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2010, Number 1

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Rev Med MD 2010; 1.2 (1)

West syndrome

Martínez-Quezada DI

Language: Spanish
References: 22
Page: 10-14
PDF size: 565.61 Kb.


ABSTRACT

The syndrome of infantile spasms with hypsarrhythmia, also known as West Syndrome (WS), is described as a serious and infrequent pediatric epileptic encephalopathy that is characterized by three main findings: epileptic spasms, psychomotor developmental delay, and hypsarrhythmia pattern in the electroencephalogram (EEG). This particular pediatric syndrome is classified as symptomatic and cryptogenic by the International League Against Epilepsy (ILAE). The symptomatic presentation is divided by etiology in three main groups according to the period of origen: prenatal, perinatal or postnatal. Its pathophysiology is unknown and a number of hypotheses have been postulated for its explanation. Diseases such as benign mioclonic child's disease, gastroesofagic reflux disease, spastic opistotonic posture and others can be confused also with WS. WS its difficult to manage and control, because it does not respond to conventional antiepileptic therapy. Nonetheless, there are three lines of pharmacological treatment, even so there is the necessity of surgery certain kind of patients who do not respond. The general prognosis of WS is not favorable.


REFERENCES

  1. Kossoff Eric H. Infantile Spasms. The Neurologist 2010; 16: 69–75.

  2. West WJ. On a peculiar form of infantile convulsions. Lancet 1841; 1:724–725.

  3. Gibbs FA, Gibbs EL. Atlas of electroencephalography, volume 2. Cambridge (MA): Addison-Wesley, 1952.

  4. Albia J, Pozo A, Desiderio-Pozo L, Desi-Pozo A. Síndrome de West: etiología, fisiopatología, aspectos clínicos y pronósticos. Rev Cubana Pediatr 2002; 74(2):151-161.

  5. Stafstrom CE. Infantile spasms: a critical review of emerging animal models. Epilepsy Curr 2009; 9:75-81.

  6. Engel J Jr. ILAE Commission Report. A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: Report of the ILAE Task Force on Classification and Terminology. Epilepsia 2001; 42:1-8.

  7. Caraballo R, Cersósimo R, Arroyo H, Fejerman N. Síndrome de West sintomático: asociaciones etiológicas particulares con respuesta inesperada al tratamiento. Rev Neurol 1998; 26: 372-375.

  8. Appleton RE. Infantile spasms. Arch Dis Child 1993; 69:614-618.

  9. Adams RD, Victor M. Normal development and deviations in development of the th nervous system. En Adams RD, Victor M. eds. Principles of Neurology, 7 ed. New York, EUA. Mc Graw-Hill, 2001:319.

  10. Hrachovy RA, Frost JD Jr, Kellaway P. Sleep characteristics in infantile spasms. Neurology 1981; 31:688-694.

  11. Hrachovy RA, Frost JD Jr. Infantile spasms: a disorder of the developing nervous system. En Kellaway P, Noebels JL, eds. Problems and concepts in developmental neurophysiology. Baltimore, EUA. Johns University Press, 1989:131-147.

  12. Chugani HT, Shewmon DA, Sankar R. Infantile spasms: II. Lenticular nuclei and brainstem activation on positrom emission tomography. Ann Neurol 1992; 31:212-219.

  13. Chugani HT, Shields WD, Shewmon DA, Olson DM, Phelps ME, Peacock WJ. Infantile spasms: I. PET identifies focal cortical dysgenesis in cryptogenic cases for surgical treatment. Ann Neurol 1990; 27:406-413.

  14. Hrachovy RA, Frost JD, Pollack M, Glaze DG. Serologic HLA typing in infantile spasms. Epilepsia 1987; 28:613-617.

  15. Liu ZS, Wang QW, Wang FL, Yang LZ. Serum cytokine levels are altered in patients with West syndrome Brain Dev 2001; 23:548-551.

  16. Ying-Xue D, Li-Ping Z, Bing H, Wei-Hua Y, Zhan-Li L, Dai Z. ACTH receptor (MC2R) promoter variants associated with infantile spasms modulate MC2R expression and responsiveness to ACTH. Pharmacogenetics and Genomics 2010; 20:71–76.

  17. Lacy JR, Penry JK. Infantile spasms. New York, EUA. Raven Press, 1976.

  18. Lombroso CT. A prospective study of infantile spasms: clinical and therapeutic correlations. Epilepsia 1983; 24:135-158.

  19. Hancock E, Osborne J, Milner P. Treatment of infantile spams (Cochrane Review). Cochrane Library, Issue 3,2003.

  20. Li-Ping Z, Qing L, Jiong Q, Fang-Cheng C, Zhi-Sheng L, Eilhard M. Evaluation of pen-label topiramate as primary or adjunctive therapy in infantile spasms. Clin Neuropharmacol 2008; 31: 86-92.

  21. Jonas R, Asarnow RF, et al. Surgery for symptomatic infant-onset epileptic encephalopathy with and without infantile spasms. Neurology 2005; 64:746-750.

  22. Bureau M, Dravet C, Genton P, Tassinari CA, eds. Les syndromes épileptiques de l´enfant et de l´adolescent, 4° ed. London, UK. John Libbey, 2005:76-89.




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