I. Benign familial neonatal epilepsy
It is a rare autosomal dominant disorder. It is characterized by tonic and clonic convulsive seizures in normal full-term newborns shortly after birth (mostly within 7 days), often combined with autonomic symptoms and motor automatism, with frequent and brief seizures. The child is in good general condition during the interictal period, and the history and examination are normal except for a family history of similar seizures and non-specific changes in the EEG. The prognosis is good, and most of the convulsive seizures disappear within 2-4 weeks. the interictal period of EEG is mostly normal, but some cases have generalized or focal abnormalities.
Early onset myoclonic encephalopathy
Characterized by segmental, wandering myoclonus from the first day to the first few weeks after birth, followed by frequent partial seizures, and some patients have huge and obvious myoclonic and tonic spastic seizures. The EEG shows an eruption-inhibition pattern. The etiology is mostly unclear, and some cases are congenital metabolic disorders. The disease is severe, mortality is high, survivors often have psychomotor retardation, and the prognosis is poor. It is an epileptic encephalopathy.
Otawara syndrome
It is also known as early infantile epileptic encephalopathy and is considered to be the earliest form of age-dependent epileptic encephalopathy. It is characterized by the early onset of tonic spastic seizures with EEG burst-inhibitory patterns and severe psychomotor impairment in infants, with intractable seizures and a very poor prognosis. Survivors often evolve into West syndrome and Lennox-Gastaut syndrome.
Benign infantile myoclonic epilepsy
It is a clinically rare epilepsy syndrome. The main feature is the appearance of generalized myoclonic seizures at the age of 1-2 years (before 3 years), basically without other seizure types. The EEG during seizures is a comprehensive (multiple) spike-slow complex waves. The seizures are easily controlled, with normal growth and development and a good prognosis.
V. Dravet syndrome
It is also known as severe myoclonic epilepsy in infants. The disease begins in normal children within 1 year of age and is characterized by a variety of seizure types including febrile or nonfebrile convulsions, clonic, myoclonic, atypical disorientation, or focal seizures. The EEG is extensive (multiple) spike-slow syndrome waves, with progressive neurological and mental-intelligence decline from the second year, resistance to drug therapy, frequent sequelae, and poor prognosis. It is an epileptic encephalopathy.
Infantile spasms
Also known as West syndrome. It usually starts at 3-12 months of age, has a complex and diverse etiology, and can be classified as symptomatic, cryptogenic and idiopathic, and is an age-dependent response to brain injury. It is characterized by a triad of epileptic spastic seizures, high amplitude EEG dysrhythmias, and psychomotor developmental disorders. It is the most common clinical form of epileptic encephalopathy with a poor overall prognosis.
Lennox-Gastaut syndrome
Lennox-Gastaut syndrome is a clinically common age-related epileptic encephalopathy. It occurs mostly in children aged 1-8 years. The etiology is complex and varied, the pathogenesis is unclear, and some cases evolve from West syndrome. The main features are multiple seizure types, extensive slow (1.5-2.5 Hz) spike-slow complex waves on the EEG, and a triad of mental retardation. The most common seizure types are tonic, atypical aphasic and atonic seizures, but also myoclonic, generalized tonic-clonic and focal seizures. The seizures are usually frequent, difficult to control with medication, and the overall prognosis is poor.
Myoclonic dystonic epilepsy
Myoclonic-atonic epilepsy is also known as Doose syndrome and myoclonic-atonic seizures. It is characterized by myoclonic and burst seizures, the latter mainly due to the atonic mechanism. The EEG during seizures is a widespread irregular 2.5-3 Hz (multi)spike-slow complex wave, with transient electro-resting periods visible on synchronized EMG. The etiology is unknown, and more than half of the patients have episodes that eventually resolve with a good prognosis. Most patients have normal or near normal intelligence.
Benign epilepsy in children with central temporal spike waves
It is also known as benign Rolando epilepsy. It is the most common epilepsy syndrome in childhood and is clearly age dependent, with most patients having onset at the age of 5-10 years. It is mainly characterized by focal motor and sensory seizures of the face and oropharynx, with occasional secondary generalized seizures. Most cases have seizures only during sleep, usually infrequent. The prognosis is good, with almost all cases remitting by the age of 16 y. The EEG is characterized by spikes in the central temporal region, with significantly more emission during sleep.
X. Childhood epilepsy with loss of consciousness
It is an idiopathic generalized epilepsy syndrome common in childhood. It is associated with genetic predisposition. The onset is usually between 4 and 10 years of age. The clinical presentation is characterized by frequent typical aphasic seizures. The EEG background is normal, and the seizure phase is a bilateral extensive, synchronous, symmetrical 3-Hz spike-slow complex wave. The child has normal physical and intellectual development and often resolves by the age of 12 years with a good prognosis.
XI. Panayiotopoulos syndrome.
Previously also known as early-onset benign occipital lobe epilepsy in children. The main clinical features are vomiting-based autonomic symptomatic seizures and seizure persistence, and EEG shows multifocal spike discharges in the occipital region, or combined with extra-occipital spike discharges. It is generally believed that the onset is genetically related and the prognosis is good.
Late onset benign occipital lobe epilepsy in children
The onset of the disease is later than that of early onset, and the age of onset is 3-16 years. The main clinical features are mainly occipital lobe seizures such as visual abnormalities, sometimes accompanied by partial or generalized convulsive seizures, and paroxysmal discharges in the occipital lobe on the EEG. The onset is generally considered to be genetically related, and the prognosis is good.
Acquired epileptic aphasia
It is also known as Landau-Kleffner syndrome. It is rare and is a unique epileptic syndrome of childhood with an unclear etiology. The onset is usually between 2 and 8 years of age. The main clinical manifestations are acquired aphasia, seizures, EEG abnormalities, and behavioral-psychological disturbances. The seizures and EEG changes are age-dependent, often resolving after the age of 15 years, and more than half of the patients have persistent language, psychological and behavioral disturbances. The EEG is characterized by continuous spike-and-slow integrated waves during slow-wave sleep, mostly bilateral, with a predominance of temporal regions.
XIV. Epileptic encephalopathy with continuous spike-and-slow waves during slow-wave sleep
The etiology is unknown, and it belongs to an epileptic encephalopathy. It is an age-dependent syndrome, mainly seen in children. The main features are EEG slow-wave sleep phase electrical continuity, multiple types of seizures, and neuropsychological and motor behavior disorders. The EEG manifestation of CSWS is the essence and hallmark of the disease. there is an overlap between CSWS and LKS, and it is controversial whether they are separate syndromes; many scholars believe that they belong to two manifestations of the same disease entity. In CSWS, the neuropsychological disorder mostly manifests as generalized intellectual regression, with interictal EEG abnormalities mainly in the anterior head (frontal lobe), whereas in LKS, the neuropsychological disorder mainly manifests as acquired aphasia, which may not be accompanied by seizures, with EEG abnormalities mainly located in the bilateral temporal lobe.
XV. Juvenile aphasic epilepsy
It is one of the common idiopathic generalized epilepsy syndromes. The age of onset is mostly 7-16 years, with a peak at 10-12 years. The main clinical features are typical aphasic seizures, with generalized tonic-clonic seizures in about 80% of cases, and myoclonic seizures in about 15% of cases. The EEG during seizures is bilateral widely synchronized, symmetrical 3-4Hz spike-slow integrated waves, and most cases resolve after treatment with good prognosis.
Myoclonic epilepsy in adolescents
Myoclonic epilepsy is a common idiopathic generalized epilepsy syndrome. It usually starts at the age of 12-18 years, with normal growth and neurological examination. More than 80% of cases have generalized tonic-clonic seizures, and about 1/3 of cases have akinetic seizures. The interictal EEG is characterized by bilateral 4-6 Hz multi-spin-slow complex waves. The disease responds well to drug therapy, but most patients require long-term treatment.
XVII. Generalized tonic-clonic seizure only epilepsy
The age of onset is 5-50 years, with a peak age range of 10-20 years. The etiology is unclear and it is idiopathic generalized epilepsy. It refers to the presence of GTCS in all patients, which can occur at any time (during sleep, wakefulness or awakening) and is essentially free of other seizure types. This syndrome encompasses tonic-clonic seizures during the waking period as proposed by the ILAE in 1989. The prognosis is good. The interictal EEG is a widespread 4-5 Hz multi-spin-slow complex wave or multi-spin wave issuance.
XVIII. Hereditary epilepsy with febrile convulsions addition
GEFS+ is a familial hereditary epilepsy syndrome with age of onset mainly in childhood and adolescence. The most common phenotypes are febrile convulsions and febrile convulsion additions, followed by FS/FS+ with myoclonic seizures, FS/FS+ with akathisia, FS/FS+ with atonic seizures, FS/FS+ with partial seizures, and other rare phenotypes are partial epilepsy, idiopathic generalized epilepsy), and individual patients present with Dravet syndrome or Myoclonic dystonic epilepsy. The diagnosis of a GEFS+ family is based on the type of seizure and the EEG characteristics of the specific phenotype.
XIX. Myoclonic dystonic epilepsy
It may be idiopathic, symptomatic, or of unknown etiology. The age of onset is 1-12 years, with a mean of 7 years. The clinical features are frequent myoclonic-atonic seizures. Some patients may also present with generalized tonic-clonic seizures or atonic seizures. The EEG during seizures is a broad 3 Hz spike-slow complex wave. The overall prognosis is not as good as in children and adolescents with atonic epilepsy.
Temporal lobe epilepsy
Temporal lobe epilepsy is the most common type of epilepsy in clinical practice. It is seen mainly in adults and adolescents. In adults, approximately 50% or more of cases are TLE. TLE can be divided into medial temporal lobe epilepsy and lateral temporal lobe epilepsy, with the majority of cases being the former. Most TLE is symptomatic or cryptogenic, and very few are idiopathic (familial TLE). Hippocampal sclerosis is the most common etiology and pathological alteration of TLE. The main clinical manifestations are simple partial seizures (aura), complex partial seizures with automatism and secondary generalized seizures. Epileptiform discharges in the temporal region are visible on the EEG during the interictal period in about 1/3 of patients. Some patients have poor responsiveness to medications and may be considered for surgical treatment.
Frontal lobe epilepsy
The majority of FLE are symptomatic or cryptogenic, and very few are idiopathic. It can develop in both children and adults. The clinical presentation is complex and varied, with great variation among individuals. Common seizure types include simple partial seizures, complex partial seizures, and secondary generalized seizures. Seizures are usually frequent, with significant motor symptoms and short duration, and are mostly seen during sleep. Some cases have a bizarre clinical presentation and sometimes need to be differentiated from non-epileptic seizures. The positive rate of routine EEG examination is low, and some patients have EEG showing epileptiform discharges in the frontal region.
Rasmussen syndrome
Rasmussen’s syndrome is also known as Rasmussen’s encephalitis. The etiology and pathogenesis are unclear. The pathology is characterized by chronic limited inflammation of one cerebral hemisphere. The clinical manifestations are drug-refractory partial motor seizures, which often progress to partial seizure continuity, progressive partial body weakness, and mental retardation. Structural brain imaging shows progressive atrophy of one side of the cerebral cortex. The disease responds poorly to drug therapy, and surgery can be effective in controlling seizures and stopping the progression of the disease. The prognosis of the disease is poor, and most of them have neurological sequelae.
Progressive myoclonic epilepsy
Myoclonic epilepsy is a clinical syndrome consisting of a group of genetic disorders, most of which run in families, but there are also disseminated cases. The common clinical features are myoclonic seizures, epileptic seizures and progressive neurological and mental intellectual decline. The disease is progressive, progression is etiologically related, and most have a poor prognosis. Common specific disorders include Won-Long’s disease, Lafora’s disease, neuronal waxy brown lipid deposition disease, and myoclonic epilepsy with crushed red fibrous disease.