| Objective: This study was aimed to provide evidences for diagnosis and treatment of West syndrome, through retrospective analysis of patients with West syndrome, including characteristics of spasms, EEG and Brain imaging, etiologies, treatment and factors influencing short-term effectiveness.Methods: A retrospective analysis was performed for West syndrome.175 cases were collected in the department of Neurology from January 1, 2010 to December 31, 2015, inChildren Hospital of Chongqing Medical University. The clinical characteristics, etiologies, Short-term effectiveness of treatment with glucocorticoid and ACTH, and factors of influencing short-term effectiveness were studied.Results:1. In 175 cases with West syndrome, male/female ratio was 2.07:1. The mean age of onset was 5.9±3.57 months, the peak age was 3 to 6 months. The early-onset West syndrome and late-onset West syndrome is comparatively less common(16.0%, 7.4%).2. The onset seizure type was infantile spasmsin 142 cases(81.1%), other seizure type could be seen in 33cases(18.9%),included partial seizures in 12 cases(6.9%), generalized seizures in 11 cases(6.3%), and unconfirmed type in 10 cases(5.7%).3. Clusters of infantile spasms was one of the features of West syndrome. The forms of infantile spasms included flexor type(83.4%), extensor type(1.1%),and mixed type(15.4%); Symmetric spasms(93.1%), asymmetric spasms(6.9%); 27 cases(15.4%) had isolated infantile spasms. There were other seizure type in 25 cases(14.3%)after the onset of infantile spasms.4. Developmental delay before infantile spasms were found in 142 cases(81.1%). Developmental regression were found in 29 cases who had normal development before onset of West syndrome.5.The interictal EEG showed typical hypsarrhythmia pattern in 105 cases(60%), and hypsarrhythmia variant pattern in 70 cases(40%).6. Positive rate of Brain MRI were 72.4%, and abnormal findings mainly included: periventricular leukomalacia(12.9%), sequela changes due to ischemic-hypoxic encephalophathy(4.3%), brain atrophy(10.4%), retardation of myelination(10.4%), congenital brain malformation(8.6%), and Tuberous sclerosis(5.5%).7. The etiologies of 175 cases with West syndrome included:genetic etiology in 7 cases(chromosomal disorders in 3 cases, and gene mutation in 4 patients), structural etiology in 109 cases, metabolic etiology in 1 cases, infectious etiology in 4 cases, and unknown in 60 cases. Perinatal brain injury was the main symptomatic etiology(28.0%). There were no significant differences among different etiologies in the clinical spectrum and the interictal EEG(P>0.05).8.Short-term effectiveness of treatment with dexamethasone and sequential prednisoneis was 73.5%, and there was no significant difference between the high dose group and the small dose group with dexamethasone(75.5% vs 72.1%, P > 0.05).There was no significant difference between prednisone and ACTH in the short-term effectiveness(37.6% vs 33.4%, P > 0.05). Other seizure type before infantile spasms was the risk factor for Short-term effectiveness of treatment.8.There was no significant difference between the two treatment groups(glucocorticoid and ACTH) in infantile spasms remission rate and in the improvement of EEG.10. Follow-up of 20 cases with West syndrome,there were 3 patients who had other seizure type after spasms free, and 18 patients who had developmental delay.Conclusions: West syndrome in which infantile spasms are associated with hypsarrhythmia and developmental regression, is a common epileptic encephalopathy in infants, and the peak age of onsetis 3~6 months old. Brain MRI, Metabolic screening and epileptic encephalopathy genes screening will be helpful to find the etiology. Glucocorticoid or ACTH could be used in West syndrome, and there is no significant difference between Glucocorticoid and ACTH in infantile spasms remission rate and in the improvement of EEG. |
PDF Full Text Request