back to 1960, when H . Gastaut organized the " 9th Colloque de Marseille " ( Marseille Colloquium ); which focused on infantile spasms.
- The methods of this meeting were published in the book " L'enc�phalopathie Myoclonique Infantile avec Hypsarythmie " ( infantile myoclonic encephalopathy with hypsarrhythmia ) and was at that conference that H . Gastaut suggested the use of West syndrome as an eponym for infantile spasms; which is widely used for the syndrome to date.
- Progress in genome and exome sequencing is revealing that some individuals diagnosed with Lennox Gastaut Syndrome have de novo mutations in a variety of genes, including CHD2, GABRB3, ALG13 and SCN2A The Epi4K study consortium ( 2013 ) observed de novo mutations in at least 15 % of a study cohort of 165 patients with LGS and Infantile Spasms using whole exome sequencing.
- In a 2012 article in the " New York Times, " journalist Andrew Pollack described how Don M . Bailey, a mechanical engineer by training who became interim president of Questcor Pharmaceuticals, Inc . ( Questcor ) in May 2007, initiated a new pricing model for Acthar in August 2007 when it was classified by FDA as an orphan drug and a specialty drug to treat infantile spasms.
- The drug is indicated as monotherapy for pediatric patients one month to two years of age with infantile spasms for whom the potential benefits outweigh the potential risk of vision loss, and as adjunctive ( add-on ) therapy for adult patients with refractory complex partial seizures ( CPS ) who have inadequately responded to several alternative treatments and for whom the potential benefits outweigh the risk of vision loss.
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