Patient's question:
On March 2, 2015, kept nodding, hand twitched, Heze Municipal HospitalDoctor's answer:
1. HormonesCurrently, it is widely believed that treatment with ACTH or oral steroids remains the most effective measure, especially for secondary cases. However, the disadvantage of this therapy is its high recurrence rate, as well as its potential to cause infections, hypertension, and electrolyte disorders. Hormones should be used as early as possible once the diagnosis is confirmed, except in cases of active infections. There is currently no standard treatment regimen.
(1) ACTH: The dosage is 25 U/d, administered intramuscularly, with a course of 4–6 weeks. Some authors suggest increasing the dose to 40–80 U/d, given once or divided into two doses intramuscularly, with a course of 4–6 weeks, which can be repeated for several cycles. If control is achieved, oral glucocorticoids should be substituted after 6 weeks, followed by gradual tapering, with complete discontinuation after 2 months.
Rikonen treated patients with ACTH in two groups: high-dose (120–160 U) × 6 weeks and low-dose (20–40 U) × 4 weeks. No significant differences were observed between the groups, suggesting that a low-dose, short-course regimen is safer and more ideal. Some children may relapse after discontinuing ACTH or corticosteroids. In recent years, it has been emphasized that attention should be paid to the presence of cytomegalovirus (CMV) infection before using ACTH. Patients with congenital microcephaly, intellectual disability, typical periventricular calcifications, or chorioretinitis should be highly suspected of possible congenital CMV infection and should discontinue the medication and switch to antiepileptic drugs as soon as possible.
(2) Long-acting hormones: Currently, long-acting hormones such as Decadron can also be used to treat infantile spasms, achieving good therapeutic effects. Method: First, intramuscularly administer 0.1–0.4 g of vitamin B6 daily for 7 consecutive days. Then, administer Decadron intramuscularly at a dose of 0.015–0.025 mg/kg daily for 10 days, followed by every other day for 10 days, then reduce the dose to twice weekly for 10 days, and finally to once weekly for 2 doses. Afterward, complete discontinuation is achieved.
During this period, vitamin B6 should still be administered concurrently. Antiepileptic drugs should also be selected based on the condition, such as clonazepam (Klonopin) and sodium valproate.
2. Antiepileptic drugs
Commonly used drugs include nitrazepam (Mogadon), clonazepam (Klonopin), and sodium valproate. However, these drugs should not be used concurrently with ACTH or corticosteroids, as there is a possibility of antagonism.
3. High-dose vitamin B6
Vitamin B6 is a cofactor of glutamic acid decarboxylase, which catalyzes the production of GABA from glutamic acid. It has inhibitory effects on receptor excitability and blocks synaptic transmission. Intravenous administration of 50 mg/(kg·d) vitamin B6 for 10–14 days may contribute to the complete control of IS.
4. Some also use a ketogenic diet therapy.
5. Surgical treatment:
(1) Cerebral cortex lesion resection
(2) Anterior temporal lobectomy
(3) Hemispheric cortex resection
(4) Corporal commissure sectioning
(5) Stereotactic surgery for epilepsy
(6) Cerebellar electrical stimulation therapy for epilepsy
Patients who do not respond to various treatments may also undergo minimally invasive separated brain pacemaker implantation, which has a significant effect.