| Infancy infantile spasms are the most common age-dependent epileptic encephalopathy, the incidence rate of 100,000 live births, 24 infants suffering from one of the 42 patients, the incidence is higher than the baby boy of the reportPathogenesis of infantile spasms: 1,5-HT in the cortex of the channel due to the continuous epileptogenic foci and activation status in hyperthyroidism, which increase cortical excitability and seizures. 2,brainstem hypothesis. 3,anti-brain antibodies or anti-presynaptic membrane receptor of acetylcholine (Ach 1 R) role, by influencing the secretion of acetylcholine, or degeneration, such as acetylcholine receptor mechanism lead to seizures. 4,brain cell membrane ion channels or barriers to gene mutation, caused by reduction of Na-K-ATP enzyme, sodium calcium, brain tissue depolarization, increased excitability, kindling effect occurred due to seizure. 5,the immune dysfunction. 6,Genetic Background: Research has shown that the incidence of infantile spasms and genetic relationship must have.Changes in EEG characteristics: one, the typical high wave dysrhythmia: EEG of the lead appears chaotic 1 ~ 3Hz high-amplitude slow-wave background, the outbreak of paroxysmal high amplitude spikes, sharp waves, spikes ( Tsim Sha Tsui) the slow wave, slow spike General affect many spines rhythm, manifested as asymmetry, non-synchronous. 2, a high degree of rhythm disorder variant form: This form with a typical waveform is different from the outbreak of asymmetry can be seen in addition to non-synchronous high-amplitude epileptiform discharges can also occur simultaneously diffuse slow spikes and sharp slow-wave General General. 3, the asymmetry (or local): This type is characterized by two hemispheres, or the side of symmetric volatility has always been abnormal for the high-amplitude slow-wave-based non-synchronous, there is a small amount of inter-spikes, sharp waves or spike wave General General and the sharp slow-wave. ACTH regulation and control, mainly through a brainstem raphe raphe and locus coeruleus of a channel in hippocampal 5-HT neurons glucocorticoid receptor to control steroid IS. Studies have shown that, IS and immune-related disorders. Gamma globulin by regulating the humoral immunity to control the IS. Topiramate block mainly through voltage-dependent sodium ion channels, GABA-A receptor to enhance GABA activity Department, block kainic acid receptor plays the role of anticonvulsant. Vitamin B6 has a strong excitatory and inhibitory receptor blockers ganglion synaptic transmission and thus play a role in anticonvulsant. Thus, we have adopted a low-dose hormone (ACTH + prednisone), topiramate, gamma globulin, vitamin B6 four drug combination therapy in children with infantile spasms to the incidence of early and effective control of their spastic attack.In order to observe the four-drug combination therapy of infantile spasms and clinical efficacy of dynamic EEG changes before and after treatment and side effects of humoral immunity, such as aspects of clinical research. Selected in our hospital from January 2001 to January 2008 a clear diagnosis of 61 cases of infantile spasms in children as research subjects, divided into 30 cases of treatment group, 31 cases of the control group, while the treatment group divided by the cause of 14 cases of symptomatic, 16 cases of cryptogenic. All 36 cases of male children, female 25 cases, aged 3 months to 3 years old, about 90% of children aged less than 12 months, the peak age of onset for the 4 June. Seizure of all children with the form of 73% for flexion, 12% for the stretching-type, 15% were mixed. All children were pre-treatment for dynamic inspection and routine EEG, biochemistry, four of humoral immunity and neuroimaging examinations. 2, each group use the program: the treatment group, control group, ACTH ,25-40IU (Shanghai No. 1 Biochemical Pharmaceutical Co., Ltd.) infusion, convulsion static control point after two weeks, reducing to 15IU / d static point 2 weeks after the disabled, changed to oral prednisolone, starting dose for 1-2mg/kg, every 1-2 weeks to reduce, by three months after; topiramate (Xi'an Janssen Pharmaceutical Co., Ltd.) orally, starting before a dose of 1mg/kg, every 1-3 days under the condition increases the amount of each increase in dose of 1mg/kg, up to 10mg/kg. Vitamin B6100mg-150mg / d, static point of 2 weeks. The total amount of gamma globulin 2g/kg, static point at 3-5 days per month 1 resting point for a course of treatment, a total of six courses. Control group: de Golden Syrup (Hangzhou livelihood Sanofi-Aventis Pharmaceutical Co., Ltd.) oral, 20mg.kg-1.d-1, sub-2 oral, 1 week after taking the author made, gradually increase the volume of 30 -40mg.kg-1.d-1 and detection of blood plasma concentration of valproic acid to maintain the effective blood concentration of 50-100 units / liter. Initial amount of clonazepam 0.03mg.kg-1.d-1, gradually increased to 0.2mg.kg-1.d-1, sub-3 oral, 2 weeks after the author has yet to control the fat, adjust the dose. 3, after the end of the treatment of clinical indicators to assess: 1) to observe the spasm attack control; 2,) for 4-6 weeks after the review AEEG; 3) medication after 5 weeks SRID determination of serum IgA, IgM , IgG, C3. 4) monitoring of blood pressure 2 times a day. And to monitor the blood, biochemical changes, adverse drug reactions observed.In the treatment group and control group, the study results showed: the treatment group 27 cases of complete control of seizures (90.0%) were cured and 2 cases (6.7%), ineffective in 1 case (3.3%); control group, 6 cases of complete control of seizures (19.4% ), effective in 4 cases (12.9%), effective in 8 cases (25.8%), 13 cases of invalid (41.9%). Treatment group and control group full control rate (27:6) P were significantly different; the treatment group and control group efficiency (29:18) P were significantly different. 2, the treatment group to change the normal EEG in 18 cases (60.0%), marked improvement in nine cases (30.0%), mild improvement in two cases (6.7%), no improvement in 1 case (3.3%). The normal control group 1 cases (3.2%), marked improvement in 6 cases (19.4%), mild improvement in 10 cases (32.3%), no improvement in 14 cases (45.1%). The treatment group and normal control group rate (18:1) P were significantly different; the treatment group and control group compared with mild improvement (9:6) P was no significant difference. 3, the treatment group and control group before and after treatment in children with blood, calcium, phosphorus, alkaline phosphatase and other indicators no significant difference. 4, 5 weeks after treatment showed the determination of humoral immunity: the treatment group IgG, IgM and IgA levels increased in the control group with no change in pre-treatment, two sets of numerical comparison testing, a significant difference (P <0. 01); the treatment group with the control group compared complement C3, there was no significant difference (P> 0.05).Treatment group, three cases of fever in children with cough (4.9%), nine cases of children with nocturnal restlessness (14.7%), to the anti-infection after oral calcium and vitamin disappeared. Three cases appears low heat, no sweat (4.9%), two weeks after the administration of these symptoms disappeared. 1 case of hypertension (3.3%) up to 155/100mmHg, blood pressure after oral compound hypotensive tablet, blood pressure returned to normal, disable antihypertensive agents. Emergence of three cases of hyperlipidemia and hypokalemia (4.9%), potassium 2.8-3.2mmol / L. 1 cases (3.3%) there trembling, disable ACTH, trihexyphenidyl hydrochloride oral symptoms. The above does not apply, after the disappearance of symptomatic treatment. VPA in the control group as the main antiepileptic drugs (if the poor can increase efficacy of clonazepam with adjuvant therapy), such as nausea and vomiting 3 cases of gastrointestinal reactions (4.9%), the three cases after the symptomatic treatment symptoms disappear, continue the above drug treatment; 1 case with mild liver function abnormalities occur (ALT60U), by reduction and liver VPA medication, two weeks after the ALT returned to normal. Increase medication use in children with post-NZP group have varying degrees of adverse reactions, some children appear drowsiness, weakness or ataxia, such as a more serious toxicity.Fully up, low-dose hormone (ACTH + prednisone), topiramate, vitamin B6, intravenous immunoglobulin treatment of infantile spasms, cramps control for quick effect, less side effects. Four drug combination therapy cryptogenic and symptomatic, treatment effects are good, no significant difference. Four drug combination therapy on the EEG changes in infantile spasms Obviously, the peak disappeared faster rhythm disorders. Therefore, four drug combination therapy with infantile spasms safety, effectiveness, reliability characteristics. Consider that a small number of samples in this study, the observation of adverse drug reaction is limited, need large sample confirmed the observation of clinical research.
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