Pattern and Outcome Of Infantile Spasms in Sohag University Hospital :A reterospective study

Background: Infantile spasms (IS) are a unique form of seizure disorder that their occurrence is almost entirely limited to infancy (the first year of life) and they are refractory to conventional anticonvulsant drugs. IS usually are associated with developmental retardation or deterioration and a characteristic electroencephalographic (EEG) pattern (hypsarrhythmia) that together form a syndrome . IS involves a sudden, generally bilateral and symmetric contractions of muscles of the neck, trunk, and extremities. Patients with IS can be classified into focal IS and diffuse groups.                                                                    
Objective: Our main objective in this study is to evaluate  the clinical profile, laboratory data, and  outcome of children having infantile spasms in Sohag University Hospital.
Methods: This retrospective study was conducted on 540 patient (mean age  8.4 ± 6.9 months.: ) from December 2015 to November  2016 in neuropediatric outpatient Clinic, Pediatric department at Sohag University Hospital. Detailed clinical data of the patient, Investigation done for the patient including the neuroimaging (MRI or CT) , EEG pattern, basic metabolic studies , serum enzymes and  Karyotyping were retrospectively collected from files and records .
Results: In this study ; ( 66.1%) of cases  were males.(33,9% ) of  cases were females ,mean age of presentation (8.4± 6.9 months). Positive consanguinity was found in (7.6%) of cases.  (79.2%)of  cases presented with  flexor spasms,  (8.9%)  with  extensor spasms and  (11.9%)  with  mixed spasms .   (8%) of  cases were idiopathic,  (13.5%) of  cases were cryptogenic and  (78.5%) of  cases were symptomatic,(15.2%) of cases showed favorable outcome, and  (84.8%) cases showed unfavorable outcome, of them  (11.5%) of casesdeveloped othertype of seizures,  (54.3%) of caseshad mental retardation,  (28.8%) of cases diedand  (5.2%) of cases had autistic features . In univeriate analysis  (symptomatic) etiology (P= <0.001, OR= 4.7) and absence of primary control of infantile spasm (P= <0.001, OR= 18.3), and frequency of spasm (P= <0.017, OR= 1.8) were significantly related to unfavorable outcome. In Multiple variable analysis  primary response to treatment(odd ratio=16.9,P-value=<0.001), etiology(symptomatic)(odd ratio=3.2,P-value=0.002) significantly associated with unfavorable outcome .
Conclusion: . Infantile spasms was common. it occured in males more than females  with ratio of 1.9:1; the majority of cases were in the age range 1-12 months. flexor spasms was more common than extensor, mixed  type of spasms. Symptomatic type was most common than cryptogenic, idiopathic. long-term neurodevelopment outcomes for children with IS had  several factors, including symptomatic etiology, abnormal  development at the time of diagnosis, poor response to initial treatment.  .
Study Limitation: Patient presented less than 2 months or above 2years