West syndrome of bad prognosis: Case Report
Main Article Content
Keywords
West syndrome, epileptic encephalopathy, hypsarrhythmia
Abstract
Infant of 5 months old, has irritability, difficulty falling asleep, deviation of the eyes and tonic movement of the shoulder. An electroencephalogram was performed and the hypsarrhythmia pattern was observed. Treatment is started with valproic acid by pediatric neurologist. At 10 years of age, she presented severe thoracolumbar scoliosis, had several hospitalizations for pneumonia, had a gastrostomy due to a complication and died at the age of 13 years due to severe pneumonia. West syndrome is a pediatric epileptic encephalopathy characterized by infantile or epileptic spasms, a hypsarrhythmic pattern in the electroencephalogram and mental retardation or arrest in psychomotor development. It is important to diagnose West Syndrome as soon as possible to avoid cognitive deterioration and sequelae that increase the morbidity and mortality of patients. The focus on neurological studies as well as the panel of epilepsies and metabolic profile is the basis to reach an effective diagnosis and also not delay to start treatment with first-line drugs
References
2. Licourt- Otero D, Travieso- Téllez D. Estrategia de evaluación genética en el síndrome de west. Rev Cien Med. 2013; 17 (1): 63- 72.
3. Tekin- Guveli B, Cokar O, Nortcan N, et al. Long-term outcomes in patients with West syndrome: An outpatient clinical study. Seizure. 2015; 68-71
4. Calderon- Romero M, Arce- Portillo E, Lopez- Lovato M, et al. Síndrome de West criptogénico: perfil clínico, respuesta al tratamiento y factores pronósticos. Serv Neurope. 2017; 1-7
5. Margarita- Lopez I, Varela X. Síndromes epilépticos en niños y adolescentes. Rev Med Clin Mont. 2013; 24 (6): 915- 927.
6. Andia-Berazain C, Sanz- Arrazola H. Impacto del Síndrome de West en pacientes del Hospital del Niño Manuel Ascencio Villarroel. Rev Cient Cienc Med. 2014;17(2): 9-13.
7. Fejerman N. Diagnósticos diferenciales del síndrome de west. Rev Neurol 2013; 57 (1):125-8.
8. Pozo-Lauzan D, Pozo Alonso A, Sayu- Stewart J. El electroencefalograma en el síndrome de West y otras entidades clínicas relacionadas. Rev Cub Pedi. 2015; 87(3):362-370.