Patient's question:
Thank you for your answer. My nephew has been taken to the hospital for detailed examinations, but he is still under observation. Could you please tell me if there is a fundamental difference between the spasms you mentioned and epilepsy? What are the main factors or causes of the onset? Is infantile spasms simply an early recurrence of epilepsy?Doctor's answer:
Infantile Spasms (West Syndrome)I. Age of Onset and Incidence of Infantile Spasms
1. Infantile spasms typically occur at an early age. According to statistics from our hospital's outpatient visits, the most common onset age is between 3 and 9 months after birth. Some patients may begin experiencing spasms within days of birth. Ninety percent of cases are diagnosed before the age of one, with only 10% occurring after the first year.
2. The incidence rate of infantile spasms is approximately 1 in 3,000 to 4,000 infants. Our epilepsy clinic admits 4 to 6 cases of infantile spasms each month, averaging 60 cases per year.
II. Clinical Manifestations of Infantile Spasms
1. The clinical manifestations of infantile spasms can be categorized into four types:
(1) Nodding Spasms: Muscle spasms primarily affect the head and neck, causing sudden nodding movements. Some patients exhibit single actions, while others have multiple consecutive nods. Frequent forehead and facial injuries are common among affected children.
(2) Stroking Spasms: These spasms last for an extremely short duration and are only noticeable when observing the child during an episode.
(3) Bowing Spasms: Sudden, brief, generalized muscle spasms where the neck, torso, and legs bend forward, while the arms extend rapidly outward.
(4) Atypical Spasms: These may include one or more of the following symptoms: rotation of the head to one side, spasms in one limb, backward tilting of the head, leg extension, upward rolling of the eyes, involuntary laughter, red or pale complexion, and postictal drowsiness.
2. The clinical manifestations of infantile spasms have three key characteristics:
(1) Each individual spasm lasts for an extremely short duration, often less than a few seconds.
(2) Spasms occur frequently, with multiple episodes occurring consecutively in a short period. Daily spasm counts can range from several to hundreds.
(3) Generalized flexion, particularly of the head and upper body, is observed.
III. Ancillary Examinations
A definitive etiological diagnosis can be made through medical history and necessary examinations.
1. A thorough physical examination and neurological assessment.
2. Electroencephalography (EEG) is essential. If routine EEG results are normal, provocative tests and 24-hour ambulatory EEG may be required. Most infantile spasms patients exhibit abnormal EEG findings, characterized by diffuse, disorganized, high-amplitude, unsynchronized activity mixed with spikes, sharp waves, or multiple spike-and-wave complexes. The amplitude, waveform, and periodicity of these waves change unpredictably, often exceeding 200 μV. This abnormality is almost continuous and represents the most severe type of EEG abnormality, termed "hypsarrhythmia."
3. To identify the underlying cause of infantile spasms, imaging studies such as CT, MRI, and TCD may be performed. Intracranial issues like birth injuries, brain malformations or delays in development, hydrocephalus, and others can lead to infantile spasms. Therefore, these tests are crucial for further etiological diagnosis and targeted treatment.
4. Blood biochemistry (glucose, calcium, phosphorus, electrolytes), cerebrospinal fluid, liver and kidney function tests, chromosomal analysis, and screening for various genetic metabolic disorders may be conducted for further diagnosis or differentiation.
IV. Etiology of Infantile Spasms
The factors or causes of infantile spasms are complex and can include:
1. Birth Injuries: Common causes of symptomatic epilepsy in infants, including forceps-assisted delivery, vacuum extraction, cephalopelvic disproportion, abnormal, macrosomia, prolonged labor, advanced maternal age, and tight birth canals.
2. Congenital Disorders: Brain malformations, hydrocephalus, chromosomal abnormalities, etc.
3. Cerebral Developmental Issues: Developmental delays, brain atrophy, etc.
4. Perinatal Complications: Amniotic fluid aspiration, fetal asphyxia, umbilical cord, placental abruption, placenta previa, cord prolapse, and cesarean delivery, all of which increase the risk of infantile spasms.
5. Infectious Diseases: Some patients may develop postictal sequelae after recovering from encephalitis, meningitis, or brain abscesses.
6. Neurocysticercosis and Echinococcosis: These are less common causes of infantile spasms.
7. Intracranial Tumors: Pediatric tumors are rare causes.
8. Cerebrovascular Diseases: Vascular malformations in children are uncommon causes.
9. Toxins: Lead,, pesticides, as well as systemic diseases like hepatic encephalopathy, rapidly progressive nephritis, and uremia can trigger spasms.
10. Nutritional and Metabolic Disorders: Hypoglycemia, diabetic coma, vitamin B6 deficiency, hyperthyroidism, and other conditions can lead to spasms.
11. Trauma: Both open and closed injuries.
12. Congenital Factors: Referring to damage to the fetus before birth, such as brain developmental abnormalities resulting from maternal abdominal injuries, uterine bleeding, ultraviolet exposure, exposure to harmful medications, and infections from microorganisms (e.g., rubella, measles viruses, and toxoplasmosis).
13. Genetics: Children of epilepsy patients have a 1 in 500 chance of developing infantile spasms.
14. Immunodeficiency: Frequent colds and fever in infants may indicate weakened immunity.
V. Harm and Prognosis of Infantile Spasms in Children
1. If left untreated, some children may experience significant delays in intellectual and motor development, with over 90% of cases showing intelligence levels below normal, with severity dependent on the underlying cause.
2. Functions acquired before onset may be lost after the onset of spasms, leading to apathy, lack of responsiveness, inability to smile, and failure to recognize people. Subsequent developmental milestones such as head control, sitting, standing, walking, and language development are also impaired. Therefore, prompt and aggressive treatment is essential upon diagnosis.
3. If untreated or if treatment is ineffective, the condition may cease spasms within one to three years (in rare cases, up to 6 years or longer), but it often transforms into other types of epilepsy, making treatment more challenging.
4. With appropriate treatment and timely control of spasms, the condition can be completely cured, with minimal impact on intellectual and motor functions.
VI. Basic Principles and Guiding Ideology of Our Epilepsy Treatment Center for Infantile Spasms
1. Integrated Traditional Chinese and Western Medicine: Rapidly control spasms. Since infantile spasms significantly affect a child's intellectual and motor development, impacting their entire life, treatment should be initiated promptly to control spasms as soon as possible, followed by systematic consolidation therapy.
2. Improve Nutritional Support and Development of Brain Nerves: Promote the repair and regeneration of damaged brain nerves. Most infantile spasms patients have congenital brain nerve damage, developmental delays, or insufficient nutritional supply to certain brain cells. In addition to spasms, some patients may also experience motor, sensory, language, or intellectual impairments, with stagnant academic progress. By combining biologically active substances extracted from animal brains with brain-nourishing traditional Chinese medicine, the treatment can accelerate brain development, repair and regenerate new neurons, restore function to damaged nerve cells, heal epileptic foci, and improve cognitive and intellectual abilities. The therapy is highly effective, free of side effects, and poses no adverse reactions.
3. Enhance Immunity: Strengthen the body's disease resistance. Infantile spasms are associated with low immunity, and some children with frequent colds and fever provide direct evidence of weakened immunity. Immunotherapy is suitable for such cases. Improving immunity not only prevents colds and fever but also treats infantile spasms, achieving a dual benefit. These drugs are derived from bovine colostrum and are administered orally, with no observed side effects.
4. Antiepileptic Drug Therapy: Appropriate antiepileptic drugs should be selected based on spasm types. Simultaneously, brain-nourishing medications can counteract potential side effects.
VII. Summary of Clinical Experience
1. Children belong to a special age group, with their physical and brain development occurring rapidly. Therefore, in terms of anatomy, physiology, biochemistry, nutrition, metabolism, immunity, pathology, as well as disease onset, progression, symptom presentation, diagnosis, treatment, prognosis, and prevention, there are significant differences between children and adults, and variations exist even among children of different ages. Thus, the treatment of pediatric epilepsy must consider individual differences and tailor strategies accordingly. Inappropriate treatment leading to recurrent spasms can hinder intellectual development and even result in refractory epilepsy, causing greater harm.
2. Treatment of epilepsy must also prioritize protecting the child's intellectual development, selecting medications with minimal adverse effects on the brain. Drugs with significant side effects, such as phenytoin, sodium valproate, and carbamazepine, should be used sparingly. Multiple Western medications are generally unnecessary; one is sufficient, and no more than two should be used.
3. Combining brain function improvement, traditional Chinese medicine regulation, and antiepileptic therapy offers several advantages:
- It not only controls spasm symptoms but also repairs and regenerates damaged brain nerves, addressing the root cause for a more curative effect.
- It leverages drug interactions to counteract potential side effects of Western medications, allowing children to take them safely.
- It provides rapid and long-term efficacy with lower recurrence rates. Some patients with mild symptoms and short histories may benefit from purely traditional Chinese medicine treatment.
4. Due to rapid physical development in children, regular follow-ups are necessary to monitor blood drug concentrations, adjust dosages, and modify medications as needed.