Infantile spasms
Infantile spasms, also known as West’s syndrome, is a type of epilepsy unique to infancy, with an early age of onset, a specific form of spasticity, and 90% associated with significant intellectual and physical developmental impairment. The onset of the disease is from a few days after birth to 30 months of age, with the majority of cases occurring between 3 and 9 months of age, with a peak between 4 and 6 months. The clinical manifestations of this disease have 3 main forms.
(1) Bowing-like spasm: It is manifested by a sudden onset of transient generalized muscle spasm, with neck, trunk and legs bent, inward or outward, and arms sharply stretched forward and outward in an embrace-like manner;
(2) Nod-like spasms: Nod-like seizures;
(3) Lightning-like spasms: they are very short-lived and usually difficult to detect if not noticed.
There are 5 characteristics of infantile spasmodic seizure presentation.
(1) Short duration of individual seizures, with one seizure lasting 2 to 10 seconds;
(2) The whole body, especially the head and upper body, is flexed forward;
(3) Frequent seizures, multiple seizures a day, each seizure can be consecutive or even dozens of times;
(4) The seizure usually occurs when the person has just fallen asleep or just woken up and is still in a hazy state of consciousness, and may be accompanied by loss of consciousness, sweating, facial cyanosis and fatigue and drowsiness;
(5) The EEG performance varies during the seizure, and the interictal period is characterized by peak arrhythmias. The disorder mostly stops within 1.5 years of age. After the seizures stop, some of them evolve into other types of seizures, and more than 90% of the children are mentally and motorly stunted, and the treatment is more effective with hormones.
Typical infantile spasms.
(1) Onset before 1 year of age, with peak incidence from March to July;
(2) Three main signs: spastic seizures (typically rapid nodding spasms, bilateral upper limb abduction, lower limb and trunk flexion, lower limb may occasionally be straightened); mental retardation; EEG peak dysrhythmia;
(3) The etiology is diverse and can be divided into idiopathic (or insidious) and symptomatic, with symptomatic being more common;
(4) generally poor prognosis, early treatment with ACTH or corticosteroids is more effective, can also try ketogenic diet treatment.
Atypical infantile spasms: no intellectual impairment, atypical form of seizures (e.g., appearance of startle seizures), or onset earlier than 3 months, may not have characteristic EEG changes.
Lennox-Gastaut syndrome
Lennox-Gastaut syndrome is a specific type of epilepsy syndrome that accounts for 5% to 10% of pediatric epilepsy. This syndrome is also known as epileptic encephalopathy in childhood with diffuse slow spikes and slow waves (formerly known as petit mal variant). The EEG term in the definition, petit mal variant, was originally proposed by Lennox in 1945 to distinguish it from the 3 Hz spikes and slow waves usually seen in typical akathisia seizures. Because children with a slow spike configuration are often associated with mental retardation and other forms of convulsions that are clinically distinct from typical aphasic seizures, it was later considered a separate disease unit, the Lennox syndrome. Subsequently, Gastaut discussed the disease in detail and specified some indispensable conditions for its diagnostic criteria, and in recent years the disease has been called Lennox-Gastaut syndrome (LGS).
Like West syndrome, Lennox-Gastaut syndrome is also divided into two categories: idiopathic and symptomatic. Anyone who has no mental retardation, no background disease, no preexisting etiology, no neurological abnormalities on initial examination, and no neuroimaging abnormalities is included in idiopathic Lennox-Gastaut syndrome, and anyone who does not meet the above five criteria is included in symptomatic Lennox-Gastaut syndrome. The etiology of symptomatic Lennox-Gastaut syndrome is largely the same as that of West syndrome, including prenatal, perinatal, and postnatal factors, congenital metabolic abnormalities, congenital brain developmental abnormalities, infections, and trauma. The pathological changes can be broadly classified into four categories.
①Bilateral diffuse lesions;
②Unilateral diffuse lesions;
(iii) Unilateral focal lesions;
④ lesions of no obvious significance. The clinical presentation of the disease is characterized by.
(1) Onset is common between the ages of l and 6 years, with rare onset after the age of 8 years, and some may develop from West syndrome;
(2) Coexistence of multiple seizure forms (atypical aphasic seizures, tonic seizures, atonic seizures, tonic clonic seizures, partial seizures) and mental retardation.