Syndrome de west pdf

West syndrome ws is one of the catastrophic epileptic syndromes in infancy characterized by a triad of infantile spasms, psychomotor deterioration and hypsarrhythmic eeg pattern. West syndrome is a type of epilepsy that develops within the first year of life and typically between four to eight months old. The infantile spasms usually begin in the first year of life, typically between 48 months. The xlinked form of west syndrome affects males more often than females. The international league against epilepsy commission classifies the aetiologies of ws into genetic, structural, metabolic and unknown. Abstract the west syndrome is of secondary generaled, with occur within the first year of life and interrupt psychomotor unrolled. The lennoxgastaut syndrome, niedermeyer e, degen r eds, alan r liss, new york 1988. West syndrome is a type of epilepsy that affects babies. It is named after the english physician, william james west 17931848, who first described it in an article published in the lancet in 1841. West syndrome is an agedependent epileptic encephalopathy characterized by electoclinical triad of epileptic spasms, retardation of psychomotor development and electroencephalographic pattern of. West syndrome symptoms, treatment, life expectancy.

West syndrome accounts for approximately 30 percent of all cases of epilepsy affecting infants. West syndrome is a severe epilepsy syndrome composed of the triad of infantile spasms, an interictal electroencephalogram eeg pattern termed hypsarrhythmia, and mental retardation, although the diagnosis can be made even if 1 of the 3 elements is missing according to the international classification. West syndrome ws, also known as infantile spasms, occurs in infancy with a peak between 4 and 7 months. Many children with west syndrome will have moderate to severe learning. West syndrome is rare condition with three distinct characteristics or symptoms, infantile spasms, jackknife seizure, abnormal brain waves, and mental retardation that appear between 3 and 12 months of age. This term denotes the concept that the epileptic activity itself might directly contribute additional cognitive and behavioral impairments. The original case actually described his own son, james edwin west 18401860. West syndrome is characterized by a specific type of seizure infantile spasms seen in infancy and childhood. The goal was to report a case of west syndrome in an ecuadorian patient.

Down syndrome program massachusetts general hospital. We analyzed the frequency and risk factors present in patients diagnosed with west syndrome. Lennoxgastaut syndrome and its relationship to infantile spasms west syndrome. In most of the children, the condition of west syndrome arises in the first year of life. Lennoxgastaut syndrome lgs is a complex, rare, and severe childhoodonset epilepsy. West syndrome is characterized by the onset of epileptic spasms, typically in the first year of life. Global developmental impairment with or without regression is typically seen. Evan six months infantile spasms west syndrome youtube. Spasms, neurodevelopmental regression and hypsarrhythmia on electroencephalogram eeg basically define ws. With predominance in sex masculine, on proportion 2. Epileptic spasms, is an uncommontorare epileptic disorder in infants, children and adults.

Enter through leftmost set of doors with no security desk and take elevators to 2nd floor. The effective treatment of west syndrome still poses a great challenge in pediatric neurology. West syndrome genetic and rare diseases information. Children with west syndrome experience seizures, the child will suddenly jerk and then their muscles become stiff resulting in them bending forward with elevated arms or legs. Ws is commonly associated with poor longterm outcome, especially in symptomatic cases, with development of other seizure types, impaired cognitive and. The life expectancy for west syndrome depends upon the cause and severity of the symptoms. Diagnosis criteria definition lgs belongs to the group of severe infantile epileptic syndromes epileptic neonatal encephalopathy with suppressionburst, west syndrome, severe myoclonic epilepsy of infancy, which represent the most distressing epileptic encephalopathies of infancy. It is characterized by multiple and concurrent seizure types, cognitive dysfunction, and slow spike waves on electroencephalogram eeg. Other names for it are generalized flexion epilepsy, infantile epileptic. Prognosis the long term prognosis is poor in the majority, however early treatment can improve prognosis. In the overall long term view, the majority of patients. West syndrome is a rare neurological syndrome that can affect males and females.